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Surgery_Schwartz. multiple endocrine neoplasia type 4 (MEN4). Endocr Con-nect. 2015;4:1-8. 73. Segiet OA, Deska M, Michalski M, Gawrychowski J, Wojnicz R. Molecular profiling in primary hyperparathyroidism. Head Neck. 2015;37:299-307. 74. Proceedings of the NIH Consensus Development Confer-ence on diagnosis and management of asymptomatic primary hyperparathyroidism. Bethesda, Maryland, October 29-31, 1990. J Bone Miner Res. 1991;6(suppl 2):S1-S166. 75. Silverberg SJ, Shane E, Jacobs TP, Siris E, Bilezikian JP. A 10-year prospective study of primary hyperparathyroid-ism with or without parathyroid surgery. N Engl J Med. 1999;341:1249-1255. 76. Bilezikian JP, Khan AA, Potts JT, Jr. Third International Workshop on the Management of Asymptomatic Primary H. Guidelines for the management of asymptomatic pri-mary hyperparathyroidism: summary statement from the third international workshop. J Clin Endocrinol Metab. 2009;94:335-339. 77. Rubin MR, Bilezikian JP, McMahon DJ, et al. The natu-ral history of


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Surgery_Schwartz. hyperparathyroidism: summary statement from the third international workshop. J Clin Endocrinol Metab. 2009;94:335-339. 77. Rubin MR, Bilezikian JP, McMahon DJ, et al. The natu-ral history of primary hyperparathyroidism with or without parathyroid surgery after 15 years. J Clin Endocrinol Metab. 2008;93:3462-3470. 78. Marcocci C, Bollerslev J, Khan AA, Shoback DM. Medical management of primary hyperparathyroidism: proceedings of the fourth International Workshop on the Management of Asymptomatic Primary Hyperparathyroidism. J Clin Endocri-nol Metab. 2014;99:3607-3618. 79. Pasieka JL, Parsons L, Jones J. The long-term benefit of parathyroidectomy in primary hyperparathyroidism: a 10-year prospective surgical outcome study. Surgery. 2009;146:1006-1013. 80. Zanocco K, Angelos P, Sturgeon C. Cost-effectiveness analy-sis of parathyroidectomy for asymptomatic primary hyper-parathyroidism. Surgery. 2006;140:874-881; discussion 81-82. 81. Udelsman R, Akerstrom G, Biagini C, et al. The


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Surgery_Schwartz. C. Cost-effectiveness analy-sis of parathyroidectomy for asymptomatic primary hyper-parathyroidism. Surgery. 2006;140:874-881; discussion 81-82. 81. Udelsman R, Akerstrom G, Biagini C, et al. The surgical management of asymptomatic primary hyperparathyroidism: proceedings of the Fourth International Workshop. J Clin Endocrinol Metab. 2014;99:3595-3606. 82. Bilezikian JP, Brandi ML, Eastell R, et al. Guidelines for the management of asymptomatic primary hyperparathy-roidism: summary statement from the Fourth International Workshop. J Clin Endocrinol Metab. 2014;99(10):3561-3569. 83. Rodgers SE, Hunter GJ, Hamberg LM, et al. Improved pre-operative planning for directed parathyroidectomy with 4-dimensional computed tomography. Surgery. 2006;140:932-940; discussion 40-41. 84. Sharma J, Milas M, Berber E, Mazzaglia P, Siperstein A, Weber CJ. Value of intraoperative parathyroid hormone moni-toring. Ann Surg Oncol. 2008;15(2):493-498.Brunicardi_Ch38_p1625-p1704.indd 170301/03/19 11:22


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Surgery_Schwartz. M, Berber E, Mazzaglia P, Siperstein A, Weber CJ. Value of intraoperative parathyroid hormone moni-toring. Ann Surg Oncol. 2008;15(2):493-498.Brunicardi_Ch38_p1625-p1704.indd 170301/03/19 11:22 AM 1704SPECIFIC CONSIDERATIONSPART II 85. Westerdahl J, Bergenfelz A. Unilateral versus bilateral neck exploration for primary hyperparathyroidism: five-year follow-up of a randomized controlled trial. Ann Surg. 2007;246:976-981. 86. Gagner M. Endoscopic subtotal parathyroidectomy in patients with primary hyperparathyroidism. Br J Surg. 1996;83:875. 87. Lowe H, McMahon DJ, Rubin MR, Bilezikian JP, Silver-berg SJ. Normocalcemic primary hyperparathyroidism: further characterization of a new clinical phenotype. J Clin Endocrinol Metab. 2007;92(8):3001-3005. 88. Eastell R, Brandi ML, Costa AG, D’Amour P, Shoback DM, Thakker RV. Diagnosis of asymptomatic primary hyperpara-thyroidism: proceedings of the Fourth International Workshop. J Clin Endocrinol Metab. 2014;99:3570-3579. 89. Cetani F, Pardi


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Surgery_Schwartz. P, Shoback DM, Thakker RV. Diagnosis of asymptomatic primary hyperpara-thyroidism: proceedings of the Fourth International Workshop. J Clin Endocrinol Metab. 2014;99:3570-3579. 89. Cetani F, Pardi E, Marcocci C. Update on parathyroid carci-noma. J Endocrinol Invest. 2016;39:595-606. 90. Silverberg SJ, Rubin MR, Faiman C, et al. Cinacalcet hydro-chloride reduces the serum calcium concentration in inop-erable parathyroid carcinoma. J Clin Endocrinol Metab. 2007;92:3803-3808. 91. Iacobone M, Citton M, Viel G, Schiavone D, Torresan F. Surgi-cal approaches in hereditary endocrine tumors. Updates Surg. 2017;69:181-191. 92. Twigt BA, van Dalen T, Vroonhoven TJ, Consten EC. Recur-rent hyperparathyroidism caused by benign neoplastic seeding: two cases of parathyromatosis and a review of the literature. Acta Chir Belg. 2013;113:228-232. 93. Guerin C, Paladino NC, Lowery A, Castinetti F, Taieb D, Sebag F. Persistent and recurrent hyperparathyroidism. Updates Surg. 2017;69:161-169. 94. Madorin C,


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Surgery_Schwartz. Acta Chir Belg. 2013;113:228-232. 93. Guerin C, Paladino NC, Lowery A, Castinetti F, Taieb D, Sebag F. Persistent and recurrent hyperparathyroidism. Updates Surg. 2017;69:161-169. 94. Madorin C, Owen RP, Fraser WD, et al. The surgical manage-ment of renal hyperparathyroidism. Eur Arch Otorhinolaryn-gol. 2012;269:1565-1576. 95. Qiu NC, Zha SL, Liu ME, et al. To assess the effects of para-thyroidectomy (TPTX versus TPTX+AT) for secondary hyper-parathyroidism in chronic renal failure: a meta-analysis. Int J Surg. 2017;44:353-362. 96. Dulfer RR, Franssen GJH, Hesselink DA, Hoorn EJ, van Eijck CHJ, van Ginhoven TM. Systematic review of surgical and medical treatment for tertiary hyperparathyroidism. Br J Surg. 2017;104:804-813. 97. Dutta RK, Soderkvist P, Gimm O. Genetics of primary hyper-aldosteronism. Endocr Relat Cancer. 2016;23:R437-R454. 98. Galati SJ. Primary aldosteronism: challenges in diagno-sis and management. Endocrinol Metab Clin North Am. 2015;44:355-369. 99. Rossi GP, Auchus


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Surgery_Schwartz. Endocr Relat Cancer. 2016;23:R437-R454. 98. Galati SJ. Primary aldosteronism: challenges in diagno-sis and management. Endocrinol Metab Clin North Am. 2015;44:355-369. 99. Rossi GP, Auchus RJ, Brown M, et al. An expert consensus statement on use of adrenal vein sampling for the subtyping of primary aldosteronism. Hypertension. 2014;63:151-160. 100. Funder JW, Carey RM, Mantero F, et al. The management of primary aldosteronism: case detection, diagnosis, and treatment: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2016;101:1889-1916. 101. Powlson AS, Gurnell M, Brown MJ. Nuclear imaging in the diagnosis of primary aldosteronism. Curr Opin Endocrinol Diabetes Obes. 2015;22:150-156. 102. Tahir A, McLaughlin K, Kline G. Severe hyperkalemia fol-lowing adrenalectomy for aldosteronoma: prediction, patho-genesis and approach to clinical management—a case series. BMC Endocr Disord. 2016;16:43. 103. Pecori Giraldi F, Ambrogio AG, De Martin M, Fatti LM, Scacchi M,


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Surgery_Schwartz. for aldosteronoma: prediction, patho-genesis and approach to clinical management—a case series. BMC Endocr Disord. 2016;16:43. 103. Pecori Giraldi F, Ambrogio AG, De Martin M, Fatti LM, Scacchi M, Cavagnini F. Specificity of first-line tests for the diagnosis of Cushing’s syndrome: assessment in a large series. J Clin Endocrinol Metab. 2007;92:4123-4129. 104. Faillot S, Assie G. Endocrine tumours: the genomics of adre-nocortical tumors. Eur J Endocrinol. 2016;174:R249-R265. 105. Copeland PM. The incidentally discovered adrenal mass. Ann Surg. 1984;199:116-122. 106. Sturgeon C, Shen WT, Clark OH, Duh QY, Kebebew E. Risk assessment in 457 adrenal cortical carcinomas: how much does tumor size predict the likelihood of malignancy? J Am Coll Surg. 2006;202:423-430. 107. Aubert S, Wacrenier A, Leroy X, et al. Weiss system revisited: a clinicopathologic and immunohistochemical study of 49 adrenocortical tumors. Am J Surg Pathol. 2002;26:1612-1619. 108. Terzolo M, Angeli A, Fassnacht M, et


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Surgery_Schwartz. Leroy X, et al. Weiss system revisited: a clinicopathologic and immunohistochemical study of 49 adrenocortical tumors. Am J Surg Pathol. 2002;26:1612-1619. 108. Terzolo M, Angeli A, Fassnacht M, et al. Adjuvant mito-tane treatment for adrenocortical carcinoma. N Engl J Med. 2007;356:2372-2380. 109. Berruti A, Grisanti S, Pulzer A, et al. Long-term outcomes of adjuvant mitotane therapy in patients with radically resected adrenocortical carcinoma. J Clin Endocrinol Metab. 2017;102:1358-1365. 110. Postlewait LM, Ethun CG, Tran TB, et al. Outcomes of adju-vant mitotane after resection of adrenocortical carcinoma: a 13-institution study by the US Adrenocortical Carcinoma Group. J Am Coll Surg. 2016;222:480-490. 111. Fassnacht M, Terzolo M, Allolio B, et al. Combination chemo-therapy in advanced adrenocortical carcinoma. N Engl J Med. 2012;366:2189-2197. 112. Rednam SP, Erez A, Druker H, et al. Von Hippel-Lindau and hereditary pheochromocytoma/paraganglioma syndromes: clinical features,


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Surgery_Schwartz. adrenocortical carcinoma. N Engl J Med. 2012;366:2189-2197. 112. Rednam SP, Erez A, Druker H, et al. Von Hippel-Lindau and hereditary pheochromocytoma/paraganglioma syndromes: clinical features, genetics, and surveillance recommendations in childhood. Clin Cancer Res. 2017;23:e68-e75. 113. Brunaud L, Boutami M, Nguyen-Thi PL, et al. Both preopera-tive alpha and calcium channel blockade impact intraoperative hemodynamic stability similarly in the management of pheo-chromocytoma. Surgery. 2014;156:1410-1417; discussion7-8. 114. Scholten A, Valk GD, Ulfman D, Borel Rinkes IH, Vriens MR. Unilateral subtotal adrenalectomy for pheochromocytoma in multiple endocrine neoplasia type 2 patients: a feasible surgi-cal strategy. Ann Surg. 2011;254:1022-1027. 115. Shen WT, Sturgeon C, Clark OH, Duh QY, Kebebew E. Should pheochromocytoma size influence surgical approach? A com-parison of 90 malignant and 60 benign pheochromocytomas. Surgery. 2004;136:1129-1137. 116. McDermott S, O’Connor OJ, Cronin


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Surgery_Schwartz. Kebebew E. Should pheochromocytoma size influence surgical approach? A com-parison of 90 malignant and 60 benign pheochromocytomas. Surgery. 2004;136:1129-1137. 116. McDermott S, O’Connor OJ, Cronin CG, Blake MA. Radio-logical evaluation of adrenal incidentalomas: current methods and future prospects. Best Pract Res Clin Endocrinol Metab. 2012;26:21-33. 117. Zeiger MA, Thompson GB, Duh QY, et al. The American Association of Clinical Endocrinologists and American Association of Endocrine Surgeons medical guidelines for the management of adrenal incidentalomas. Endocr Pract. 2009;15(suppl 1):1-20. 118. Glenn JA, Kiernan CM, Yen TW, et al. Management of sus-pected adrenal metastases at 2 academic medical centers. Am J Surg. 2016;211:664-670. 119. Economopoulos KP, Mylonas KS, Stamou AA, et al. Laparo-scopic versus robotic adrenalectomy: a comprehensive meta-analysis. Int J Surg. 2017;38:95-104.Brunicardi_Ch38_p1625-p1704.indd 170401/03/19 11:22 AM


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Surgery_Schwartz. INTRODUCTIONIn his 1953 classic textbook entitled The Surgery of Infancy and Childhood, Dr. Robert E. Gross summarized the essential challenge of pediatric surgery: “Those who daily operate upon adults, even with the greatest of skill, are sometimes appalled—or certainly are not at their best —when called upon to operate upon and care for a tiny patient. Something more than diminu-tive instruments or scaled-down operative manipulations are necessary to do the job in a suitable manner.” To this day, surgi-cal residents and other trainees often approach the pediatric sur-gical patient with the same mix of fear, trepidation, and anxiety. These same trainees often complete their pediatric surgical rotations with a profound respect for the resilience of young children to undergo complex operations and an appreciation for the precision required from their caregivers, both in the operat-ing room and during the perioperative period. Over the decades, the specialty of pediatric surgery has


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Surgery_Schwartz. and an appreciation for the precision required from their caregivers, both in the operat-ing room and during the perioperative period. Over the decades, the specialty of pediatric surgery has evolved considerably in its care for the smallest of surgical patients, such that in utero sur-gery is now an option in an increasing number of circumstances. Similarly, our understanding of the pathophysiology of the dis-eases that pediatric surgeons face has increased to the point that some pediatric surgical diseases are now understood at the level of molecular or cellular signaling pathways. Pediatric surgery provides the opportunity to intervene in a wide array of diseases and to exert a long-lasting impact on the lives of children and their grateful parents. The scope of diseases encountered in the standard practice of pediatric surgery is immense, with patients Pediatric SurgeryDavid J. Hackam, Jeffrey Upperman, Tracy Grikscheit, Kasper Wang, and Henri R. Ford


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Surgery_Schwartz. of diseases encountered in the standard practice of pediatric surgery is immense, with patients Pediatric SurgeryDavid J. Hackam, Jeffrey Upperman, Tracy Grikscheit, Kasper Wang, and Henri R. Ford 39chapterIntroduction1705Pediatric Surgical Themes: Pitfalls and Pearls1706General Considerations1707Fluid and Electrolyte Balance / 1707Acid-Base Equilibrium / 1707Blood Volume and Blood Replacement / 1707Parenteral Alimentation and Nutrition / 1708Venous Access / 1709Thermoregulation / 1709Pain Control / 1710Neck Masses1710Lymphadenopathy / 1710Thyroglossal Duct Remnants / 1710Branchial Cleft Anomalies / 1711Lymphatic Malformation / 1711Torticollis / 1712Respiratory System1712Congenital Diaphragmatic Hernia (Bochdalek) / 1712Congenital Lobar Emphysema / 1714Bronchopulmonary Foregut Malformations / 1715Bronchiectasis / 1716Foreign Bodies / 1716Esophagus1717Esophageal Atresia and Tracheoesophageal Fistula / 1717Corrosive Injury of the Esophagus / 1721Gastroesophageal Reflux /


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Surgery_Schwartz. Malformations / 1715Bronchiectasis / 1716Foreign Bodies / 1716Esophagus1717Esophageal Atresia and Tracheoesophageal Fistula / 1717Corrosive Injury of the Esophagus / 1721Gastroesophageal Reflux / 1721Gastrointestinal Tract1722An Approach to the Vomiting Infant / 1722Hypertrophic Pyloric Stenosis / 1722Intestinal Obstruction in the Newborn / 1723Duodenal Obstruction / 1724Intestinal Atresia / 1724Malrotation and Midgut Volvulus / 1725Meconium Ileus / 1726Necrotizing Enterocolitis / 1727Short Bowel Syndrome / 1730Intussusception / 1731Appendicitis / 1731Intestinal Duplications / 1733Meckel’s Diverticulum / 1733Mesenteric Cysts / 1733Hirschsprung’s Disease / 1734Anorectal Malformations / 1735Jaundice1737The Approach to the Jaundiced Infant / 1737Biliary Atresia / 1737Choledochal Cyst / 1739Deformities of the Abdominal Wall1740Embryology of the Abdominal Wall / 1740Umbilical Hernia / 1740Patent Urachus / 1740Omphalocele / 1740Gastroschisis / 1741Prune-Belly Syndrome / 1743Inguinal


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Surgery_Schwartz. 1739Deformities of the Abdominal Wall1740Embryology of the Abdominal Wall / 1740Umbilical Hernia / 1740Patent Urachus / 1740Omphalocele / 1740Gastroschisis / 1741Prune-Belly Syndrome / 1743Inguinal Hernia / 1743Genitalia1744Undescended testis / 1744Vaginal Anomalies / 1745Ovarian Cysts and Tumors / 1745Ambiguous Genitalia / 1746Pediatric Malignancy1747Wilms’ Tumor / 1747Neuroblastoma / 1748Rhabdomyosarcoma / 1749Teratoma / 1750Liver Tumors / 1751Trauma in Children1751Mechanisms of Injury / 1751Initial Management / 1752Evaluation of Injury / 1752Injuries to the Central Nervous System / 1752Thoracic Injuries / 1752Abdominal Injuries / 1752Fetal Intervention1753Fetal Surgery for Lower Urinary Tract Obstruction / 1754Fetal Surgery for Myelomeningocele / 1754The EXIT Procedure / 1754Brunicardi_Ch39_p1705-p1758.indd 170512/02/19 11:26 AM 1706Key Points1 In infants with Bochdalek-type congenital diaphragmatic hernia, the severity of pulmonary hypoplasia and the resul-tant pulmonary


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Surgery_Schwartz. 170512/02/19 11:26 AM 1706Key Points1 In infants with Bochdalek-type congenital diaphragmatic hernia, the severity of pulmonary hypoplasia and the resul-tant pulmonary hypertension are key determinants of sur-vival. Barotrauma and hypoxia should be avoided.2 During initial management of an infant with esophageal atresia and distal tracheoesophageal fistula, every effort should be made to avoid distending the gastrointestinal tract, especially when using mechanical ventilation. The patient should be evaluated for components of the VAC-TERRL (vertebral, anorectal, cardiac, tracheoesophageal, renal, radial limb) anomalies. Timing and extent of surgery are dictated by the stability of the patient.3 Although malrotation with midgut volvulus occurs most commonly within the first few weeks of life, it should always be considered in the differential diagnosis in a child with bilious emesis. Volvulus is a surgical emergency; therefore, in a critically ill child, prompt surgical


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Surgery_Schwartz. few weeks of life, it should always be considered in the differential diagnosis in a child with bilious emesis. Volvulus is a surgical emergency; therefore, in a critically ill child, prompt surgical interven-tion should not be delayed for any reason.4 When evaluating a newborn infant for vomiting, it is criti-cal to distinguish between proximal and distal causes of intestinal obstruction using both prenatal and postnatal history, physical examination, and abdominal radiographs.5 Risk factors for necrotizing enterocolitis (NEC) include prematurity, formula feeding, bacterial infection, and intestinal ischemia. Critical to the management of infants with advanced (Bell stage III) or perforated NEC is timely and adequate source control of peritoneal contamination. Early sequelae of NEC include perforation, sepsis, and death. Later sequelae include short bowel syndrome and stricture.6 In patients with intestinal obstruction secondary to Hirschsprung’s disease, a leveling ostomy or


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Surgery_Schwartz. include perforation, sepsis, and death. Later sequelae include short bowel syndrome and stricture.6 In patients with intestinal obstruction secondary to Hirschsprung’s disease, a leveling ostomy or endorectal pull-through should be performed using ganglionated bowel, proximal to the transition zone between ganglionic and aganglionic intestine.7 Prognosis of infants with biliary atresia is directly related to age at diagnosis and timing of portoenterostomy. Infants with advanced age at the time of diagnosis or infants who fail to demonstrate evidence of bile drainage after porto-enterostomy usually require liver transplantation.8 Infants with omphaloceles have greater associated morbid-ity and mortality than infants with gastroschisis due to a higher incidence of congenital anomalies and pulmonary hypoplasia. Gastroschisis can be associated with intestinal atresia, but not with other congenital anomalies. An intact omphalocele can be repaired electively, whereas gastros-chisis requires


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Surgery_Schwartz. hypoplasia. Gastroschisis can be associated with intestinal atresia, but not with other congenital anomalies. An intact omphalocele can be repaired electively, whereas gastros-chisis requires urgent intervention to protect the exposed intestine.9 Prognosis for children with Wilms’ tumor is defined by the stage of disease at the time of diagnosis and the histo-logic type (favorable vs. unfavorable). Preoperative che-motherapy is indicated for bilateral involvement, a solitary kidney, or tumor in the inferior vena cava above the hepatic veins. Gross tumor rupture during surgery auto-matically changes the stage to 3 (at a minimum).10 Injury is the leading cause of death in children older than 1 year of age. Blunt mechanisms account for the majority of pediatric injuries. The central nervous system is the most commonly injured organ system and the leading cause of death in injured children.ranging in age from the fetus to 18 years old, and it includes pathologies in the head and neck,


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Surgery_Schwartz. system is the most commonly injured organ system and the leading cause of death in injured children.ranging in age from the fetus to 18 years old, and it includes pathologies in the head and neck, thoracic, gastrointestinal, and genitourinary regions. This chapter is not designed to cover the entire spectrum of diseases a pediatric surgeon is expected to master; rather, it presents a synopsis of the most commonly encountered pediatric surgical conditions that a practicing gen-eral surgeon is likely to treat over the course of her or his career.PEDIATRIC SURGICAL THEMES: PITFALLS AND PEARLSThis chapter focuses on the unique considerations regarding the diagnosis and management of surgical diseases in the pediatric population. Many surgical trainees approach the surgical care of children with some degree of fear and trepidation. As any pediatric caregiver will attest to, the surgical management of infants and children requires delicate, careful, and professional interactions with their


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Surgery_Schwartz. some degree of fear and trepidation. As any pediatric caregiver will attest to, the surgical management of infants and children requires delicate, careful, and professional interactions with their parents. The stress that the parents of sick children experience in the hospital setting can, at times, be over-whelming. It is due, in part, to the uncertainty regarding a par-ticular prognosis, the feeling of helplessness that evolves when one is unable to care for one’s own child, and in certain cases, the guilt or remorse that one feels for not seeking medical care earlier, or for consenting to a particular procedure. Management of the sick child and his or her family requires not only a cer-tain set of skills but also a unique knowledge base. This section is included to summarize some important general principles in accomplishing this task.1. Children are not little adults, but they are little people. In practical terms, this often-heard refrain implies that children have unique fluid,


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Surgery_Schwartz. general principles in accomplishing this task.1. Children are not little adults, but they are little people. In practical terms, this often-heard refrain implies that children have unique fluid, electrolyte, and medication needs. Thus, the dosage of medications and the administration of IV fluids should at all times be based on their weight. The corollary of this point is that infants and young children are extremely sensitive to perturbations in their normal physiology and may be easily tipped into fluid overload or dehydration.2. Sick children whisper before they shout. Children with surgi-cal diseases can deteriorate very quickly. But before they dete-riorate, they often manifest subtle physical findings. These findings—referred to as “whispers”—may include signs such as tachycardia, bradycardia, hypothermia, fever, recurrent emesis, or feeding intolerance. Meticulous attention to these subtle findings may unmask the development of potentially serious, life-threatening


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Surgery_Schwartz. bradycardia, hypothermia, fever, recurrent emesis, or feeding intolerance. Meticulous attention to these subtle findings may unmask the development of potentially serious, life-threatening physiological disturbances.3. Always listen to the mother and the father. Surgical diseases in children can be very difficult to diagnose because children are often minimally communicative, and information that they communicate may be confusing, conflicting, or both. In all cases, it is wise to listen to the child’s parents, who have closely observed their child and know him or her best. Most importantly, the child’s parents know with certainty Brunicardi_Ch39_p1705-p1758.indd 170612/02/19 11:26 AM 1707PEDIATRIC SURGERYCHAPTER 39whether or not the child is sick or not, despite not always knowing the precise diagnosis.4. Pediatric tissue must be handled delicately and with pro-found respect.5. Children suffer pain after surgery. Timely and adequate pain management must accompany surgical


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Surgery_Schwartz. the precise diagnosis.4. Pediatric tissue must be handled delicately and with pro-found respect.5. Children suffer pain after surgery. Timely and adequate pain management must accompany surgical interventions.6. Pay particular attention to the postoperative pediatric patient whose pain cannot be soothed by the administration of stan-dard amounts of analgesic agents. Ask yourself whether a sig-nificant yet unrecognized postoperative complication exists.GENERAL CONSIDERATIONSFluid and Electrolyte BalanceIn managing the pediatric surgical patient, an understanding of fluid and electrolyte balance is critical as the margin between dehydration and fluid overload is small. This is particularly true in infants, who have little reserve at baseline and even less when ill. Failure to pay meticulous attention to their hydration status can result in significant fluid overload or dehydration. Several surgical diagnoses such as gastroschisis or short-gut syndrome are characterized by a


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Surgery_Schwartz. meticulous attention to their hydration status can result in significant fluid overload or dehydration. Several surgical diagnoses such as gastroschisis or short-gut syndrome are characterized by a predisposition to fluid loss. Others require judicious restoration of intravascular volume in order to pre-vent cardiac failure as is the case in patients with congenital diaphragmatic hernia and associated pulmonary hypertension.The infant’s physiologic day is approximately eight hours in duration. Accordingly, careful assessment of the individual patient’s fluid balance, including fluid intake and output for the previous eight hours, is essential to prevent dehydration or fluid overload. Clinical signs of dehydration include tachycardia, decreased urine output, reduced skin turgor, depressed fonta-nelle, absent tears, lethargy, and poor feeding. Fluid overload is often manifested by the onset of a new oxygen requirement, respiratory distress, tachypnea, and tachycardia. The physi-cal


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Surgery_Schwartz. fonta-nelle, absent tears, lethargy, and poor feeding. Fluid overload is often manifested by the onset of a new oxygen requirement, respiratory distress, tachypnea, and tachycardia. The physi-cal assessment of the fluid status of each child must include a complete head-to-toe evaluation, with emphasis on determining whether perturbations in normal physiology are present.At 12 weeks’ gestation, the total body water of a fetus is approximately 94 cc/kg. By the time the fetus reaches full term, the total body water has decreased to approximately 80 cc/kg. Total body water drops an additional 5% within the first week of life, and by 1 year of life, total body water approaches adult levels, around 60 to 65 cc/kg. Parallel to the drop in total body water is the reduction in extracellular fluid. These changes are accelerated in the preterm infant who may face additional fluid losses due to coexisting congenital anomalies or surgery. Nor-mal daily maintenance fluids for most children can be


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Surgery_Schwartz. These changes are accelerated in the preterm infant who may face additional fluid losses due to coexisting congenital anomalies or surgery. Nor-mal daily maintenance fluids for most children can be estimated using the following formula:100 mL/kg for the first 10 kg, plus 50 mL/kg for 11 to 20 kg, plus 25 mL/kg for each additional kilogram of body weight thereafter.Because IV (I.V.) fluid orders are written as milliliters per hour, this can be conveniently converted to:4 mL/kg/h up to 10 kg, add 2 mL/kg/h for 11 to 20 kg, and add 1 mL/kg/h for each additional kilogram body weight thereafter.For example, a 26-kg child has an estimated maintenance fluid requirement of (10 × 4) + (10 × 2) + (6 × 1) = 66 mL/h in the absence of massive fluid losses or shock. A newborn infant with gastroschisis will manifest significant evaporative losses from the exposed bowel such that fluid requirements can be on the order of 150 to 180 cc/kg/day.Precise management of a neonate’s fluid status requires


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Surgery_Schwartz. will manifest significant evaporative losses from the exposed bowel such that fluid requirements can be on the order of 150 to 180 cc/kg/day.Precise management of a neonate’s fluid status requires an understanding of changes in the glomerular filtration rate (GFR) and tubular function of the kidney. The term newborn’s GFR is approximately 21 mL/min/1.73 m2 compared to 70 mL/min/1.73 m2 in an adult. Within the first 2 weeks of life GFR increases to approximately 60, and by 2 years of age it is essentially at adult levels. The capacity to concentrate urine is very limited in preterm and term infants. In comparison to an adult who can concentrate urine to 1200 mOsm/kg, infants can concentrate urine at best to 600 mOsm/kg. While infants are capable of secreting antidiuretic hormone, ADH, the aquaporin water channel–mediated osmotic water permeability of the infant’s collecting tubules is severely limited compared to that of adults, leading to an insensitivity to ADH.Sodium requirements


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Surgery_Schwartz. aquaporin water channel–mediated osmotic water permeability of the infant’s collecting tubules is severely limited compared to that of adults, leading to an insensitivity to ADH.Sodium requirements range from 2 mEq/kg per day in term infants up to 5 mEq/kg per day in critically ill preterm infants as a consequence of salt wasting. Potassium require-ments are on the order of 1 to 2 mEq/kg per day. Calcium and magnesium supplementation of IV fluids is essential to prevent laryngospasm, dysrhythmias, and tetany.Acid-Base EquilibriumAcute metabolic acidosis usually implies inadequate tissue perfusion and is a serious disorder in children. Potentially life-threatening causes that are specific for the pediatric population must be sought; they include intestinal ischemia from necro-tizing enterocolitis (in the neonate), midgut volvulus, or incar-cerated hernia. Other causes include chronic bicarbonate loss from the gastrointestinal tract or acid accumulation as in chronic renal failure.


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Surgery_Schwartz. (in the neonate), midgut volvulus, or incar-cerated hernia. Other causes include chronic bicarbonate loss from the gastrointestinal tract or acid accumulation as in chronic renal failure. Respiratory acidosis implies hypoventilation, the cause of which should be apparent. Treatment of acute meta-bolic acidosis should be aimed at restoring tissue perfusion by addressing the underlying abnormality first. For severe meta-bolic acidemia where the serum pH is less than 7.25, sodium bicarbonate should be administered using the following guide-line: base deficit × weight in kilograms × 0.5 (in newborns). The last factor in the equation should be 0.4 for smaller children and 0.3 for older children. The dose should be diluted to a concentra-tion of 0.5 mEq/mL because full-strength sodium bicarbonate is hyperosmolar. One-half the corrective dose is given, and the serum pH is measured again. During cardiopulmonary resusci-tation (CPR), one-half the corrective dose can be given as an intravenous


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Surgery_Schwartz. is hyperosmolar. One-half the corrective dose is given, and the serum pH is measured again. During cardiopulmonary resusci-tation (CPR), one-half the corrective dose can be given as an intravenous bolus and the other half given slowly intravenously.Respiratory alkalosis is usually caused by hyperventila-tion, which is readily correctable. Metabolic alkalosis most commonly implies gastric acid loss, as in the child with pyloric stenosis, or aggressive diuretic therapy. In the child with gastric fluid loss, IV fluids of 5% dextrose, 0.5% normal saline, and 20 mEq KCl/L usually correct the alkalosis.Blood Volume and Blood ReplacementCriteria for blood transfusion in infants and children remain poorly defined. The decision to transfuse a critically ill pediatric patient may depend on a number of clinical features that include the patient’s age, primary diagnosis, the presence of ongoing bleeding, coagulopathy, hypoxia, hemodynamic compromise, lactic acidosis, cyanotic heart disease, and


Surgery_Schwartz

Surgery_Schwartz. of clinical features that include the patient’s age, primary diagnosis, the presence of ongoing bleeding, coagulopathy, hypoxia, hemodynamic compromise, lactic acidosis, cyanotic heart disease, and overall severity of illness. A recent survey of transfusion practices among pediatric intensivists showed that the baseline hemoglobin levels that would prompt them to recommend RBC transfusion ranged from 7 to 13 g/dL. Patients with cyanotic heart disease are often transfused to Brunicardi_Ch39_p1705-p1758.indd 170712/02/19 11:26 AM 1708SPECIFIC CONSIDERATIONSPART IIhigher hemoglobin values, although the threshold for transfusion in this population remains to be defined. In general terms, there is a trend towards an avoidance of the use of RBC products whenever possible as current studies suggest that lower hemoglobin concentrations are well tolerated by many groups of patients and that administration of RBCs may have unintended negative consequences, including perhaps an increase in


Surgery_Schwartz

Surgery_Schwartz. suggest that lower hemoglobin concentrations are well tolerated by many groups of patients and that administration of RBCs may have unintended negative consequences, including perhaps an increase in predisposition to the development of necrotizing enterocolitis, although this finding is controversial. In addition, there is increasing evidence that PRBC transfusion may have adverse effects on the host immune in both children and adults. These effects are poorly understood but may include effects due to RBC storage and due to factors that are particular to the individual RBC donor. The TRIPICU randomized controlled trial by Lacroix et al in 2007, which was performed in stable critically ill children, determined that a restrictive Hb transfusion trigger (70 g/L) was as safe as a liberal Hb trigger (95 g/L) and was associated with reduced blood use. It remains uncertain whether this can be extrapolated to unstable patients. Expert opinion now generally favors an Hb transfusion trigger of


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Surgery_Schwartz. (95 g/L) and was associated with reduced blood use. It remains uncertain whether this can be extrapolated to unstable patients. Expert opinion now generally favors an Hb transfusion trigger of 70 g/L in stable critically ill children, which is the same as the recommendation for adult patients (see Chapter 7). A higher threshold should be considered if the child has symptomatic anemia or impaired cardiorespiratory function.A useful guideline for estimating blood volume for the newborn infant is approximately 80 mL/kg of body weight. When packed red blood cells are required, the transfusion requirement is usually administered in 10 mL/kg increments, which is roughly equivalent to a 500-mL transfusion for a 70-kg adult. The following formula may be used to determine the vol-ume (ml) of PRBC to be transfused:(Target hematocrit—Current Hematocrit) × weight (kg) × 80/65 (65 represents the estimated hematocrit of a unit of PRBC)As a general rule, blood is recommended for replacement of


Surgery_Schwartz

Surgery_Schwartz. to be transfused:(Target hematocrit—Current Hematocrit) × weight (kg) × 80/65 (65 represents the estimated hematocrit of a unit of PRBC)As a general rule, blood is recommended for replacement of volume loss if the child’s perfusion is inadequate despite administration of 2 to 3 boluses of 20 mL/kg of isotonic crystalloid. Consideration should be given for the administration of 10 mL/kg of packed red blood cells as soon as possible. Type O blood can be administered without a cross-match and is relatively safe; type-specific blood can be obtained quite quickly; however, unlike fully cross-matched blood, incompatibilities other than ABO and Rh may exist.In the child, coagulation deficiencies may rapidly assume clinical significance after extensive blood transfusion. It is advisable to have fresh frozen plasma and platelets available if more than 30 mL/kg have been transfused. Plasma is given in a dose of 10 to 20 mL/kg, and platelets are given in a dose of 1 unit/5 kg. Each unit of


Surgery_Schwartz

Surgery_Schwartz. fresh frozen plasma and platelets available if more than 30 mL/kg have been transfused. Plasma is given in a dose of 10 to 20 mL/kg, and platelets are given in a dose of 1 unit/5 kg. Each unit of platelets consists of 40 to 60 mL of fluid (plasma plus platelets). Following transfusion of PRBCs to neonates with tenuous fluid balance, a single dose of a diuretic (such as furosemide 1 mg/kg) may help to facilitate excretion of the extra fluid load. Many clinicians prefer to administer fresh products to minimize the deleterious effects of red cell storage.In pediatric patients who have lost greater than 30 mL/kg with ongoing bleeding, consideration should be given to initia-tion of a massive transfusion protocol. Such a protocol involves transfusion, based on weight, of 1:1:1 transfusion of RBCs, plasma, and platelets.Parenteral Alimentation and NutritionThe nutritional requirements of the surgical neonate must be met in order for the child to grow and to heal surgical wounds. Table


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Surgery_Schwartz. of RBCs, plasma, and platelets.Parenteral Alimentation and NutritionThe nutritional requirements of the surgical neonate must be met in order for the child to grow and to heal surgical wounds. Table 39-1Nutritional requirements for the pediatric surgical patientAGECALORIESPROTEIN(kcal/kg/d)(gram/kg/d)0–6 months100–12026 months–1 year1001.51–3 years1001.24–6 years9017–10 years70111–14 years55115–18 years451If inadequate protein and carbohydrate calories are given, the child may not only fail to recover from surgery but may also exhibit growth failure and impaired development of the central nervous system. In general terms, the adequacy of growth must be assessed frequently by determining both total body weight as well as head circumference. Neonates that are particularly predisposed to protein-calorie malnutrition include those with gastroschisis, intestinal atresia, or intestinal insufficiency from other causes, such as necrotizing enterocolitis. The protein and caloric requirements


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Surgery_Schwartz. malnutrition include those with gastroschisis, intestinal atresia, or intestinal insufficiency from other causes, such as necrotizing enterocolitis. The protein and caloric requirements for the surgical neonate are shown in Table 39-1.Nutrition can be provided via either the enteral or parenteral routes. Whenever possible, the enteral route is preferred because it not only promotes the growth and function of the gastrointestinal system, it also ensures that the infant learns how to feed. There are various enteral feeding preparations available; these are outlined in Table 39-2. The choice of formula is based upon the individual clinical state of the child. Pediatric surgeons are often faced with situations where oral feeding is not possible. This problem can be seen in the extremely premature infant who has not yet developed the feeding skills, or in the infant with concomitant craniofacial anomalies that impair sucking, for example. In these instances, enteral feeds can be


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Surgery_Schwartz. premature infant who has not yet developed the feeding skills, or in the infant with concomitant craniofacial anomalies that impair sucking, for example. In these instances, enteral feeds can be administered either a nasojejunal or a gastrostomy tube.When the gastrointestinal tract cannot be used because of mechanical, ischemic, inflammatory, or functional disorders, parenteral alimentation must be given. Prolonged parenteral nutrition is delivered via a central venous catheter. Peripheral IV alimentation can be given, utilizing less concentrated but greater volumes of solutions. Long-term parenteral nutrition should include supplemental copper, zinc, and iron to prevent the development of trace metal deficiencies. A major complica-tion of long-term total parenteral nutrition (TPN) is the devel-opment of parenteral nutrition–associated cholestasis, which can eventually progress to liver failure. To prevent this major complication, concomitant enteral feedings should be instituted, and


Surgery_Schwartz

Surgery_Schwartz. devel-opment of parenteral nutrition–associated cholestasis, which can eventually progress to liver failure. To prevent this major complication, concomitant enteral feedings should be instituted, and the gastrointestinal tract should be used as soon as pos-sible. When proximal stomas are in place, gastrointestinal con-tinuity should be restored as soon as possible. Where intestinal insufficiency is associated with dilation of the small intestine, tapering or intestinal lengthening procedures may be beneficial. Brunicardi_Ch39_p1705-p1758.indd 170812/02/19 11:26 AM 1709PEDIATRIC SURGERYCHAPTER 39Table 39-2Formulas for pediatric surgical neonatesFORMULAkcal/mLPROTEIN (g/mL)FAT (g/mL)CARBOHYDRATE (g/mL)Human milk0.670.0110.040.07Milk-based formula Enfamil 200.670.0150.0380.069Similac 200.670.0150.0360.072Soy-based formula Prosobee0.670.020.0360.07Isomil0.670.0180.0370.068Special formula Pregestimil.67.019.028.091Alimentum.67.019.038.068Preterm Enfamil


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Surgery_Schwartz. 200.670.0150.0360.072Soy-based formula Prosobee0.670.020.0360.07Isomil0.670.0180.0370.068Special formula Pregestimil.67.019.028.091Alimentum.67.019.038.068Preterm Enfamil Premature.80.024.041.089Other strategies to minimize the development of TPN-related liver disease include meticulous catheter care to avoid infec-tion, which increases cholestatic symptoms, aggressive treat-ment of any infection, and early cycling of parenteral nutrition in older children who can tolerate not receiving continuous dextrose solution for a limited period. Evidence suggests that cholestasis eventually resolves in most cases after parenteral nutrition is discontinued, as measured by levels of total bili-rubin. Preliminary evidence suggests that substituting omega-3 fish oil lipid emulsion in parenteral nutrition for the standard soybean-based emulsions may prevent the development of TPN-related cholestasis and reverse the effects of established liver disease. A phase 2 trial to determine whether


Surgery_Schwartz

Surgery_Schwartz. nutrition for the standard soybean-based emulsions may prevent the development of TPN-related cholestasis and reverse the effects of established liver disease. A phase 2 trial to determine whether parenteral nutrition–associated liver disease can be reversed or its progres-sion halted by using a parenteral fat emulsion prepared from fish oil as measured by normalization of serum levels of hepatic enzymes and bilirubin is ongoing (ClinicalTrials.gov, identifier NCT00826020).Venous AccessObtaining reliable vascular access in an infant or child is an important task that often becomes the responsibility of the pedi-atric surgeon. The goal should always be to place the catheter in the least invasive, least risky, and least painful manner, and in a location that is most accessible and allows for use of the catheter without complications for as long as it is needed. In infants, cen-tral venous access may be established using a cutdown approach, either in the antecubital fossa, external


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Surgery_Schwartz. for use of the catheter without complications for as long as it is needed. In infants, cen-tral venous access may be established using a cutdown approach, either in the antecubital fossa, external jugular vein, facial vein, or proximal saphenous vein. If the internal jugular vein is used, care is taken to prevent venous occlusion. In infants over 3 kg and in older children, percutaneous access of the subclavian, internal jugular, or femoral veins is possible in most cases, and central access is achieved using the Seldinger technique. The use of ultrasound (US) is considered standard of care for placement of central lines in this population for the internal jugular vein and femoral veins, and it significantly improves the safety of the insertion procedure. The catheters are tunneled to an exit site separate from the venotomy site. Where available, PICC lines (peripherally inserted central catheters) may be placed, typically via the antecubital fossa. Regardless of whether the catheter


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Surgery_Schwartz. exit site separate from the venotomy site. Where available, PICC lines (peripherally inserted central catheters) may be placed, typically via the antecubital fossa. Regardless of whether the catheter is placed by a cutdown approach or percutaneously, a chest X-ray to confirm central location of the catheter tip and to exclude the presence of a pneumothorax or hemothorax is mandatory. When discussing the placement of central venous catheters with par-ents, it is important to note that the complication rate for central venous lines in children can be high. The incidence of catheter-related sepsis or infection remains a problem, yet should be less than 1% with meticulous attention to catheter insertion care and exit site management. Superior or inferior vena caval occlusion is a significant risk after the placement of multiple lines, particu-larly in the smallest premature patients.ThermoregulationCareful regulation of the ambient environment of infants and children is crucial as these


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Surgery_Schwartz. risk after the placement of multiple lines, particu-larly in the smallest premature patients.ThermoregulationCareful regulation of the ambient environment of infants and children is crucial as these patients are extremely thermolabile. Premature infants are particularly susceptible to changes in envi-ronmental temperature. Because they are unable to shiver and lack stores of fat, their potential for thermogenesis is impaired. The innate inability to regulate temperature is compounded by the administration of anesthetic and paralyzing agents. Since these patients lack adaptive mechanisms to cope with the envi-ronment, the environment must be carefully regulated. Attention to heat conservation during transport of the infant to and from the operating room is essential. Transport systems incorporating heating units are necessary for premature infants. In the operat-ing room, the infant is kept warm by the use of overhead heat-ing lamps, a heating blanket, warming of inspired gases, and


Surgery_Schwartz

Surgery_Schwartz. heating units are necessary for premature infants. In the operat-ing room, the infant is kept warm by the use of overhead heat-ing lamps, a heating blanket, warming of inspired gases, and coverage of the extremities and head with occlusive materials. During abdominal surgery, extreme care is taken to avoid wet and cold drapes. All fluids used to irrigate the chest or abdomen must be warmed to body temperature. Laparoscopic approaches for abdominal operations may result in more stable thermoregu-lation due to decreased heat loss from the smaller wound size. Constant monitoring of the child’s temperature is critical in a lengthy procedure, and the surgeon should continuously com-municate with the anesthesiologist regarding the temperature of the patient. The development of hypothermia in infants and chil-dren can result in cardiac arrhythmias or coagulopathy. These potentially life-threatening complications can be avoided by careful attention to


Surgery_Schwartz

Surgery_Schwartz. The development of hypothermia in infants and chil-dren can result in cardiac arrhythmias or coagulopathy. These potentially life-threatening complications can be avoided by careful attention to thermoregulation.Brunicardi_Ch39_p1705-p1758.indd 170912/02/19 11:26 AM 1710SPECIFIC CONSIDERATIONSPART IIPain ControlAll children including neonates experience pain; the careful recognition and management of pediatric pain represents an important component of the perioperative management of all pediatric surgical patients. There is a range of pain manage-ment options that can improve the child’s well-being, as well as the parents’ sense of comfort. Given that morphine and fentanyl have an acceptable safety margin, they should be administered to neonates and children when indicated, bear-ing in mind that withholding analgesia poses a significant risk, as does administration of excessive analgesic agents. A recent randomized trial of neonates on ventilators showed that the use of a morphine


Surgery_Schwartz

Surgery_Schwartz. mind that withholding analgesia poses a significant risk, as does administration of excessive analgesic agents. A recent randomized trial of neonates on ventilators showed that the use of a morphine infusion decreased the incidence of intraventricular hemorrhage by 50%. Additional analge-sic modalities include the use of topical anesthetic ointment (EMLA cream) and the use of regional anesthesia, such as caudal blocks for hernias and epidural or incisional catheter infusions (On-Q) for large abdominal or thoracic incisions. In surgical neonates that have been administered large con-centrations of narcotics over a prolonged period, transient physical dependence should not only be expected but also anticipated. When narcotics are discontinued, symptoms of narcotic withdrawal may develop, including irritability, rest-lessness, and episodes of hypertension and tachycardia. Early recognition of these signs is essential, as is timely treatment using nalaxone and other agents. It is


Surgery_Schwartz

Surgery_Schwartz. including irritability, rest-lessness, and episodes of hypertension and tachycardia. Early recognition of these signs is essential, as is timely treatment using nalaxone and other agents. It is important to admin-ister pain control in concert with a well-qualified and col-laborative pediatric pain-management team, which typically includes anesthesiologists with expertise in pain management, as well as advance practice nurses who can respond rapidly when the pain control is inadequate or excessive. By ensuring that the pediatric surgical patient has adequate analgesia, the surgeon ensures that the patient receives the most humane and thorough treatment and provides important reassurance to all other members of the healthcare team and to the family that pain control is a very high priority.NECK MASSESThe management of neck masses in children is determined by their location and the length of time that they have been pres-ent. Neck lesions are found either in the midline or lateral


Surgery_Schwartz

Surgery_Schwartz. MASSESThe management of neck masses in children is determined by their location and the length of time that they have been pres-ent. Neck lesions are found either in the midline or lateral com-partments. Midline masses include thyroglossal duct remnants, thyroid masses, thymic cysts, or dermoid cysts. Lateral lesions include branchial cleft remnants, cystic hygromas, vascular mal-formations, salivary gland tumors, torticollis, and lipoblastoma (a rare benign mesenchymal tumor of embryonal fat occurring in infants and young children). Enlarged lymph nodes and rare malignancies such as rhabdomyosarcoma can occur either in the midline or laterally.LymphadenopathyThe most common cause of a neck mass in a child is an enlarged lymph node, which typically can be found laterally or in the midline. The patient is usually referred to the pedi-atric surgeon for evaluation after the mass has been present for several weeks. A detailed history and physical examination often helps determine the


Surgery_Schwartz

Surgery_Schwartz. The patient is usually referred to the pedi-atric surgeon for evaluation after the mass has been present for several weeks. A detailed history and physical examination often helps determine the likely etiology of the lymph node and the need for excisional biopsy. Enlarged tender lymph nodes are usually the result of a bacterial infection (Staphy-lococcus or Streptococcus). Treatment of the primary cause (e.g., otitis media or pharyngitis) with antibiotics often is all that is necessary. However, when the involved nodes become fluctuant, incision and drainage are indicated. In many North American institutions, there has been an increasing prevalence of methicillin-resistant Staphylococcus aureus infection of the skin and soft tissues, leading to increased staphylococcal lymphadenitis in children. More chronic forms of lymphadeni-tis, including infections with atypical mycobacteria, as well as cat-scratch fever, are diagnosed based on serologic findings or excisional biopsy. The


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Surgery_Schwartz. in children. More chronic forms of lymphadeni-tis, including infections with atypical mycobacteria, as well as cat-scratch fever, are diagnosed based on serologic findings or excisional biopsy. The lymphadenopathy associated with infectious mononucleosis can be diagnosed based on serology. When the neck nodes are firm, fixed, and others are also pres-ent in the axillae or groin, or the history suggests lymphoma, excisional biopsy is indicated. In these cases, it is essential to obtain a chest radiograph to look for the presence of a medias-tinal mass. Significant mediastinal load portends cardiorespira-tory collapse due to loss of venous return and compression of the tracheobronchial tree with general anesthesia.Thyroglossal Duct RemnantsPathology and Clinical Manifestations. The thyroid gland buds off the foregut diverticulum at the base of the tongue in the region of the future foramen cecum at 3 weeks of embryonic life. As the fetal neck develops, the thyroid tissue becomes more


Surgery_Schwartz

Surgery_Schwartz. gland buds off the foregut diverticulum at the base of the tongue in the region of the future foramen cecum at 3 weeks of embryonic life. As the fetal neck develops, the thyroid tissue becomes more anterior and caudad until it rests in its normal position. The “descent” of the thyroid is intimately connected with the development of the hyoid bone. Residual thyroid tis-sue left behind during the migration may persist and subse-quently present in the midline of the neck as a thyroglossal duct cyst. The mass is most commonly appreciated in the 2to 4-year-old child when the baby fat disappears and irregulari-ties in the neck become more readily apparent. Usually the cyst is encountered in the midline at or below the level of the hyoid bone and moves up and down with swallowing or with protrusion of the tongue. Occasionally it presents as an intrathyroidal mass. Most thyroglossal duct cysts are asymp-tomatic. If the duct retains its connection with the pharynx, infection may occur, and the


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Surgery_Schwartz. of the tongue. Occasionally it presents as an intrathyroidal mass. Most thyroglossal duct cysts are asymp-tomatic. If the duct retains its connection with the pharynx, infection may occur, and the resulting abscess will necessitate incision and drainage, occasionally resulting in a salivary fis-tula. Submental lymphadenopathy and midline dermoid cysts can be confused with a thyroglossal duct cyst. Rarely, midline ectopic thyroid tissue masquerades as a thyroglossal duct cyst and may represent the patient’s only thyroid tissue. Therefore, if there is any question regarding the diagnosis or if the thyroid gland cannot be palpated in its normal anatomic position, it is advisable to obtain a nuclear scan to confirm the presence of a normal thyroid gland. Although rarely the case in children, in adults the thyroglossal duct may contain thyroid tissue that can undergo malignant degeneration. The presence of malignancy in a thyroglossal cyst should be suspected when the cyst grows rapidly or


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Surgery_Schwartz. adults the thyroglossal duct may contain thyroid tissue that can undergo malignant degeneration. The presence of malignancy in a thyroglossal cyst should be suspected when the cyst grows rapidly or when US demonstrates a complex anechoic pattern or the presence of calcification.Treatment. If the thyroglossal duct cyst presents with an abscess, treatment should first consist of drainage and antibiot-ics. Following resolution of the inflammation, resection of the cyst in continuity with the central portion of the hyoid bone and the tract connecting to the pharynx in addition to ligation at the foramen cecum (the Sistrunk operation), is curative in over 90% of patients. Lesser operations result in unacceptably high recur-rence rates, and recurrence is more frequent following infection. According to a recent review, factors predictive of recurrence included more than two infections prior to surgery, age under 2 years, and inadequate initial operation.Brunicardi_Ch39_p1705-p1758.indd


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Surgery_Schwartz. to a recent review, factors predictive of recurrence included more than two infections prior to surgery, age under 2 years, and inadequate initial operation.Brunicardi_Ch39_p1705-p1758.indd 171012/02/19 11:26 AM 1711PEDIATRIC SURGERYCHAPTER 39Branchial Cleft AnomaliesPaired branchial clefts and arches develop early in the fourth gestational week. The first cleft and the first, second, third, and fourth pouches give rise to adult organs. The embryologic com-munication between the pharynx and the external surface may persist as a fistula. A fistula is seen most commonly with the second branchial cleft, which normally disappears, and extends from the anterior border of the sternocleidomastoid muscle superiorly, inward through the bifurcation of the carotid artery, and enters the posterolateral pharynx just below the tonsillar fossa. In contrast, a third branchial cleft fistula passes posterior to the carotid bifurcation. The branchial cleft remnants may con-tain small pieces of


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Surgery_Schwartz. pharynx just below the tonsillar fossa. In contrast, a third branchial cleft fistula passes posterior to the carotid bifurcation. The branchial cleft remnants may con-tain small pieces of cartilage and cysts, but internal fistulas are rare. A second branchial cleft sinus is suspected when clear fluid is noted draining from the external opening of the tract at the anterior border of the lower third of the sternomastoid muscle. Rarely, branchial cleft anomalies occur in association with bili-ary atresia and congenital cardiac anomalies, an association that is referred to as Goldenhar’s complex.Treatment. Complete excision of the cyst and sinus tract is necessary for cure. Dissection of the sinus tract is facilitated with passage of a fine lacrimal duct probe through the external opening into the tract and utilizing it as a guide for dissection. Injection of a small amount of methylene blue dye into the tract also may be useful. A series of two or sometimes three small transverse


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Surgery_Schwartz. into the tract and utilizing it as a guide for dissection. Injection of a small amount of methylene blue dye into the tract also may be useful. A series of two or sometimes three small transverse incisions in a “stepladder” fashion is preferred to a long oblique incision in the neck, which is cosmetically unde-sirable. Branchial cleft cysts can present as abscesses. In these cases, initial treatment includes incision and drainage with a course of antibiotics to cover Staphylococcus and Streptococ-cus species, followed by excision of the cyst after the infection resolves.Lymphatic MalformationEtiology and Pathology. Lymphatic malformation (cystic hygroma or lymphangioma) occurs as a result of sequestration or obstruction of developing lymph vessels in approximately 1 in 12,000 births. Although the lesion can occur anywhere, the most common sites are in the posterior triangle of the neck, axilla, groin, and mediastinum. The cysts are lined by endo-thelium and filled with lymph.


Surgery_Schwartz

Surgery_Schwartz. Although the lesion can occur anywhere, the most common sites are in the posterior triangle of the neck, axilla, groin, and mediastinum. The cysts are lined by endo-thelium and filled with lymph. Occasionally unilocular cysts occur, but more often there are multiple cysts “infiltrating” the surrounding structures and distorting the local anatomy. A particularly troublesome variant of lymphatic malformation is that which involves the tongue, floor of the mouth, and struc-tures deep in the neck. Adjacent connective tissue may show extensive lymphocytic infiltration. The mass may be apparent at birth or may appear and enlarge rapidly in the early weeks or months of life as lymph accumulates; most present by age 2 years (Fig. 39-1A). Extension of the lesion into the axilla or mediastinum occurs about 10% of the time and can be demon-strated preoperatively by chest X-ray, US, or computed tomo-graphic (CT) scan, although magnetic resonance imaging (MRI) is preferable. Occasionally lymphatic


Surgery_Schwartz

Surgery_Schwartz. about 10% of the time and can be demon-strated preoperatively by chest X-ray, US, or computed tomo-graphic (CT) scan, although magnetic resonance imaging (MRI) is preferable. Occasionally lymphatic malformations contain nests of vascular tissue. These poorly supported vessels may bleed and produce rapid enlargement and discoloration of the lesion. Infection within the lymphatic malformations, usually caused by Streptococcus or Staphylococcus, may occur. In the neck, this can cause rapid enlargement, which may result in airway compromise. Rarely, it may be necessary to carry out percutaneous aspiration of a cyst to relieve respiratory distress.The diagnosis of lymphatic malformation by prenatal US, before 30 weeks’ gestation, has detected a “hidden mortality” as well as a high incidence of associated anomalies, including abnormal karyotypes and hydrops fetalis. Occasionally, very large lesions can cause obstruction of the fetal airway. Such obstruction can result in the development of


Surgery_Schwartz

Surgery_Schwartz. associated anomalies, including abnormal karyotypes and hydrops fetalis. Occasionally, very large lesions can cause obstruction of the fetal airway. Such obstruction can result in the development of polyhydramnios by impairing the ability of the fetus to swallow amniotic fluid. In these circumstances, the airway is usually markedly distorted, which can result in immediate airway obstruction unless the air-way is secured at the time of delivery. Orotracheal intubation or emergency tracheostomy while the infant remains attached to the placenta, the so-called EXIT procedure (ex utero intrapar-tum technique) may be necessary to secure the airway.Treatment. The modern management of most lymphatic malformations includes image-guided sclerotherapy as first-line therapy, which often involves multiple injections. Cyst excision may be used in cases where injection is inadequate. BAFigure 39-1. A. Left cervical cystic hygroma in a 2-day old baby. B. Intraoperative photograph showing a vessel


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Surgery_Schwartz. injections. Cyst excision may be used in cases where injection is inadequate. BAFigure 39-1. A. Left cervical cystic hygroma in a 2-day old baby. B. Intraoperative photograph showing a vessel loop around the spinal accessory nerve.Brunicardi_Ch39_p1705-p1758.indd 171112/02/19 11:26 AM 1712SPECIFIC CONSIDERATIONSPART IIFigure 39-2. Prenatal ultrasound of a fetus with a congenital dia-phragmatic hernia. Arrows point to the location of the diaphragm. Arrowhead points to the stomach, which is in the thoracic cavity.Total removal of all gross disease is often not possible because of the extent of the lymphatic malformation and its proximity to, and intimate relationship with, adjacent nerves, muscles, and blood vessels (Fig. 39-1B). Radical ablative surgery is not indicated for these lesions, which are always benign. Conservative excision and unroofing of remaining cysts is advised, with repeated partial excision of residual cysts and sclerotherapy if necessary, preserving all


Surgery_Schwartz

Surgery_Schwartz. lesions, which are always benign. Conservative excision and unroofing of remaining cysts is advised, with repeated partial excision of residual cysts and sclerotherapy if necessary, preserving all adjacent crucial structures. In cases in which surgical excision is performed, closed-suction drainage is recommended. Nevertheless, fluid may accumulate beneath the surgically created flaps in the area from which the lymphatic malformation was excised, requiring multiple needle aspirations. A combined sclerotherapy/resectional approach is particularly useful for masses that extend to the base of the tongue or the floor of the mouth.TorticollisThe presence of a lateral neck mass in infancy in association with rotation of the head towards the opposite side of the mass indicates the presence of congenital torticollis. This lesion results from fibrosis of the sternocleidomastoid muscle. The mass may be palpated in the affected muscle in approximately two-thirds of cases, or it may be diagnosed


Surgery_Schwartz

Surgery_Schwartz. torticollis. This lesion results from fibrosis of the sternocleidomastoid muscle. The mass may be palpated in the affected muscle in approximately two-thirds of cases, or it may be diagnosed by US. Histologi-cally, the lesion is characterized by the deposition of collagen and fibroblasts around atrophied muscle cells. In the vast major-ity of cases, physical therapy based on passive stretching of the affected muscle is of benefit. Rarely, surgical transection of the sternocleidomastoid may be indicated.RESPIRATORY SYSTEMCongenital Diaphragmatic Hernia (Bochdalek)Pathology. The septum transversum extends to divide the pleural and coelomic cavities during fetal development. This precursor of the diaphragm normally completes separation of these two cavities at the posterolateral aspects of this mesen-chymally derived structure. The most common variant of a congenital diaphragmatic hernia is a posterolateral defect, also known as a Bochdalek hernia. Diaphragmatic defects allow abdominal


Surgery_Schwartz

Surgery_Schwartz. this mesen-chymally derived structure. The most common variant of a congenital diaphragmatic hernia is a posterolateral defect, also known as a Bochdalek hernia. Diaphragmatic defects allow abdominal viscera to fill the chest cavity. The abdominal cav-ity is small and underdeveloped and remains scaphoid after birth. Both lungs are hypoplastic, with decreased bronchial and pulmonary artery branching. Lung weight, lung volume, and DNA content are also decreased, and these findings are more striking on the ipsilateral side. This anomaly is encountered more commonly on the left (80–90%). Linkage analyses have recently implicated genetic mutations in syndromic variants of congenital diaphragmatic hernias. In many instances, there is a surfactant deficiency, which compounds the degree of respira-tory insufficiency. Amniocentesis with karyotype may identify chromosomal defects, especially trisomy 18 and 21. Associated anomalies, once thought to be uncommon, were identified in 65 of 166


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Surgery_Schwartz. insufficiency. Amniocentesis with karyotype may identify chromosomal defects, especially trisomy 18 and 21. Associated anomalies, once thought to be uncommon, were identified in 65 of 166 patients in one study, predominately of the heart, fol-lowed by abdominal wall defects, chromosomal changes, and other defects.Prenatal ultrasonography is successful in making the diag-nosis of congenital diaphragmatic hernia (CDH) as early as 15 weeks’ gestation, and early antenatal diagnosis is associated with worse outcomes. US findings include herniated abdominal viscera in the chest that may also look like a mass or lung anom-aly, changes in liver position, and mediastinal shift away from the herniated viscera (Fig. 39-2). Accurate prenatal prediction of outcome for fetuses who have CDH remains a challenge. One index of severity for patients with left CDH is the lung-to-head ratio (LHR), which is the product of the length and the width of the right lung at the level of the cardiac atria divided


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Surgery_Schwartz. One index of severity for patients with left CDH is the lung-to-head ratio (LHR), which is the product of the length and the width of the right lung at the level of the cardiac atria divided by the head circumference (all measurements in millimeters). An LHR value of less than 1.0 is associated with a very poor prognosis, whereas an LHR greater than 1.4 predicts a more favorable outcome. The utility of the LHR in predicting outcome in patients with CDH has recently been questioned because of the tremendous interobserver variability in calculating this ratio for a par-ticular patient, as well as the lack of reliable measures to deter-mine postnatal disease severity. Because the LHR is not gestational age independent, Jani and colleagues proposed the introduction of a new measurement: the observed to expected (o/e) LHR, to correct for gestational age. The observed LHR may be expressed as a percentage of the expected mean for ges-tational age of the observed/expected lung-to-head ratio


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Surgery_Schwartz. observed to expected (o/e) LHR, to correct for gestational age. The observed LHR may be expressed as a percentage of the expected mean for ges-tational age of the observed/expected lung-to-head ratio (o/e LHR), which is considered extreme if <15%, severe at 15% to 25%, moderate at 26% to 35%, and mild at 36% to 45%. The most reliable prenatal predictor of postnatal survival is absence of liver herniation, where in 710 fetuses, there was significantly higher survival rate in fetuses without herniation (74% without herniation vs. 45% with herniation).Following delivery, the diagnosis of CDH is made by CXR (Fig. 39-3). The differential diagnosis includes broncho-pulmonary foregut malformations, in which the intrathoracic loops of bowel may be confused for lung or foregut pathol-ogy. The vast majority of infants with CDH develop immedi-ate respiratory distress, which is due to the combined effects of three factors. First, the air-filled bowel in the chest compresses the mobile


Surgery_Schwartz

Surgery_Schwartz. The vast majority of infants with CDH develop immedi-ate respiratory distress, which is due to the combined effects of three factors. First, the air-filled bowel in the chest compresses the mobile mediastinum, which shifts to the opposite side of the chest, compromising air exchange in the contralateral lung. Second, pulmonary hypertension develops. This phenomenon results in persistent fetal circulation with resultant decreased pulmonary perfusion and impaired gas exchange. Finally, the lung on the affected side is often hypoplastic, such that it is essentially nonfunctional. Varying degrees of pulmonary hypo-plasia on the opposite side may compound these effects. The second and third factors are thought to be the most important. Neonates with CDH are usually in respiratory distress requiring 1Brunicardi_Ch39_p1705-p1758.indd 171212/02/19 11:26 AM 1713PEDIATRIC SURGERYCHAPTER 39Figure 39-3. Chest X-ray showing a left congenital diaphragmatic hernia.ventilation and intensive care,


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Surgery_Schwartz. 1Brunicardi_Ch39_p1705-p1758.indd 171212/02/19 11:26 AM 1713PEDIATRIC SURGERYCHAPTER 39Figure 39-3. Chest X-ray showing a left congenital diaphragmatic hernia.ventilation and intensive care, and the overall mortality in most series is around 50%.Treatment. CDH care has been improved through effective use of improved methods of ventilation and timely cannula-tion for extracorporeal membrane oxygenation (ECMO). Many infants are symptomatic at birth due to hypoxia, hypercarbia, and metabolic acidosis. Prompt cardiorespiratory stabilization is mandatory. It is noteworthy that the first 24 to 48 hours after birth are often characterized by a period of relative stability with high levels of PaO2 and relatively good perfusion. This has been termed the “honeymoon period” and is often followed by progressive cardiorespiratory deterioration. In the past, cor-rection of the hernia was believed to be a surgical emergency, and patients underwent surgery shortly after birth. It is now accepted


Surgery_Schwartz

Surgery_Schwartz. by progressive cardiorespiratory deterioration. In the past, cor-rection of the hernia was believed to be a surgical emergency, and patients underwent surgery shortly after birth. It is now accepted that the presence of persistent pulmonary hyperten-sion that results in right-to-left shunting across the open fora-men ovale or the ductus arteriosus, and the degree of pulmonary hypoplasia, are the leading causes of cardiorespiratory insuffi-ciency. Current management therefore is directed toward man-aging the pulmonary hypertension, and minimizing barotrauma while optimizing oxygen delivery. To achieve this goal, infants are placed on mechanical ventilation using relatively low or “gentle” settings that prevent overinflation of the noninvolved lung. Levels of PaCO2 in the range of 50 to 60 mmHg or higher are accepted as long as the pH remains ≥7.25. If these objec-tives cannot be achieved using conventional ventilation, high frequency oscillatory ventilation (HFOV) may be employed to


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Surgery_Schwartz. mmHg or higher are accepted as long as the pH remains ≥7.25. If these objec-tives cannot be achieved using conventional ventilation, high frequency oscillatory ventilation (HFOV) may be employed to avoid the injurious effects of conventional tidal volume venti-lation. Echocardiography will assess the degree of pulmonary hypertension and identify the presence of any coexisting cardiac anomaly. ICU goals include minimal sedation, meticulous atten-tion to endotracheal tube secretions, and gradual changes to ven-tilator settings to avoid inducing pulmonary hypertension via hypoxia. To minimize the degree of pulmonary hypertension, inhaled nitric oxide may be administered, and in some patients, this improves pulmonary perfusion. Nitric oxide is administered into the ventilation circuit and is used in concentrations up to 40 parts per million. Correction of acidosis using bicarbonate solution may minimize the degree of pulmonary hypertension. As the degree of pulmonary hypertension becomes


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Surgery_Schwartz. used in concentrations up to 40 parts per million. Correction of acidosis using bicarbonate solution may minimize the degree of pulmonary hypertension. As the degree of pulmonary hypertension becomes hemody-namically significant, right-sided heart failure develops, and systemic perfusion is impaired. Administration of excess IV fluid will compound the degree of cardiac failure and lead to marked peripheral edema. Inotropic support using epinephrine, dopamine, and milrinone alone or in combination may be useful in optimizing cardiac contractility and maintaining mean arterial pressure.Infants with CDH who remain severely hypoxic despite maximal ventilatory care may be candidates for treatment of their respiratory failure ECMO, with access via venovenous (VV) or venoarterial (VA) routes. VV bypass is established with a single cannula through the right internal jugular vein, with blood removed from and infused into the right atrium by separate ports. VA bypass provides additional cardiac


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Surgery_Schwartz. bypass is established with a single cannula through the right internal jugular vein, with blood removed from and infused into the right atrium by separate ports. VA bypass provides additional cardiac support, whereas VV bypass requires a well-functioning heart and relies on the lungs for some oxygenation as well. In VA ECMO, the right atrium is cannulated by means of the internal jugular vein and the aortic arch through the right common carotid artery. As much of the cardiac output is directed through the membrane oxygenator as is necessary to provide oxygenated blood to the infant and remove carbon dioxide. The infant is maintained on bypass until the pulmonary hypertension is resolved and lung function, as measured by compliance and the ability to oxy-genate and ventilate, is improved. This is usually seen within 7 to 10 days, but in some infants, it may take up several weeks to occur. Complications associated with ECMO increase after 14 days and include cannula malposition,


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Surgery_Schwartz. This is usually seen within 7 to 10 days, but in some infants, it may take up several weeks to occur. Complications associated with ECMO increase after 14 days and include cannula malposition, bleeding in multiple locations, and infection. The use of ECMO is associated with significant risk. Because patients require systemic anticoagu-lation, bleeding complications are the most significant. They may occur intracranially or at the site of cannula insertion, and they can be life-threatening. Systemic sepsis is a significant problem and may necessitate decannulation. Criteria for plac-ing infants on ECMO include the presence of normal cardiac anatomy by echocardiography, the absence of fatal chromosome anomalies, and the expectation that the infant would die with-out ECMO. Traditionally, a threshold of weight greater than 2 kg and gestational age greater than 34 weeks has been applied, although success has been achieved at weights as low as 1.8 kg. Upon decannulation, some centers


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Surgery_Schwartz. a threshold of weight greater than 2 kg and gestational age greater than 34 weeks has been applied, although success has been achieved at weights as low as 1.8 kg. Upon decannulation, some centers repair the carotid artery. In instances in which the child is cannulated for a brief period (5 days or less) this may be feasible. A recent study failed to show any benefit from repairing the carotid artery, although this finding remains to be studied further.A strategy that does not involve the use of ECMO but instead emphasizes the use of permissive hypercapnia and the avoidance of barotrauma may provide equal overall outcome in patients with CDH. This likely reflects the fact that mortality is related to the degree of pulmonary hypoplasia and the pres-ence of congenital anomalies, neither of which are correctable by ECMO.Brunicardi_Ch39_p1705-p1758.indd 171312/02/19 11:26 AM 1714SPECIFIC CONSIDERATIONSPART IIFigure 39-4. Congenital lobar emphysema of the left upper lobe in a


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Surgery_Schwartz. of which are correctable by ECMO.Brunicardi_Ch39_p1705-p1758.indd 171312/02/19 11:26 AM 1714SPECIFIC CONSIDERATIONSPART IIFigure 39-4. Congenital lobar emphysema of the left upper lobe in a 2-week-old boy. Mediastinal shift is present.The timing of diaphragmatic hernia repair still varies from center to center, particularly when the infant is on ECMO. In patients that are not on ECMO, repair should be performed once the hemodynamic status has been optimized. In neonates that are on ECMO, some surgeons perform early repair on bypass; oth-ers wait until the infant’s lungs are improved and the pulmonary hypertension has subsided and then repair the diaphragm and discontinue bypass within hours of surgery. Still others repair the diaphragm only after the infant is off bypass. Operative repair of the diaphragmatic hernia may be accomplished either by an abdominal or transthoracic approach and can be performed either via open or minimally invasive techniques. Through a subcostal


Surgery_Schwartz

Surgery_Schwartz. repair of the diaphragmatic hernia may be accomplished either by an abdominal or transthoracic approach and can be performed either via open or minimally invasive techniques. Through a subcostal incision the abdominal viscera are withdrawn from the chest, exposing the defect in the diaphragm. Care must be taken when reducing the spleen and liver, as bleeding from these structures can be fatal. The anterior margin is often apparent, while the posterior muscular rim is attenuated. If the infant is heparinized on bypass, minimal dissection of the muscular margins is per-formed. Electrocautery is used liberally to minimize postopera-tive bleeding. Most infants who require ECMO support prior to hernia repair have large defects, often lacking the medial and posterior margins. About three-fourths of infants repaired on bypass require prosthetic material to patch the defect, suturing it to the diaphragmatic remnant or around ribs or costal cartilages for the large defects. If there is


Surgery_Schwartz

Surgery_Schwartz. of infants repaired on bypass require prosthetic material to patch the defect, suturing it to the diaphragmatic remnant or around ribs or costal cartilages for the large defects. If there is adequate muscle for closure, a single layer of nonabsorbable horizontal mattress suture, pled-geted or not, closes the defect. Just before the repair is complete, a chest tube may be positioned in the thoracic cavity but is not mandatory. Patients repaired on ECMO are at risk for develop-ing a hemothorax, which can significantly impair ventilation. Anatomic closure of the abdominal wall may be impossible after reduction of the viscera. Occasionally, a prosthetic patch or acellular material may be sutured to the fascia to facilitate closure. The patch can be removed at a later time, and the ventral hernia can be closed at that time or subsequently. In patients who are deemed to be candidates for a minimally invasive approach (stable patients, >2 kg, no pulmonary hypertension), a thoraco-scopic


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Surgery_Schwartz. hernia can be closed at that time or subsequently. In patients who are deemed to be candidates for a minimally invasive approach (stable patients, >2 kg, no pulmonary hypertension), a thoraco-scopic repair may be safely performed although concerns have been raised about possible effects of the longer operative time for thoracoscopic repair and higher recurrence rates. If the dia-phragm has been repaired on ECMO, weaning and decannulation are accomplished as soon as possible. All infants are ventilated postoperatively to maintain preductal arterial oxygenation of 80 to 100 torr. Very slow weaning from the ventilator is necessary to avoid recurrent pulmonary hypertension.Fetal tracheal occlusion is an experimental prenatal ther-apy for the treatment of severe congenital diaphragmatic hernia that reverses lung hypoplasia. The rationale for this approach is that the occlusion of the fetal trachea leads to net accumula-tion of lung liquid under pressure, which results in the develop-ment


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Surgery_Schwartz. that reverses lung hypoplasia. The rationale for this approach is that the occlusion of the fetal trachea leads to net accumula-tion of lung liquid under pressure, which results in the develop-ment of large fluid-filled lungs. The balloon may be placed into the trachea under laparoscopic guidance, then removed prior to delivery when maximal lung growth has been achieved. The use of fetal tracheal occlusion remains investigational, although early reports are promising.Congenital Lobar EmphysemaCongenital lobar emphysema (CLE) is a condition manifested during the first few months of life as a progressive hyperexpan-sion of one or more lobes of the lung. It can be life-threatening in the newborn period if extensive lung tissue is involved, but in the older infant and in cases in which the lesion is less severely distended it causes less respiratory distress. Air entering during inspiration is trapped in the lobe; on expiration, the lobe can-not deflate and progressively overexpands,


Surgery_Schwartz

Surgery_Schwartz. lesion is less severely distended it causes less respiratory distress. Air entering during inspiration is trapped in the lobe; on expiration, the lobe can-not deflate and progressively overexpands, causing atelectasis of the adjacent lobe or lobes. This hyperexpansion eventually shifts the mediastinum to the opposite side and compromises the other lung. CLE usually occurs in the upper lobes of the lung (left greater than right), followed next in frequency by the right middle lobe, but it also can occur in the lower lobes. It is caused by intrinsic bronchial obstruction from poor bronchial cartilage development or extrinsic compression. Approximately 14% of children with this condition have cardiac defects, with an enlarged left atrium or a major vessel causing compression of the ipsilateral bronchus.Symptoms range from mild respiratory distress to full-fledged respiratory failure with tachypnea, dyspnea, cough, and late cyanosis. These symptoms may be stationary or they may progress


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Surgery_Schwartz. bronchus.Symptoms range from mild respiratory distress to full-fledged respiratory failure with tachypnea, dyspnea, cough, and late cyanosis. These symptoms may be stationary or they may progress rapidly or result in recurrent pneumonia. Occasionally, infants with CLE present with failure to thrive, which likely reflects the increased work associated with the overexpanded lung. A hyperexpanded hemithorax on the ipsilateral side is pathogneumonic for CLE. Diagnosis is typically confirmed by chest X-ray that shows a hyperlucent affected lobe with adja-cent lobar compression and atelectasis. The mediastinum may be shifted as a consequence of mass effect to the contralateral side causing compression and atelectasis of the contralateral lung (Fig. 39-4). Although chest radiograph is usually sufficient, it is sometimes important to obtain at CT scan of the chest to clearly establish the diagnosis of CLE. This should be done only in the stable patient. Unless foreign body or mucous plugging


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Surgery_Schwartz. it is sometimes important to obtain at CT scan of the chest to clearly establish the diagnosis of CLE. This should be done only in the stable patient. Unless foreign body or mucous plugging is suspected as a cause of hyperinflation, bronchoscopy is not advisable because it can lead to more air trapping and cause life-threatening respiratory distress in a stable infant. Treatment is resection of the affected lobe, which can be safely performed using either an open or thoracoscopic approach. Unless symp-toms necessitate earlier surgery, resection can usually be per-formed after the infant is several months of age. The prognosis is excellent.Brunicardi_Ch39_p1705-p1758.indd 171412/02/19 11:26 AM 1715PEDIATRIC SURGERYCHAPTER 39Figure 39-5. Computed tomography scan of the chest showing a congenital cystic adenomatoid malformation of the left lower lobe.Figure 39-6. Intraoperative photograph showing left lower lobe congenital cystic adenomatoid malformation seen in Fig.


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Surgery_Schwartz. showing a congenital cystic adenomatoid malformation of the left lower lobe.Figure 39-6. Intraoperative photograph showing left lower lobe congenital cystic adenomatoid malformation seen in Fig. 39-5.Bronchopulmonary Foregut MalformationsBronchopulmonary foregut malformations include foregut duplication cysts, congenital pulmonary airway malformations, and pulmonary sequestrations as discussed in the following sections.Congenital Pulmonary Airway Malformations. Previ-ously denoted as congenital cystic adenomatous malformation, (CCAM), congenital pulmonary airway malformations (CPAM) exhibits cystic proliferation of the terminal airway, producing cysts lined by mucus-producing respiratory epithelium, and elastic tissue in the cyst walls without cartilage formation. There may be a single cyst with a wall of connective tissue contain-ing smooth muscle. Cysts may be large and multiple (type I), smaller and more numerous (type II), or they may resemble fetal lung without macroscopic cysts


Surgery_Schwartz

Surgery_Schwartz. with a wall of connective tissue contain-ing smooth muscle. Cysts may be large and multiple (type I), smaller and more numerous (type II), or they may resemble fetal lung without macroscopic cysts (type III). CPAMs frequently occur in the left lower lobe. However, this lesion can occur in any location and may occur in more than one lobe on more than one side, although this is rare. Clinical symptoms range from none to severe respiratory failure at birth. Over time, these mal-formations can be subject to repeated infections and produce fever and cough in older infants and children. The diagnosis is usually confirmed by CT for surgical planning and charac-teristic features that might delineate other bronchopulmonary foregut malformations (Fig. 39-5). Prenatal US may suggest the diagnosis. Resection is curative and may need to be performed urgently in the infant with severe respiratory distress. Long term, there is a risk of malignant degeneration in unresected CPAMs, but this risk


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Surgery_Schwartz. Resection is curative and may need to be performed urgently in the infant with severe respiratory distress. Long term, there is a risk of malignant degeneration in unresected CPAMs, but this risk occurs over decades and has not been fully defined. As a result, resection of the affected lobe is usually per-formed (Fig. 39-6). Antenatal resection may be rarely indicated in those instances in which fetal development is complicated by hydrops as a result of the mechanical and vascular effects of the lung lesion.Pulmonary Sequestration. Pulmonary sequestration is uncommon and consists of a mass of lung tissue, usually in the left lower chest, occurring without the usual connections to the pulmonary artery or tracheobronchial tree, yet with a systemic blood supply from the aorta. There are two kinds of sequestra-tion. Extralobar sequestration is usually a small area of nonaer-ated lung separated from the main lung mass, with a systemic blood supply, located immediately above the left


Surgery_Schwartz

Surgery_Schwartz. two kinds of sequestra-tion. Extralobar sequestration is usually a small area of nonaer-ated lung separated from the main lung mass, with a systemic blood supply, located immediately above the left diaphragm. It is commonly found in cases of CDH. Intralobar sequestration more commonly occurs within the parenchyma of the left lower lobe but can occur on the right. There is no major connection to the tracheobronchial tree, but a secondary connection may be established, perhaps through infection or via adjacent intra-pulmonary shunts. The blood supply frequently originates from the aorta below the diaphragm; multiple vessels may be present (Fig. 39-7). Venous drainage of both types can be systemic or pulmonary. The cause of sequestration is unknown but most probably involves an abnormal budding of the developing lung that picks up a systemic blood supply and never becomes con-nected with the bronchus or pulmonary vessels. Sequestrations may, in some cases, exhibit mixed pathology with


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Surgery_Schwartz. of the developing lung that picks up a systemic blood supply and never becomes con-nected with the bronchus or pulmonary vessels. Sequestrations may, in some cases, exhibit mixed pathology with components consistent with CCAMs. Extralobar sequestration is asymptom-atic and is usually discovered incidentally on chest X-ray. If the diagnosis can be confirmed, e.g., by CT scan, resection is not necessary. Diagnosis of intralobar sequestration may be made prenatally and confirmed on postnatal CT scan. Alternatively, the diagnosis of intralobar sequestration may be established after repeated infections manifested by cough, fever, and con-solidation in the posterior basal segment of the left lower lobe. Increasingly the diagnosis is being made in the early months of life by US, and color Doppler often can be helpful in delin-eating the systemic arterial supply. Removal of the entire left lower lobe is usually necessary since the diagnosis often is made late after multiple infections.


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Surgery_Schwartz. Doppler often can be helpful in delin-eating the systemic arterial supply. Removal of the entire left lower lobe is usually necessary since the diagnosis often is made late after multiple infections. Occasionally segmental resection Figure 39-7. Arteriogram showing large systemic artery supply to intralobar sequestration of the left lower lobe.Brunicardi_Ch39_p1705-p1758.indd 171512/02/19 11:26 AM 1716SPECIFIC CONSIDERATIONSPART IIof the sequestered part of the lung can be performed using an open, or ideally, a thoracoscopic approach. If an open approach is used, it is important to open the chest through a low inter-costal space (sixth or seventh) to gain access to the vascular attachments to the aorta. These attachments may insert into the aorta below the diaphragm; in these cases, division of the ves-sels as they traverse the thoracic cavity is essential. Prognosis is generally excellent. However, failure to obtain adequate control of these vessels may result in their retraction


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Surgery_Schwartz. of the ves-sels as they traverse the thoracic cavity is essential. Prognosis is generally excellent. However, failure to obtain adequate control of these vessels may result in their retraction into the abdomen and result in uncontrollable hemorrhage. It is also possible to perform a combined thoracoscopic and open approach, wherein the vessels are clipped and divided thoracoscopically and then the lesion safely removed through a limited thoracotomy.Bronchogenic Cyst. Bronchogenic cysts are duplication cysts originating from the airway, regardless of the identity of the lining epithelial identity. They can occur anywhere along the respiratory tract and can present at any age, although typically they present after accumulation of intraluminal contents and not within the newborn period. Histologically, they are hamartoma-tous and usually consist of a single cyst lined with an epithe-lium; the mesenchyme contains cartilage and smooth muscle. They are probably embryonic rests of foregut


Surgery_Schwartz

Surgery_Schwartz. they are hamartoma-tous and usually consist of a single cyst lined with an epithe-lium; the mesenchyme contains cartilage and smooth muscle. They are probably embryonic rests of foregut origin that have been pinched off from the main portion of the developing tra-cheobronchial tree and are closely associated in causation with other foregut duplication cysts such as those arising from the esophagus. Bronchogenic cysts may be seen on prenatal US but are discovered most often incidentally on postnatal chest X-ray. Although they may be completely asymptomatic, bronchogenic cysts may produce symptoms, usually compressive, depending on the anatomic location and size, which increases over time if there is no egress for building luminal contents. In the para-tracheal region of the neck they can produce airway compres-sion and respiratory distress. In the lung parenchyma, they may become infected and present with fever and cough. In addition, they may cause obstruction of the bronchial lumen


Surgery_Schwartz

Surgery_Schwartz. produce airway compres-sion and respiratory distress. In the lung parenchyma, they may become infected and present with fever and cough. In addition, they may cause obstruction of the bronchial lumen with distal atelectasis and infection, or they may cause mediastinal com-pression. Rarely, rupture of the cyst can occur. Chest X-ray usu-ally shows a dense mass, and CT scan or MRI delineates the precise anatomic location of the lesion. Treatment consists of resection of the cyst, which may need to be undertaken in emer-gency circumstances for airway or cardiac compression. Resec-tion can be performed either as an open procedure, or more commonly using a thoracoscopic approach. If resection of a common wall will result in injury to the airway, resection of the inner epithelial cyst lining after marsupialization is acceptable.BronchiectasisBronchiectasis is an abnormal and irreversible dilatation of the bronchi and bronchioles associated with chronic suppura-tive disease of the airways.


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Surgery_Schwartz. marsupialization is acceptable.BronchiectasisBronchiectasis is an abnormal and irreversible dilatation of the bronchi and bronchioles associated with chronic suppura-tive disease of the airways. Usually patients have an underlying congenital pulmonary anomaly, cystic fibrosis, or immunologic deficiency. Bronchiectasis can also result from chronic infection secondary to a neglected bronchial foreign body. The symptoms include a chronic cough, often productive of purulent secretions, recurrent pulmonary infection, and hemoptysis. The diagnosis is suggested by a chest X-ray that shows increased bronchovas-cular markings in the affected lobe. Chest CT delineates bron-chiectasis with excellent resolution. The preferred treatment for bronchiectasis is medical, consisting of antibiotics, postural drainage, and bronchodilator therapy because many children with the disease show signs of airflow obstruction and bron-chial hyperresponsiveness. Lobectomy or segmental resection is indicated for


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Surgery_Schwartz. drainage, and bronchodilator therapy because many children with the disease show signs of airflow obstruction and bron-chial hyperresponsiveness. Lobectomy or segmental resection is indicated for localized disease that has not responded appro-priately to medical therapy. In severe cases, lung transplantation may be required to replace the terminally damaged, septic lung.Foreign BodiesThe inherent curiosity of children and their innate propensity to place new objects into their mouths to fully explore them place them at great risk for aspiration. Aspirated objects can be found either in the airway or in the esophagus; in both cases the results can be life-threatening.Airway Ingestion. Aspiration of foreign bodies most com-monly occurs in the toddler age group. Peanuts are the most common object that is aspirated, although other materials (pop-corn, for instance) may also be involved. A solid foreign body often will cause air trapping, with hyperlucency of the affected lobe or lung seen