Surgery_Schwartz

Surgery_Schwartz. of the fascia together. If an atresia is noted at the time of closure, it is prudent to reduce the bowel at the first operation and return after several weeks once the edema has resolved to correct the atresia. Intestinal function does not typically return for several weeks in patients with gastroschisis. This is especially true if the bowel is thickened and edematous. As a result, these patients will require central line placement and institution of total parenteral nutrition in order to grow. Feeding advancement should be slow and typically requires weeks to arrive at full enteral nutrition.Brunicardi_Ch39_p1705-p1758.indd 174212/02/19 11:27 AM 1743PEDIATRIC SURGERYCHAPTER 39There has been recent success with the utilization of non-surgical closure of gastroschisis. In this technique, the umbili-cal cord is placed over the defect, which is then covered with a transparent occlusive dressing. Over the ensuing days, the cord provides a tissue barrier, and the defect spontaneously


Surgery_Schwartz

Surgery_Schwartz. the umbili-cal cord is placed over the defect, which is then covered with a transparent occlusive dressing. Over the ensuing days, the cord provides a tissue barrier, and the defect spontaneously closes. This approach allows for nonsurgical coverage in a majority of cases of gastroschisis, even in the setting of very large openings. Questions remain regarding the long-term presence of umbilical hernias in these children and the total hospitalization.Prune-Belly SyndromeClinical Presentation. Prune-belly syndrome refers to a dis-order that is characterized by extremely lax lower abdominal musculature, dilated urinary tract including the bladder, and bilateral undescended testes (Fig. 39-34). The term prune-belly syndrome appropriately describes the wrinkled appearance of the anterior abdominal wall that characterizes these patients. Prune-belly syndrome is also known as Eagle-Barrett syn-drome as well as the triad syndrome because of the three major manifestations. The incidence is


Surgery_Schwartz

Surgery_Schwartz. wall that characterizes these patients. Prune-belly syndrome is also known as Eagle-Barrett syn-drome as well as the triad syndrome because of the three major manifestations. The incidence is significantly higher in males. Patients manifest a variety of comorbidities. The most signifi-cant is pulmonary hypoplasia, which can be unsurvivable in the most severe cases. Skeletal abnormalities include dislocation or dysplasia of the hip and pectus excavatum.The major genitourinary manifestation in prune-belly syn-drome is ureteral dilation. The ureters are typically long and tortuous and become more dilated distally. Ureteric obstruction is rarely present, and the dilation may be caused by decreased smooth muscle and increased collagen in the ureters. Approxi-mately eighty percent of these patients will have some degree of vesicureteral reflux, which can predispose to urinary tract infection. Despite the marked dilatation of the urinary tract, most children with prune-belly syndrome have


Surgery_Schwartz

Surgery_Schwartz. will have some degree of vesicureteral reflux, which can predispose to urinary tract infection. Despite the marked dilatation of the urinary tract, most children with prune-belly syndrome have adequate renal parenchyma for growth and development. Factors associated with the development of long-term renal failure include the presence of abnormal kidneys on US or renal scan and persis-tent pyelonephritis.Treatment. Despite the ureteric dilation, there is currently no role for ureteric surgery unless an area of obstruction develops. The testes are invariably intraabdominal, and bilateral orchido-pexy can be performed in conjunction with abdominal wall recon-struction at 6 to 12 months of age. Despite orchiopexy, fertility in Figure 39-34. Eagle-Barrett (prune-belly) syndrome. Notice the lax, flaccid abdomen.a boy with prune-belly syndrome is unlikely as spermatogenesis over time is insufficient. Deficiencies in the production of pros-tatic fluid and a predisposition to retrograde


Surgery_Schwartz

Surgery_Schwartz. lax, flaccid abdomen.a boy with prune-belly syndrome is unlikely as spermatogenesis over time is insufficient. Deficiencies in the production of pros-tatic fluid and a predisposition to retrograde ejaculation contrib-ute to infertility. Abdominal wall repair is accomplished through an abdominoplasty, which typically requires a transverse inci-sion in the lower abdomen extending into the flanks.Inguinal HerniaAn understanding of the management of pediatric inguinal her-nias is a central component of modern pediatric surgical prac-tice. Inguinal hernia repair represents one of the most common operations performed in children. The presence of an inguinal hernia in a child is an indication for surgical repair. The opera-tion is termed a herniorrhaphy because it involves closing off the patent processus vaginalis. This is to be contrasted with the hernioplasty that is performed in adults, which requires a recon-struction of the inguinal floor.Embryology. In order to understand how to


Surgery_Schwartz

Surgery_Schwartz. processus vaginalis. This is to be contrasted with the hernioplasty that is performed in adults, which requires a recon-struction of the inguinal floor.Embryology. In order to understand how to diagnose and treat inguinal hernias in children, it is critical to understand their embryologic origin. It is very useful to describe these events to the parents, who often are under the misconception that the her-nia was somehow caused by their inability to console their crying child, or the child’s high activity level. Inguinal hernia results from a failure of closure of the processus vaginalis; a finger-like projection of the peritoneum that accompanies the testicle as it descends into the scrotum. Closure of the processus vaginalis normally occurs a few months prior to birth. This explains the high incidence of inguinal hernias in premature infants. When the processes vaginalis remains completely patent, a commu-nication persists between the peritoneal cavity and the groin, resulting in a


Surgery_Schwartz

Surgery_Schwartz. high incidence of inguinal hernias in premature infants. When the processes vaginalis remains completely patent, a commu-nication persists between the peritoneal cavity and the groin, resulting in a hernia. Partial closure can result in entrapped fluid, which results in the presence of a hydrocele. A communicating hydrocele refers to a hydrocele that is in communication with the peritoneal cavity and can therefore be thought of as a hernia. Using the classification system that is typically applied to adult hernias, all congenital hernias in children are by definition indi-rect inguinal hernias. Children also present with direct inguinal and femoral hernias, although these are much less common.Clinical Manifestation. Inguinal hernias occur more com-monly in males than females (10:1) and are more common on the right side than the left. Infants are at high risk for incar-ceration of an inguinal hernia because of the narrow inguinal ring. Patients most commonly present with a groin bulge


Surgery_Schwartz

Surgery_Schwartz. more common on the right side than the left. Infants are at high risk for incar-ceration of an inguinal hernia because of the narrow inguinal ring. Patients most commonly present with a groin bulge that is noticed by the parents as they change the diaper (Fig. 39-35). Figure 39-35. Right inguinal hernia in a 4-month-old male. The arrows point to the bulge in the right groin.Brunicardi_Ch39_p1705-p1758.indd 174312/02/19 11:27 AM 1744SPECIFIC CONSIDERATIONSPART IIOlder children may notice the bulge themselves. On examina-tion, the cord on the affected side will be thicker, and pressure on the lower abdomen usually will display the hernia on the affected side. The presence of an incarcerated hernia is mani-fested by a firm bulge that does not spontaneously resolve and may be associated with fussiness and irritability in the child. The infant that has a strangulated inguinal hernia will manifest an edematous, tender bulge in the groin, occasionally with over-lying skin changes. The


Surgery_Schwartz

Surgery_Schwartz. with fussiness and irritability in the child. The infant that has a strangulated inguinal hernia will manifest an edematous, tender bulge in the groin, occasionally with over-lying skin changes. The child will eventually develop intestinal obstruction, peritonitis, and systemic toxicity.Usually an incarcerated hernia can be reduced. Occasion-ally this may require light sedation. Gentle pressure is applied on the sac from below in the direction of the internal inguinal ring. Following reduction of the incarcerated hernia, the child may be admitted for observation, and herniorrhaphy is per-formed within the next 24 hours to prevent recurrent incarcera-tion. Alternatively, the child may be scheduled for surgery at the next available time slot. If the hernia cannot be reduced, or if evidence of strangulation is present, emergency operation is necessary. This may require a laparotomy and bowel resection.When the diagnosis of inguinal hernia is made in an oth-erwise normal child, operative


Surgery_Schwartz

Surgery_Schwartz. of strangulation is present, emergency operation is necessary. This may require a laparotomy and bowel resection.When the diagnosis of inguinal hernia is made in an oth-erwise normal child, operative repair should be planned. Spon-taneous resolution does not occur, and therefore a nonoperative approach cannot ever be justified. An inguinal hernia in a female infant or child frequently contains an ovary rather than intestine. Although the gonad usually can be reduced into the abdomen by gentle pressure, it often prolapses in and out until surgical repair is carried out. In some patients, the ovary and fallopian tube constitute one wall of the hernial sac (sliding hernia), and in these patients, the ovary can be reduced effectively only at the time of operation. If the ovary is irreducible, prompt hernia repair is indicated to prevent ovarian torsion or strangulation.When a hydrocele is diagnosed in infancy and there is no evidence of a hernia, observation is proper therapy until the


Surgery_Schwartz

Surgery_Schwartz. prompt hernia repair is indicated to prevent ovarian torsion or strangulation.When a hydrocele is diagnosed in infancy and there is no evidence of a hernia, observation is proper therapy until the child is older than 12 months. If the hydrocele has not disappeared by 12 months, invariably there is a patent processus vaginalis, and operative hydrocelectomy with excision of the processus vaginalis is indicated. When the first signs of a hydrocele are seen after 12 months of age, the patient should undergo elective hydrocelectomy, which in a child is always performed through a groin incision. Aspiration of hydroceles is discouraged because almost all without a patent processus vaginalis will resorb spon-taneously and those with a communication to the peritoneum will recur and require operative repair eventually. Transillumi-nation as a method to distinguish between hydrocele and hernia is nonspecific. A noncommunicating hydrocele is better identi-fied by palpation of a nonreducible oval


Surgery_Schwartz

Surgery_Schwartz. repair eventually. Transillumi-nation as a method to distinguish between hydrocele and hernia is nonspecific. A noncommunicating hydrocele is better identi-fied by palpation of a nonreducible oval structure that appears to have a blunt end below the external ring, indicating an isolated fluid collection without a patent connection to the peritoneum.Surgical Repair. The repair of a pediatric inguinal hernia can be extremely challenging, particularly in the premature child with incarceration. A small incision is made in a skin crease in the groin directly over the internal inguinal ring. Scarpa’s fascia is seen and divided. The external oblique muscle is dis-sected free from overlying tissue, and the location of the exter-nal ring is confirmed. The external oblique aponeurosis is then opened along the direction of the external oblique fibers over the inguinal canal. The undersurface of the external oblique is then cleared from surrounding tissue. The cremasteric fibers are separated


Surgery_Schwartz

Surgery_Schwartz. along the direction of the external oblique fibers over the inguinal canal. The undersurface of the external oblique is then cleared from surrounding tissue. The cremasteric fibers are separated from the cord structures and hernia sac, and these are then elevated into the wound. Care is taken not to grasp the vas deferens. The hernia sac is then dissected up to the internal ring and doubly suture ligated. The distal part of the hernia sac is opened widely to drain any hydrocele fluid. When the hernia is very large and the patient very small, tightening of the internal inguinal ring or even formal repair of the inguinal floor may be necessary, although the vast majority of children do not require any treatment beyond high ligation of the hernia sac.Controversy exists regarding the role for exploration of an asymptomatic opposite side in a child with an inguinal hernia. Several reports indicate that frequency of a patent processus vaginalis on the side opposite the obvious hernia is


Surgery_Schwartz

Surgery_Schwartz. for exploration of an asymptomatic opposite side in a child with an inguinal hernia. Several reports indicate that frequency of a patent processus vaginalis on the side opposite the obvious hernia is approxi-mately 30%, although this figure decreases with increasing age of the child. Management options include never exploring the opposite side, to exploring only under certain conditions such as in premature infants or in patients in whom incarceration is pres-ent. The opposite side may readily be explored laparoscopically. To do so, a blunt 3-mm trochar is placed into the hernia sac of the affected side. The abdominal cavity is insufflated, and the 2.7-mm 70° camera is placed through the trochar such that the opposite side is visualized. The status of the processes vaginalis on the opposite side can be visualized. However, the presence of a patent processus vaginalis by laparoscopy does not always imply the presence of a hernia.There has been quite widespread adoption of laparoscopic


Surgery_Schwartz

Surgery_Schwartz. side can be visualized. However, the presence of a patent processus vaginalis by laparoscopy does not always imply the presence of a hernia.There has been quite widespread adoption of laparoscopic approach in the management of inguinal hernias in children, especially those under the age of 2 years. This technique requires insufflation through the umbilicus and the placement of an extra-peritoneal suture to ligate the hernia sac. Proponents of this pro-cedure emphasize the fact that no groin incision is used, so there is a decreased chance of injuring cord structures, and that visu-alization of the contralateral side is achieved immediately. The long-term results of this technique have been quite excellent.Inguinal hernias in children recur in less than 1% of patients, and recurrences usually result from missed hernia sacs at the first procedure, a direct hernia, or a missed femoral hernia. All children should have local anesthetic administered either by caudal injection or by direct


Surgery_Schwartz

Surgery_Schwartz. result from missed hernia sacs at the first procedure, a direct hernia, or a missed femoral hernia. All children should have local anesthetic administered either by caudal injection or by direct injection into the wound. Spinal anesthesia in preterm infant decreases the risk of postoperative apnea when compared with general anesthesia.GENITALIAUndescended testisEmbryology. The term undescended testicle (cryptorchidism) refers to the interruption of the normal descent of the testis into the scrotum. The testicle may reside in the retroperineum, in the internal inguinal ring, in the inguinal canal, or even at the external ring. The testicle begins as a thickening on the uro-genital ridge in the fifth to sixth week of embryologic life. In the seventh and eighth months, the testicle descends along the inguinal canal into the upper scrotum, and with its progress the processus vaginalis is formed and pulled along with the migrat-ing testicle. At birth, approximately 95% of infants have the


Surgery_Schwartz

Surgery_Schwartz. along the inguinal canal into the upper scrotum, and with its progress the processus vaginalis is formed and pulled along with the migrat-ing testicle. At birth, approximately 95% of infants have the testicle normally positioned in the scrotum.A distinction should be made between an undescended testicle and an ectopic testicle. An ectopic testis, by definition, is one that has passed through the external ring in the normal pathway and then has come to rest in an abnormal location over-lying either the rectus abdominis or external oblique muscle, or the soft tissue of the medial thigh, or behind the scrotum in the perineum. A congenitally absent testicle results from failure of normal development or an intrauterine accident leading to loss of blood supply to the developing testicle.Brunicardi_Ch39_p1705-p1758.indd 174412/02/19 11:27 AM 1745PEDIATRIC SURGERYCHAPTER 39Clinical Presentation. The incidence of undescended testes is approximately 30% in preterm infants, and 1% to 3% at


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Surgery_Schwartz. 174412/02/19 11:27 AM 1745PEDIATRIC SURGERYCHAPTER 39Clinical Presentation. The incidence of undescended testes is approximately 30% in preterm infants, and 1% to 3% at term. For diagnosis, the child should be examined in the supine posi-tion, where visual inspection may reveal a hypoplastic or poorly rugated scrotum. Usually a unilateral undescended testicle can be palpated in the inguinal canal or in the upper scrotum. Occa-sionally, the testicle will be difficult or impossible to palpate, indicating either an abdominal testicle or congenital absence of the gonad. If the testicle is not palpable in the supine position, the child should be examined with his legs crossed while seated. This maneuver diminishes the cremasteric reflex and facilitates identification of the location of the testicle. If there is uncer-tainty regarding location of a testis, repeated evaluations over time may be helpful.It is now established that cryptorchid testes demonstrate an increased predisposition


Surgery_Schwartz

Surgery_Schwartz. testicle. If there is uncer-tainty regarding location of a testis, repeated evaluations over time may be helpful.It is now established that cryptorchid testes demonstrate an increased predisposition to malignant degeneration. In addition, fertility is decreased when the testicle is not in the scrotum. For these reasons, surgical placement of the testicle in the scrotum (orchidopexy) is indicated. It should be emphasized that this procedure does improve the fertility potential, although it is never normal. Similarly, the testicle is still at risk of malignant change, although its location in the scrotum facilitates poten-tially earlier detection of a testicular malignancy. Other reasons to consider orchidopexy include the risk of trauma to the testicle located at the pubic tubercle and incidence of torsion, as well as the psychological impact of an empty scrotum in a developing male. The reason for malignant degeneration is not established, but the evidence points to an inherent


Surgery_Schwartz

Surgery_Schwartz. incidence of torsion, as well as the psychological impact of an empty scrotum in a developing male. The reason for malignant degeneration is not established, but the evidence points to an inherent abnormality of the testicle that predisposes it to incomplete descent and malignancy rather than malignancy as a result of an abnormal environment.Treatment. Males with bilateral undescended testicles are often infertile. When the testicle is not present within the scrotum, it is subjected to a higher temperature, resulting in decreased spermatogenesis. Mengel and coworkers studied 515 undescended testicles by histology and demonstrated reduced spermatogonia after 2 years of age. It is now recommended that the undescended testicle be surgically repositioned by 1 year of age. Despite orchidopexy, the incidence of infertility is approx-imately two times higher in men with unilateral orchidopexy compared to men with normal testicular descent.The use of chorionic gonadotropin occasionally may be


Surgery_Schwartz

Surgery_Schwartz. the incidence of infertility is approx-imately two times higher in men with unilateral orchidopexy compared to men with normal testicular descent.The use of chorionic gonadotropin occasionally may be effective in patients with bilateral undescended testes, suggest-ing that these patients are more apt to have a hormone insuf-ficiency than children with unilateral undescended testicle. The combination of micro-penis and bilateral undescended testes is an indication for hormonal evaluation and testoster-one replacement if indicated. If there is no testicular descent after a month of endocrine therapy, operative correction should be undertaken. A child with unilateral cryptorchidism should have surgical correction of the problem. The operation is typi-cally performed through a combined groin and scrotal incision. The cord vessels are fully mobilized, and the testicle is placed in a dartos pouch within the scrotum. An inguinal hernia often accompanies a cryptorchid testis. This should be


Surgery_Schwartz

Surgery_Schwartz. and scrotal incision. The cord vessels are fully mobilized, and the testicle is placed in a dartos pouch within the scrotum. An inguinal hernia often accompanies a cryptorchid testis. This should be repaired at the time of orchidopexy.Patients with a nonpalpable testicle present a challenge in management. The current approach involves laparoscopy to identify the location of the testicle. If the spermatic cord is found to traverse the internal ring or the testis is found at the ring and can be delivered into the scrotum, a groin incision is made and an orchidopexy is performed. If an abdominal testis is identified that is too far to reach the scrotum, a two-staged Fowler-Stephens approach is used. In the first stage, the testicular vessels are clipped laparoscopically, which promotes the development of new blood vessels along the vas deferens. Several months later, the second stage is performed during which the testis is mobilized laparoscopically along with a swath of peritoneum with


Surgery_Schwartz

Surgery_Schwartz. of new blood vessels along the vas deferens. Several months later, the second stage is performed during which the testis is mobilized laparoscopically along with a swath of peritoneum with collateralized blood supply along the vas. Preservation of the gubernacular attachments with its collaterals to the testicle may confer improved testicular survival following orchidopex in over 90%. It is, nonetheless, preferable to preserve the testicular vessels whenever possible and complete mobilization of the testicle with its vessels intact.Vaginal AnomaliesSurgical diseases of the vagina in children are either congenital or acquired. Congenital anomalies include a spectrum of dis-eases that range from simple defects (imperforate hymen) to more complex forms of vaginal atresia, including distal, proxi-mal, and, most severe, complete. These defects are produced by abnormal development of müllerian ducts and/or urogenital sinus. The diagnosis is made most often by physical examina-tion.


Surgery_Schwartz

Surgery_Schwartz. proxi-mal, and, most severe, complete. These defects are produced by abnormal development of müllerian ducts and/or urogenital sinus. The diagnosis is made most often by physical examina-tion. Secretions into the obstructed vagina produce hydrocol-pos, which may present as a large, painful abdominal mass. The anatomy may be defined using US. Pelvic magnetic resonance imaging provides the most thorough and accurate assessment of the pelvic structures. Treatment is dependent on the extent of the defect. For an imperforate hymen, division of the hymen is curative. More complex forms of vaginal atresia require mobi-lization of the vaginal remnants and creation of an anastomosis at the perineum. Laparoscopy can be extremely useful, both in mobilizing the vagina, in draining hydrocolpos, and in evaluat-ing the internal genitalia. Complete vaginal atresia requires the construction of skin flaps or the creation of a neovagina using a segment of colon.The most common acquired disorder of the


Surgery_Schwartz

Surgery_Schwartz. evaluat-ing the internal genitalia. Complete vaginal atresia requires the construction of skin flaps or the creation of a neovagina using a segment of colon.The most common acquired disorder of the vagina is the straddle injury. This often occurs as young girls fall on blunt objects which cause a direct injury to the perineum. Typical manifestations include vaginal bleeding and inability to void. Unless the injury is extremely superficial, patients should be examined in the operating room where the lighting is optimal and sedation can be administered. Examination under anesthe-sia is particularly important in girls who are unable to void, suggesting a possible urethral injury. Vaginal lacerations are repaired using absorbable sutures, and the proximity to the ure-thra should be carefully assessed. Prior to hospital discharge, it is important that girls are able to void spontaneously. In all cases of vaginal trauma, it is essential that the patient be assessed for the presence of


Surgery_Schwartz

Surgery_Schwartz. assessed. Prior to hospital discharge, it is important that girls are able to void spontaneously. In all cases of vaginal trauma, it is essential that the patient be assessed for the presence of sexual abuse. In these cases, early contact with the sexual abuse service is necessary so that the appropriate microbiologic and photographic evidence can be obtained.Ovarian Cysts and TumorsPathologic Classification. Ovarian cysts and tumors may be classified as nonneoplastic or neoplastic. Nonneoplastic lesions include cysts (simple, follicular, inclusion, paraovarian, or cor-pus luteum), endometriosis, and inflammatory lesions. Neo-plastic lesions are classified based on the three primordia that contribute to the ovary: mesenchymal components of the uro-genital ridge, germinal epithelium overlying the urogenital ridge, and germ cells migrating from the yolk sac. The most common variety is germ cell tumors. Germ cell tumors are classified based on the degree of differentiation and the


Surgery_Schwartz

Surgery_Schwartz. overlying the urogenital ridge, and germ cells migrating from the yolk sac. The most common variety is germ cell tumors. Germ cell tumors are classified based on the degree of differentiation and the cellular components Brunicardi_Ch39_p1705-p1758.indd 174512/02/19 11:27 AM 1746SPECIFIC CONSIDERATIONSPART IIinvolved. The least differentiated tumors are the dysgermino-mas, which share features similar to the seminoma in males. Although these are malignant tumors, they are extremely sensi-tive to radiation and chemotherapy. The most common germ cell tumors are the teratomas, which may be mature, immature, or malignant. The degree of differentiation of the neural elements of the tumor determines the degree of immaturity. The sex cord stromal tumors arise from the mesenchymal components of the urogenital ridge. These include the granulosa-theca cell tumors and the Sertoli-Leydig cell tumors. These tumors often produce hormones that result in precocious puberty or hirsutism,


Surgery_Schwartz

Surgery_Schwartz. of the urogenital ridge. These include the granulosa-theca cell tumors and the Sertoli-Leydig cell tumors. These tumors often produce hormones that result in precocious puberty or hirsutism, respec-tively. Although rare, epithelial tumors do occur in children. These include serous and mucinous cystadenomas.Clinical Presentation. Children with ovarian lesions usually present with abdominal pain. Other signs and symptoms include a palpable abdominal mass, evidence of urinary obstruction, symp-toms of bowel obstruction, and endocrine imbalance. The surgical approach depends on the appearance of the mass at operation (i.e., whether it is benign-appearing or is suspicious for malignancy). In the case of a simple ovarian cyst, surgery depends on the size of the cyst and the degree of symptoms it causes. In general, large cysts (over 4–5 cm) in size should be resected, as they are unlikely to resolve, may be at risk of torsion, and may mask an underlying malignancy. Resection may be


Surgery_Schwartz

Surgery_Schwartz. it causes. In general, large cysts (over 4–5 cm) in size should be resected, as they are unlikely to resolve, may be at risk of torsion, and may mask an underlying malignancy. Resection may be performed laparoscopically, and ovarian tissue should be spared in all cases.Surgical Management. For ovarian lesions that appear malignant, it is important to obtain tumor markers including α-fetoprotein (teratomas), LDH (dysgerminoma), β-human cho-rionic gonadotropin (choriocarcinoma), and CA-125 (epithelial tumors). Although the diagnostic sensitivity of these markers is not always reliable, they provide material for postoperative follow-up and indicate the response to therapy. When a malig-nancy is suspected, the patient should undergo a formal cancer operation. This procedure is performed through either a mid-line incision or a Pfannenstie approach. Ascites and peritoneal washings should be collected for cytologic study. The liver and diaphragm are inspected carefully for metastatic


Surgery_Schwartz

Surgery_Schwartz. through either a mid-line incision or a Pfannenstie approach. Ascites and peritoneal washings should be collected for cytologic study. The liver and diaphragm are inspected carefully for metastatic disease. An omentectomy is performed if there is any evidence of tumor present. Pelvic and para-aortic lymph nodes are biopsied, and the primary tumor is resected completely. Finally, the contra-lateral ovary is carefully inspected, and if a lesion is seen, it should be biopsied. Dysgerminomas and epithelial tumors may be bilateral in up to 15% of cases. The surgical approach for a benign lesion of the ovary should include preservation of the ipsi-lateral fallopian tube and preservation of the noninvolved ovary.Ovarian Cysts in the Newborn. Ovarian cysts may be detected by prenatal US. The approach to lesions less than 4 cm should include serial US evaluation every 2 months or so as many of these lesions will resolve spontaneously. Consid-eration should be given to laparoscopic excision of


Surgery_Schwartz

Surgery_Schwartz. to lesions less than 4 cm should include serial US evaluation every 2 months or so as many of these lesions will resolve spontaneously. Consid-eration should be given to laparoscopic excision of cysts larger than 4 cm to avoid the risks of ovarian torsion or development of abdominal symptoms. For smaller lesions, resolution occurs by approximately 6 months of age. A laparoscopic approach is preferable in these cases. By contrast, complex cysts of any size require surgical intervention at presentation to exclude the pos-sibility of malignancy.Ambiguous GenitaliaEmbryology. Normal sexual differentiation occurs in the sixth fetal week. In every fetus, wolffian (male) and müllerian (female) ducts are present until the onset of sexual differentiation. Normal sexual differentiation is directed by the sex determining region of the Y chromosome (SRY). This is located on the distal end of the short arm of the Y chromosome. SRY provides a genetic switch that initiates gonadal differentiation in


Surgery_Schwartz

Surgery_Schwartz. the sex determining region of the Y chromosome (SRY). This is located on the distal end of the short arm of the Y chromosome. SRY provides a genetic switch that initiates gonadal differentiation in the mammalian urogenital ridge. Secretion of Müllerian-inhibiting substance (MIS) by the Sertoli cells of the seminiferous tubules results in regression of the müllerian duct, the anlage of the uterus, Fal-lopian tubes, and the upper vagina. The result of MIS secretion therefore is a phenotypic male. In the absence of SRY in the Y chromosome, MIS is not produced, and the müllerian duct derivatives are preserved. Thus, the female phenotype prevails.In order for the male phenotype to develop, the embryo must have a Y chromosome, the SRY must be normal with-out point mutations or deletions, testosterone and MIS must be produced by the differentiated gonad, and the tissues must respond to these hormones. Any disruption of the orderly steps in sexual differentiation may be reflected clinically


Surgery_Schwartz

Surgery_Schwartz. and MIS must be produced by the differentiated gonad, and the tissues must respond to these hormones. Any disruption of the orderly steps in sexual differentiation may be reflected clinically as variants of the intersex syndromes.These may be classified as (a) true hermaphroditism (with ovarian and testicular gonadal tissue), (b) male pseudohermaph-roditism (testicles only), (c) female pseudohermaphroditism (ovarian tissue only), and (d) mixed gonadal dysgenesis (usually underdeveloped or imperfectly formed gonads).True Hermaphroditism This represents the rarest form of ambiguous genitalia. Patients have both normal male and female gonads, with an ovary on one side and a testis on the other. Occasionally, an ovotestis is present on one or both sides. The majority of these patients have a 46,XX karyotype. Both the tes-tis and the testicular portion of the ovotestis should be removed.Male Pseudohermaphroditism This condition occurs in infants with an XY karyotype but deficient


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Surgery_Schwartz. have a 46,XX karyotype. Both the tes-tis and the testicular portion of the ovotestis should be removed.Male Pseudohermaphroditism This condition occurs in infants with an XY karyotype but deficient masculinization of the external genitalia. Bilateral testes are present, but the duct structures differentiate partly as phenotypic females. The causes include inadequate testosterone production due to biosynthetic error, inability to convert testosterone to dihy-drotestosterone due to 5α-reductase deficiency or deficiencies in androgen receptors. The latter disorder is termed testicular feminization syndrome. Occasionally, the diagnosis in these children is made during routine inguinal herniorrhaphy in a phenotypic female at which time testes are found. The testes should be resected due to the risk of malignant degeneration, although this should be performed only after a full discussion with the family has occurred.Female Pseudohermaphroditism The most common cause of female


Surgery_Schwartz

Surgery_Schwartz. due to the risk of malignant degeneration, although this should be performed only after a full discussion with the family has occurred.Female Pseudohermaphroditism The most common cause of female pseudohermaphroditism is congenital adrenal hyper-plasia. These children have a 46,XX karyotype but have been exposed to excessive androgens in utero. Common enzyme deficiencies include 21-hydroxylase, 11-hydroxylase, and 3β-hydroxysteroid dehydrogenase. These deficiencies result in overproduction of intermediary steroid hormones, which results in masculinization of the external genitalia of the XX fetus. These patients are unable to synthesize cortisol. In 90% of cases, deficiency of 21-hydroxylase causes adrenocorticotropic hor-mone (ACTH) to stimulate the secretion of excessive quantities of adrenal androgen, which masculinizes the developing female (Fig. 39-36). These infants are prone to salt loss, and require cortisol replacement. Those with mineralocorticoid deficiency also require


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Surgery_Schwartz. adrenal androgen, which masculinizes the developing female (Fig. 39-36). These infants are prone to salt loss, and require cortisol replacement. Those with mineralocorticoid deficiency also require fluorocortisone replacement.Mixed Gonadal Dysgenesis This syndrome is associated with dysgenetic gonads and retained mullerian structures. The typical karyotype is mosaic, usually 45XO,46XY. A high incidence of Brunicardi_Ch39_p1705-p1758.indd 174612/02/19 11:27 AM 1747PEDIATRIC SURGERYCHAPTER 39Figure 39-36. Ambiguous genitalia manifest as enlarged clitoris and labioscrotal folds in a baby with the adrenogenital syndrome.malignant tumors occur in the dysgenetic gonads, most com-monly gonadoblastoma. Therefore, they should be removed.Management. In the differential diagnosis of patients with intersex anomalies, the following diagnostic steps are necessary: (a) evaluation of the genetic background and family history; (b) assessment of the anatomic structures by physical exami-nation,


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Surgery_Schwartz. intersex anomalies, the following diagnostic steps are necessary: (a) evaluation of the genetic background and family history; (b) assessment of the anatomic structures by physical exami-nation, US, and/or chromosome studies; (c) determination of biochemical factors in serum and urine to evaluate the presence of an enzyme defect; and (d) laparoscopy for gonadal biopsy. Treatment should include correction of electrolyte and volume losses, in cases of congenital adrenal hyperplasia, and replace-ment of hormone deficiency. Surgical assignment of gender should never be determined at the first operation. Although his-torically female gender had been assigned, there is abundant and convincing evidence that raising a genotypic male as a female has devastating consequences, not only anatomically but also psychosocially. This is particularly relevant given the role of preand postnatal hormones on gender imprinting and identity. In general terms, surgical reconstruction should be performed


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Surgery_Schwartz. but also psychosocially. This is particularly relevant given the role of preand postnatal hormones on gender imprinting and identity. In general terms, surgical reconstruction should be performed after a full genetic workup and with the involvement of pediatric endocrinologists, pediatric plastic surgeons, and ethicists with expertise in gender issues. Discussion with the family also plays an important role. This approach will serve to reduce the anxi-ety associated with these disorders and will help to ensure the normal physical and emotional development of these patients.PEDIATRIC MALIGNANCYCancer is the second leading cause of death in children after trauma and accounts for approximately 11% of all pediatric deaths in the United States. The following description will be restricted to the most commonly encountered tumors in children.Wilms’ TumorClinical Presentation. Wilms’ tumor is the most common primary malignant tumor of the kidney in children. There are approximately 500 new


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Surgery_Schwartz. the most commonly encountered tumors in children.Wilms’ TumorClinical Presentation. Wilms’ tumor is the most common primary malignant tumor of the kidney in children. There are approximately 500 new cases annually in the United States, and most are diagnosed between 1 and 5 years with the peak inci-dence at age 3. Advances in the care of patients with Wilms’ tumor has resulted in an overall cure rate of roughly 90%, even in the presence of metastatic spread. The tumor usually develops in otherwise healthy children as an asymptomatic mass in the flank or upper abdomen. Frequently, the mass is discovered by a parent while bathing or dressing the child. Other symptoms include hypertension, hematuria, obstipation, and weight loss. Occasionally the mass is discovered following blunt abdominal trauma.Genetics of Wilms’ Tumor. Wilms’ tumor can arise from both germline and somatic mutations and can occur in the presence or absence of a family history. Nearly 97% of Wilms’ tumors are sporadic


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Surgery_Schwartz. trauma.Genetics of Wilms’ Tumor. Wilms’ tumor can arise from both germline and somatic mutations and can occur in the presence or absence of a family history. Nearly 97% of Wilms’ tumors are sporadic in that they occur in the absence of a heritable or congenital cause or risk factor. When a heritable risk factor is identified, the affected children often present at an earlier age, and the tumors are frequently bilateral. Most of these tumors are associated with germline mutations. It is well established that there is a genetic predisposition to Wilms’ tumor in WAGR syndrome, which consists of Wilms’ tumor, aniridia, genitouri-nary abnormalities, and mental retardation. In addition, there is an increased incidence of Wilms’ tumor in certain overgrowth conditions, particularly Beckwith–Wiedemann syndrome and hemihypertrophy. WAGR syndrome has been shown to result from the deletion of one copy each of the Wilms’ tumor gene, WT1, and the adjacent aniridia gene, PAX6, on chromosome 11p13.


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Surgery_Schwartz. syndrome and hemihypertrophy. WAGR syndrome has been shown to result from the deletion of one copy each of the Wilms’ tumor gene, WT1, and the adjacent aniridia gene, PAX6, on chromosome 11p13. Beckwith–Wiedemann syndrome is an overgrowth syn-drome that is characterized by visceromegaly, macroglossia, and hyperinsulinemic hypoglycemia. It arises from mutations at the 11p15.5 locus. There is evidence to suggest that analysis of the methylation status of several genes in the 11p15 locus could predict the individual risk to the development of Wilms’ tumor. Importantly, most patients with Wilms’ tumor do not have mutations at these genetic loci.Surgical Treatment. Before operation, all patients suspected of having Wilms’ tumor should undergo abdominal and chest computerized tomography. These studies characterize the mass, identify the presence of metastases, and provide information on the opposite kidney (Fig. 39-37). CT scanning also indicates the presence of nephrogenic rests, which are


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Surgery_Schwartz. characterize the mass, identify the presence of metastases, and provide information on the opposite kidney (Fig. 39-37). CT scanning also indicates the presence of nephrogenic rests, which are precursor lesions to Wilms’ tumor. An abdominal US should be performed to evalu-ate the presence of renal vein or vena caval extension.The management of patients with Wilms’ tumor has been carefully analyzed within the context of large studies involving thousands of patients. These studies have been coordinated by the National Wilms’ Tumor Study Group (NWTSG) in North America and the International Society of Paediatric Oncology Figure 39-37. Wilms’ tumor of the right kidney (arrow) in a 3-year-old girl.Brunicardi_Ch39_p1705-p1758.indd 174712/02/19 11:27 AM 1748SPECIFIC CONSIDERATIONSPART IITable 39-3Staging of Wilms’ tumorStage I: Tumor limited to the kidney and completely excised.Stage II: Tumor that extends beyond the kidney but is completely excised. This includes penetration of the renal


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Surgery_Schwartz. 39-3Staging of Wilms’ tumorStage I: Tumor limited to the kidney and completely excised.Stage II: Tumor that extends beyond the kidney but is completely excised. This includes penetration of the renal capsule, invasion of the soft tissues of the renal sinus, or blood vessels within the nephrectomy specimen outside the renal parenchyma containing tumor. No residual tumor is apparent at or beyond the margins of excision.a Stage III: Residual nonhematogenous tumor confined to the abdomen. Lymph nodes in the abdomen or pelvis contain tumor. Peritoneal contamination by the tumor, such as by spillage or biopsy of tumor before or during surgery. Tumor growth that has penetrated through the peritoneal surface. Implants are found on the peritoneal surfaces. Tumor extends beyond the surgical margins either microscopically or grossly. Tumor is not completely resectable because of local infiltration into vital structures. The tumor was treated with preoperative chemotherapy with or without


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Surgery_Schwartz. margins either microscopically or grossly. Tumor is not completely resectable because of local infiltration into vital structures. The tumor was treated with preoperative chemotherapy with or without biopsy. Tumor is removed in greater than one piece.Stage IV: Hematogenous metastases or lymph node involvement outside the abdomino-pelvic region.Stage V: Bilateral renal involvement.International Neuroblastoma Staging SystemStage 1: Localized tumor with complete gross resection, with or without microscopic residual diseaseStage 2A: Localized tumor with incomplete gross excision; representative ipsilateral nonadherent lymph nodes negative for tumorStage 2B: Localized tumor with or without complete gross excision, with ipsilateral nonadherent lymph nodes positive for tumor. Enlarged contralateral lymph nodes must be negative microscopicallyStage 3: Unresectable unilateral tumor crossing midline, with or without regional lymph node involvement; or localized unilateral tumor with


Surgery_Schwartz

Surgery_Schwartz. contralateral lymph nodes must be negative microscopicallyStage 3: Unresectable unilateral tumor crossing midline, with or without regional lymph node involvement; or localized unilateral tumor with contralateral regional lymph node involvement; or midline tumorStage 4: Any primary tumor with dissemination to distant lymph nodes, bone, bone marrow, liver, skin, and/or other organsStage 4S: In infants <1 year of age; localized primary tumor with dissemination limited to skin, liver, and/or bone marrowInternational Neuroblastoma Risk Group Staging SystemL1 Localized tumor not involving vital structures as defined by the list of IDRFs and confined to one body compartmentL2 Locoregional tumor with the presence of one or more IDRFsM Distant metastatic disease (except MS)MS Metastatic disease in children <18 months confined to skin, liver, and bone marrow aRupture or spillage confined to the flank, including biopsy of the tumor, is no longer included in stage II and is now included in


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Surgery_Schwartz. in children <18 months confined to skin, liver, and bone marrow aRupture or spillage confined to the flank, including biopsy of the tumor, is no longer included in stage II and is now included in stage III.(SIOP), mainly involving European countries. Significant dif-ferences in the approach to patients with Wilms’ tumor have been highlighted by these studies. NWTSG supports a strat-egy of surgery followed by chemotherapy in most instances, whereas the SIOP approach is to shrink the tumor using preoper-ative chemotherapy. There are instances were preoperative che-motherapy is supported by both groups, including the presence of bilateral involvement or inferior vena cava involvement that extends above the hepatic veins and involvement of a solitary kidney by Wilms’ tumor. The NWTSG proponents argue that preoperative therapy in other instances results in a loss of impor-tant staging information, and therefore places patients at higher risk for recurrence; alternatively, it may lead to


Surgery_Schwartz

Surgery_Schwartz. argue that preoperative therapy in other instances results in a loss of impor-tant staging information, and therefore places patients at higher risk for recurrence; alternatively, it may lead to overly aggres-sive treatment in some cases and greater morbidity. However, the overall survival rates are not different between the NWTSG and SIOP approaches.The goal of surgery is complete removal of the tumor. It is crucial to avoid tumor rupture or injury to contiguous organs. A sampling of regional lymph nodes should be included, and all suspicious nodes should be sampled. Typically, a large transverse abdominal incision is made, and a transperitoneal approach is used. The opposite side is carefully inspected to ensure that there is no disease present. Although historically this involved the complete mobilization of the contralateral kidney, current evidence indicates that preoperative, high-resolution CT scanning is of sufficient accuracy for the detection of clinically significant


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Surgery_Schwartz. the complete mobilization of the contralateral kidney, current evidence indicates that preoperative, high-resolution CT scanning is of sufficient accuracy for the detection of clinically significant lesions if they are present. Provided only unilateral disease is present, a radical nephroureterectomy is then performed with control of the renal pedicle as an initial step. If there is spread above the hepatic veins, an intrathoracic approach may be required. If bilateral disease is encountered, both lesions are biopsied, and chemotherapy is administered followed by a nephron-sparing procedure.Chemotherapy. Following nephroureterectomy for Wilms’ tumor, the need for chemotherapy and/or radiation therapy are determined by the histology of the tumor and the clinical stage of the patient (Table 39-3). Essentially, patients who have dis-ease confined to one kidney completely excised surgically receive a short course of chemotherapy and can expect a 97% 4-year survival, with tumor relapse


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Surgery_Schwartz. 39-3). Essentially, patients who have dis-ease confined to one kidney completely excised surgically receive a short course of chemotherapy and can expect a 97% 4-year survival, with tumor relapse rare after that time. Patients with more advanced disease or with unfavorable histol-ogy receive more intensive chemotherapy and radiation. Even in stage IV, high cure rates may be achieved. The survival rates are worse in the small percentage of patients considered to have unfavorable histology.NeuroblastomaClinical Presentation. Neuroblastoma is the third most com-mon pediatric malignancy and accounts for approximately 10% of all childhood cancers. The vast majority of patients have advanced disease at the time of presentation, and unlike Wilms’ tumor, in which cure is expected in the vast majority of patients, the overall survival of patients with neuroblastoma is significantly lower. Over 80% of cases present before the age of 4 years, and the peak incidence is two years of age.


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Surgery_Schwartz. vast majority of patients, the overall survival of patients with neuroblastoma is significantly lower. Over 80% of cases present before the age of 4 years, and the peak incidence is two years of age. Neuro-blastomas arise from the neural crest cells and show different levels of differentiation. The tumor originates most frequently in the adrenal glands, posterior mediastinum, neck, or pelvis but can arise in any sympathetic ganglion. The clinical presen-tation depends on the site of the primary and the presence of metastases.9Brunicardi_Ch39_p1705-p1758.indd 174812/02/19 11:27 AM 1749PEDIATRIC SURGERYCHAPTER 39Two-thirds of these tumors are first noted as an asymp-tomatic abdominal mass. The tumor may cross the midline, and a majority of patients will already show signs of metastatic disease. Occasionally, children may experience pain from the tumor mass or from bony metastases. Proptosis and perior-bital ecchymosis may occur due to the presence of retrobulbar metastasis. Because


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Surgery_Schwartz. disease. Occasionally, children may experience pain from the tumor mass or from bony metastases. Proptosis and perior-bital ecchymosis may occur due to the presence of retrobulbar metastasis. Because they originate in paraspinal ganglia, neuro-blastomas may invade through neural foramina and compress the spinal cord, causing muscle weakness or sensory changes. Rarely, children may have severe watery diarrhea due to the secretion of vasoactive intestinal peptide by the tumor, or with paraneoplastic neurologic findings including cerebellar ataxia or opsoclonus/myoclonus. The International Neuroblastoma Stag-ing System and the International Neuroblastoma Risk Group Staging System are provided in Table 39-3.Diagnostic Evaluation. Since these tumors derive from the sympathetic nervous system, catecholamines and their metabo-lites will be produced at increased levels. These include elevated levels of serum catecholamines (dopamine, norepinephrine) or urine catecholamine metabolites:


Surgery_Schwartz

Surgery_Schwartz. catecholamines and their metabo-lites will be produced at increased levels. These include elevated levels of serum catecholamines (dopamine, norepinephrine) or urine catecholamine metabolites: vanillylmandelic acid (VMA) or homovanillic acid (HVA). Measurement of VMA and HVMA in serum and urine aids in the diagnosis and in monitoring ade-quacy of future treatment and recurrence. The minimum criterion for a diagnosis of neuroblastoma is based on one of the following: (a) an unequivocal pathologic diagnosis made from tumor tissue by light microscopy (with or without immunohistology, electron microscopy, or increased levels of serum catecholamines or uri-nary catecholamine metabolites); (b) the combination of bone marrow aspirate or biopsy containing unequivocal tumor cells and increased levels of serum catecholamines or urinary catechol-amine metabolites as described earlier.The patient should be evaluated by abdominal computer-ized tomography, which may show displacement and


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Surgery_Schwartz. levels of serum catecholamines or urinary catechol-amine metabolites as described earlier.The patient should be evaluated by abdominal computer-ized tomography, which may show displacement and occasion-ally obstruction of the ureter of an intact kidney (Fig. 39-38). Prior to the institution of therapy, a complete staging workup should be performed. This includes radiograph of the chest, bone marrow biopsy, and radionuclide scans to search for metastases. Any abnormality on chest X-ray should be followed up with CT of the chest.Prognostic Indicators. A number of biologic variables have been studied in children with neuroblastoma. An open biopsy is required in order to provide tissue for this analysis. Hyperdip-loid tumor DNA is associated with a favorable prognosis, and Figure 39-38. Abdominal neuroblastoma arising from the right retroperitoneum (arrow).N-myc amplification is associated with a poor prognosis regard-less of patient age. The Shimada classification describes tumors as


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Surgery_Schwartz. neuroblastoma arising from the right retroperitoneum (arrow).N-myc amplification is associated with a poor prognosis regard-less of patient age. The Shimada classification describes tumors as either favorable or unfavorable histology based on the degree of differentiation, the mitosis-karyorrhexis index, and the pres-ence or absence of schwannian stroma. In general, children of any age with localized neuroblastoma and infants younger than 1 year of age with advanced disease and favorable disease char-acteristics have a high likelihood of disease-free survival. By contrast, older children with advanced-stage disease have a sig-nificantly decreased chance for cure despite intensive therapy. For example, aggressive multiagent chemotherapy has resulted in a 2-year survival rate of approximately 20% in older children with stage IV disease. Neuroblastoma in the adolescent has a worse long-term prognosis regardless of stage or site and, in many cases, a more prolonged course.Surgery. The


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Surgery_Schwartz. 20% in older children with stage IV disease. Neuroblastoma in the adolescent has a worse long-term prognosis regardless of stage or site and, in many cases, a more prolonged course.Surgery. The goal of surgery is complete resection. However, this is often not possible at initial presentation due to the exten-sive locoregional spread of the tumor at the time of presenta-tion. Under these circumstances, a biopsy is performed, and preoperative chemotherapy is provided based upon the stage of the tumor. After neoadjuvant treatment has been administered, surgical resection is performed. The principal goal of surgery is to obtain at least 95% resection without compromising major structures. Abdominal tumors are approached through a trans-verse incision. Thoracic tumors may be approached through a posterolateral thoracotomy or through a thoracoscopic approach. These may have an intraspinal component. In all cases of intra-thoracic neuroblastoma, particularly those at the thoracic inlet, it


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Surgery_Schwartz. a posterolateral thoracotomy or through a thoracoscopic approach. These may have an intraspinal component. In all cases of intra-thoracic neuroblastoma, particularly those at the thoracic inlet, it is important to be aware of the possibility of a Horner’s syn-drome (anhidrosis, ptosis, meiosis) developing. This typically resolves, although it may take many months to do so.Neuroblastoma in Infants. Spontaneous regression of neu-roblastoma has been well described in infants, especially in those with stage 4S disease. Regression generally occurs only in tumors with a near triploid number of chromosomes that also lack N-myc amplification and loss of chromosome 1p. Recent studies indicate that infants with asymptomatic, small, low-stage neuroblastoma detected by screening may have tumors that spontaneously regress. These patients may be observed safely without surgical intervention or tissue diagnosis.RhabdomyosarcomaRhabdomyosarcoma is a primitive soft tissue tumor that arises from


Surgery_Schwartz

Surgery_Schwartz. that spontaneously regress. These patients may be observed safely without surgical intervention or tissue diagnosis.RhabdomyosarcomaRhabdomyosarcoma is a primitive soft tissue tumor that arises from mesenchymal tissues. The most common sites of origin include the head and neck (36%), extremities (19%), genitourinary tract (2%), and trunk (9%), although the tumor can arise virtually anywhere. The clinical presentation of the tumor depends on the site of origin. The diagnosis is confirmed with incisional or excisional biopsy after evaluation by MRI, CT scans of the affected area and the chest, and bone marrow biopsy. The tumor grows locally into surrounding structures and metastasizes widely to lung, regional lymph nodes, liver, brain, and bone marrow. The staging system for rhabdomyosarcoma is based upon the TNM system, as established by the Soft Tissue Sarcoma Committee of the Children’s Oncology Group. It is shown in Table 39-4. Surgery is an important component of the staging


Surgery_Schwartz

Surgery_Schwartz. is based upon the TNM system, as established by the Soft Tissue Sarcoma Committee of the Children’s Oncology Group. It is shown in Table 39-4. Surgery is an important component of the staging strategy and involves biopsy of the lesion and evaluation of lymphatics. Primary resection should be undertaken when complete excision can be performed without causing disability. If this is not possible, the lesion is biopsied, and intensive che-motherapy is administered. It is important to plan the biopsy so that it does not interfere with subsequent resection. After the Brunicardi_Ch39_p1705-p1758.indd 174912/02/19 11:27 AM 1750SPECIFIC CONSIDERATIONSPART IItumor has decreased in size, resection of gross residual disease should be performed. Radiation therapy is effective in achieving local control when microscopic or gross residual disease exists following initial treatment. Patients with completely resected tumors of embryonal histology do well without radiation ther-apy, but radiation


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Surgery_Schwartz. when microscopic or gross residual disease exists following initial treatment. Patients with completely resected tumors of embryonal histology do well without radiation ther-apy, but radiation therapy benefits patients with group I tumors with alveolar or undifferentiated histology.Prognosis. The prognosis for rhabdomyosarcoma is related to the site of origin, resectability, presence of metastases, number of metastatic sites, and histopathology. Primary sites with more favorable prognoses include the orbit and nonparameningeal head and neck, paratestis and vagina (nonbladder, nonprostate genitourinary), and the biliary tract. Patients with tumors less than 5 cm in size have improved survival compared to children with larger tumors, while children with metastatic disease at diagnosis have the poorest prognosis. Tumor histology influ-ences prognosis and the embryonal variant is favorable while the alveolar subtype is unfavorable.TeratomaTeratomas are tumors composed of tissue from all


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Surgery_Schwartz. the poorest prognosis. Tumor histology influ-ences prognosis and the embryonal variant is favorable while the alveolar subtype is unfavorable.TeratomaTeratomas are tumors composed of tissue from all three embry-onic germ layers. They may be benign or malignant, they may arise in any part of the body, and they are usually found in mid-line structures. Thoracic teratomas usually present as an anterior mediastinal mass. Ovarian teratomas present as an abdominal mass often with symptoms of torsion, bleeding, or rupture. Ret-roperitoneal teratomas may present as a flank or abdominal mass.Mature teratomas usually contain well-differentiated tis-sues and are benign, while immature teratomas contain vary-ing degrees of immature neuroepithelium or blastemal tissues. Immature teratomas can be graded from 1 to 3 based on the amount of immature neuroglial tissue present. Tumors of higher grade are more likely to have foci of yolk sac tumor. Malignant germ cell tumors usually contain frankly


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Surgery_Schwartz. graded from 1 to 3 based on the amount of immature neuroglial tissue present. Tumors of higher grade are more likely to have foci of yolk sac tumor. Malignant germ cell tumors usually contain frankly neoplastic tissues of germ cell origin (i.e., yolk sac carcinoma, embryonal carcinoma, germinoma, or choriocarcinoma). Yolk sac carci-nomas produce α-fetoprotein (AFP), while choriocarcinomas produce β-human chorionic gonadotropin (BHCG) resulting in elevation of these substances in the serum, which can serve as tumor markers. In addition, germinomas can also produce elevation of serum BHCG but not to the levels associated with choriocarcinoma.Table 39-4Staging of RhabdomyosarcomaSTAGESITESTSIZENM1Orbit, nonparameningeal head and neck, genitourinary (other than kidney, bladder, and prostate), and biliaryT1 or T2a or bAny NM02Bladder/prostate, extremity, cranial parameningeal, otherT1 or T2a N0 or NXM03Bladder/prostate, extremity, cranial parameningeal, otherT1 or T2aN1M0 bAny NM04AllT1


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Surgery_Schwartz. and biliaryT1 or T2a or bAny NM02Bladder/prostate, extremity, cranial parameningeal, otherT1 or T2a N0 or NXM03Bladder/prostate, extremity, cranial parameningeal, otherT1 or T2aN1M0 bAny NM04AllT1 or T2a or bAny NM1T1 = tumor confined to anatomic site of origin; T2 = tumor extension and/or fixed to surrounding tissues; a = ≤5 cm; b = >5 cm; N0 = regional nodes not clinically involved; N1 = regional nodes clinically involved; NX = regional node status unknown; M0 = no distant metastasis; M1 = metastasis present.Clinical group:Group 1: Localized disease, completely resected, no regional lymph node involvement.Group 2: Localized disease, gross total resection but microscopic residual disease; or regional lymph nodes involved.Group 3: Localized disease with gross residual disease after incomplete resection or biopsy only.Group 4: Metastatic disease at diagnosis.Figure 39-39. Sacrococcygeal teratoma in a 2-day-old boy.Sacrococcygeal Teratoma. Sacrococcygeal teratoma usually presents as a


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Surgery_Schwartz. resection or biopsy only.Group 4: Metastatic disease at diagnosis.Figure 39-39. Sacrococcygeal teratoma in a 2-day-old boy.Sacrococcygeal Teratoma. Sacrococcygeal teratoma usually presents as a large mass extending from the sacrum in the new-born period. Diagnosis may be established by prenatal US. In fetuses with evidence of hydrops and a large sacrococcygeal teratoma, prognosis is poor; thus, prenatal intervention has been advocated in such patients. The mass may be as small as a few centimeters in diameter or as massive as the size of the infant (Fig. 39-39). The tumor has been classified based upon the location and degree of intrapelvic extension. Lesions that grow predominantly into the presacral space often present later in childhood. The differential diagnosis consists of neural tumors, lipoma, and myelomeningoceles.Most tumors are identified at birth and are benign. Malig-nant yolk sac tumor histology occurs in a minority of these tumors. Complete resection of the tumor as


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Surgery_Schwartz. tumors, lipoma, and myelomeningoceles.Most tumors are identified at birth and are benign. Malig-nant yolk sac tumor histology occurs in a minority of these tumors. Complete resection of the tumor as early as possible is essential. The rectum and genital structures are often distorted by the tumor but usually can be preserved in the course of resection. Perioperative complications of hypothermia and hemorrhage can occur with massive tumors and may prove lethal. This is of particular concern in small, preterm infants with large tumors. The cure rate is excellent if the tumor is excised completely. Brunicardi_Ch39_p1705-p1758.indd 175012/02/19 11:27 AM 1751PEDIATRIC SURGERYCHAPTER 39The majority of patients who develop recurrent disease are sal-vageable with subsequent platinum-based chemotherapy.Liver TumorsMore than two-thirds of all liver tumors in children are malig-nant. There are two major histologic subgroups: hepatoblastoma and hepatocellular carcinoma. The age of onset of


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Surgery_Schwartz. TumorsMore than two-thirds of all liver tumors in children are malig-nant. There are two major histologic subgroups: hepatoblastoma and hepatocellular carcinoma. The age of onset of liver cancer in children is related to the histology of the tumor. Hepatoblastoma is the most common malignancy of the liver in children, with most of these tumors diagnosed before 4 years of age. Hepatocel-lular carcinoma is the next most common, with a peak age inci-dence between 10 and 15 years. Malignant mesenchymomas and sarcomas are much less common but constitute the remainder of the malignancies. The finding of a liver mass does not necessar-ily imply that a malignancy is present. Nearly 50% of all masses are benign, and hemangiomas are the most common lesion.Most children with a liver tumor present with an abdomi-nal mass that is usually painless, which the parents note while changing the child’s clothes or while bathing the child. The patients are rarely jaundiced but may complain of anorexia and


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Surgery_Schwartz. an abdomi-nal mass that is usually painless, which the parents note while changing the child’s clothes or while bathing the child. The patients are rarely jaundiced but may complain of anorexia and weight loss. Most liver function tests are normal. AFP levels are increased in 90% of children with hepatoblastomas but much less commonly in other liver malignancies. Radiographic evaluation of these children should include an abdominal CT scan to identify the lesion and to determine the degree of local invasiveness (Fig. 39-40). For malignant appearing lesions, a biopsy should be performed unless the lesion can be completely resected easily. Hepatoblastoma is most often unifocal, while hepatocellular carcinoma is often extensively invasive or multi-centric. If a hepatoblastoma is completely removed, the majority of patients survive, but only a minority of patients have lesions amenable to complete resection at diagnosis.A staging system based on postsurgical extent of tumor and surgical


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Surgery_Schwartz. removed, the majority of patients survive, but only a minority of patients have lesions amenable to complete resection at diagnosis.A staging system based on postsurgical extent of tumor and surgical resectability is shown in Table 39-5. The overall survival rate for children with hepatoblastoma is 70%, but it is only 25% for hepatocellular carcinoma. Children diagnosed with stage I and II hepatoblastoma have a cure rate of greater than 90% compared to 60% for stage III and approximately 20% for stage IV. In children diagnosed with hepatocellular carcinoma, those with stage I have a good outcome, whereas stages III and IV are usually fatal. The fibrolamellar variant of hepatocel-lular carcinoma may have a better prognosis.Surgery. The abdominal CT scan usually will determine the resectability of the lesion, although occasionally this can only Figure 39-40. Computed tomography of the abdomen showing a hepatocellular carcinoma in a 12-year-old boy.be determined at the time of


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Surgery_Schwartz. resectability of the lesion, although occasionally this can only Figure 39-40. Computed tomography of the abdomen showing a hepatocellular carcinoma in a 12-year-old boy.be determined at the time of exploration. Complete surgical resection of the tumor is the primary goal and is essential for cure. For tumors that are unresectable, preoperative chemother-apy should be administered to reduce the size of the tumor and improve the possibility for complete removal. Chemotherapy is more successful for hepatoblastoma than for hepatocellular carcinoma. Areas of locally invasive disease, such as the dia-phragm, should be resected at the time of surgery. For unre-sectable tumors, liver transplantation may be offered in select patients. The fibrolamellar variant of hepatocellular carcinoma may have a better outcome with liver transplantation than other hepatocellular carcinomas.TRAUMA IN CHILDRENInjury is the leading cause of death among children older than 1 year. In fact, trauma accounts for


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Surgery_Schwartz. a better outcome with liver transplantation than other hepatocellular carcinomas.TRAUMA IN CHILDRENInjury is the leading cause of death among children older than 1 year. In fact, trauma accounts for almost half of all pediatric deaths, more than cancer, congenital anomalies, pneumonia, heart disease, homicide, and meningitis combined. Death from unintentional injuries accounts for 65% of all injury-related deaths in children younger than 19 years. Motor vehicle colli-sions are the leading cause of death in people age 1 to 19 years, followed by homicide or suicide (predominantly with firearms) and drowning. Each year, approximately 20,000 children and teenagers die as a result of injury in the United States. For every child who dies from an injury, it is calculated that 40 others are hospitalized and 1120 are treated in emergency departments. An estimated 50,000 children acquire permanent disabilities each year, most of which are the result of head injuries. Thus, the problem of


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Surgery_Schwartz. hospitalized and 1120 are treated in emergency departments. An estimated 50,000 children acquire permanent disabilities each year, most of which are the result of head injuries. Thus, the problem of pediatric trauma continues to be one of the major threats to the health and well-being of children.Specific considerations apply to trauma in children that influence management and outcome. These relate to the mecha-nisms of injury, the anatomic variations in children compared to adults, and the physiologic responses.Mechanisms of InjuryMost pediatric trauma is blunt. Penetrating injuries are seen in the setting of gun violence, falls onto sharp objects, or penetra-tion by glass after falling through windows. Age and gender significantly influence the patterns of injury. Male children between 14 and 18 years of age are exposed to contact sports, gun violence, and in some jurisdictions drive motor vehicles. As a result, they have a different pattern of injury than younger children,


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Surgery_Schwartz. between 14 and 18 years of age are exposed to contact sports, gun violence, and in some jurisdictions drive motor vehicles. As a result, they have a different pattern of injury than younger children, characterized by higher injury severity scores. In the infant and toddler age group, falls are a 10Table 39-5Staging of pediatric liver cancerStage I: No metastases, tumor completely resectedStage II: No metastases, tumor grossly resected with microscopic residual disease (i.e., positive margins); or tumor rupture, or tumor spill at the time of surgeryStage III: No distant metastases, tumor unresectable or resected with gross residual tumor, or positive lymph nodesStage IV: Distant metastases regardless of the extent of liver involvementData from Douglass E, Ortega J, Feusner J, et al. Hepatocellular carcinoma (HCA) in children and adolescents: results from the Pediatric Intergroup Hepatoma Study (CCG 8881/POG 8945), Proc Am Soc Clin Oncol. 1994;13:A-1439.Brunicardi_Ch39_p1705-p1758.indd


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Surgery_Schwartz. carcinoma (HCA) in children and adolescents: results from the Pediatric Intergroup Hepatoma Study (CCG 8881/POG 8945), Proc Am Soc Clin Oncol. 1994;13:A-1439.Brunicardi_Ch39_p1705-p1758.indd 175112/02/19 11:27 AM 1752SPECIFIC CONSIDERATIONSPART IIcommon cause of severe injury. Injuries in the home are extremely common. These include falls, near-drownings, caustic ingestion, and nonaccidental injuries.Initial ManagementThe goals of managing the pediatric trauma patient are similar to those of adults and follow Advanced Trauma Life Support guidelines as established by the American College of Surgeons Committee on Trauma. Airway control is the first priority. In a child, respiratory arrest can proceed quickly to cardiac arrest. It is important to be aware of the anatomic differences between the airway of the child and the adult. The child has a large head, shorter neck, smaller and anterior larynx, floppy epiglottis, short trachea, and large tongue. The size of the endotracheal tube


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Surgery_Schwartz. the airway of the child and the adult. The child has a large head, shorter neck, smaller and anterior larynx, floppy epiglottis, short trachea, and large tongue. The size of the endotracheal tube can be estimated by the formula (age + 16)/4. It is important to use uncuffed endotracheal tubes in children younger than 8 years in order to minimize tracheal trauma. After evaluation of the airway, breathing is assessed. It is important to consider that gastric distention from aerophagia can severely compromise respirations. A nasogastric tube should therefore be placed early during the resuscitation if there is no head injury suspected, or an orogastric tube in cases of head injury. Pneumothorax or hemothorax should be treated promptly. When evaluating the circulation, it is important to recognize that tachycardia is usu-ally the earliest measurable response to hypovolemia. Other signs of impending hypovolemic shock in children include changes in mentation, delayed capillary refill, skin


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Surgery_Schwartz. that tachycardia is usu-ally the earliest measurable response to hypovolemia. Other signs of impending hypovolemic shock in children include changes in mentation, delayed capillary refill, skin pallor, and hypothermia. IV access should be rapidly obtained once the patient arrives in the trauma bay. The first approach should be to use the antecubital fossae. If this is not possible, a cut-down into the saphenous at the groin can be performed quickly and safely. Intraosseous cannulation can provide temporary access in children and young adults until IV access is established. US-guided central line placement in the groin or neck should be considered in patients in whom large bore peripheral IV access is not obtained. Blood is drawn for cross-match and evaluation of liver enzymes, lipase, amylase, and hematologic profile after the IV lines are placed.In patients who show signs of volume depletion, a 20 mL/kg bolus of saline or lactated Ringer’s should be promptly given. If the patient


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Surgery_Schwartz. and hematologic profile after the IV lines are placed.In patients who show signs of volume depletion, a 20 mL/kg bolus of saline or lactated Ringer’s should be promptly given. If the patient does not respond to three boluses, blood should be transfused (10 mL/kg). The source of bleeding should be established. Common sites include the chest, abdomen, pel-vis, extremity fractures, or large scalp wounds. These should be carefully sought. Care is taken to avoid hypothermia by infusing warmed fluids and by using external warming devices.Evaluation of InjuryAll patients should receive an X-ray of the cervical spine, chest, and abdomen with pelvis. All extremities that are suspicious for fracture should also be evaluated by X-ray. Plain cervical spine films are preferable to performing routine neck CT scans in the child, as X-rays provide sufficient anatomic detail. But if a head CT is obtained, it may be reasonable to obtain images down to C-2 since odontoid views in small children are


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Surgery_Schwartz. neck CT scans in the child, as X-rays provide sufficient anatomic detail. But if a head CT is obtained, it may be reasonable to obtain images down to C-2 since odontoid views in small children are difficult to obtain. In most children, it is possible to diagnose clinically sig-nificant cervical spine injuries using this approach while mini-mizing the degree of radiation exposure. Screening blood work that includes AST, ALT, and amylase/lipase is useful for the evaluation of liver and pancreatic injures. Significant elevation in these tests requires further evaluation by CT scanning. The child with significant abdominal tenderness and a mechanism of injury that could cause intra-abdominal injury should undergo abdominal CT scanning using IV and oral contrast in all cases. There is a limited role for diagnostic peritoneal lavage (DPL) in children as a screening test. However, this can be occasionally useful in the child who is brought emergently to the operating room for management of


Surgery_Schwartz

Surgery_Schwartz. role for diagnostic peritoneal lavage (DPL) in children as a screening test. However, this can be occasionally useful in the child who is brought emergently to the operating room for management of significant intracranial hemorrhage. At the time of craniotomy, a DPL, or alternatively, a diagnostic laparoscopy, can be performed concurrently to identify abdomi-nal bleeding. Although focused abdominal US (FAST exam) is extremely useful in the evaluation of adult abdominal trauma, it is not widely accepted in the management of pediatric blunt abdominal trauma. In part, this relates to the widespread use of nonoperative treatment for most solid-organ injuries. Thus, a positive abdominal US scan would not alter this approach in a hemodynamically stable patient.Injuries to the Central Nervous SystemThe central nervous system (CNS) is the most commonly injured organ system and is the leading cause of death among injured children. In the toddler age group, nonaccidental trauma is the most


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Surgery_Schwartz. SystemThe central nervous system (CNS) is the most commonly injured organ system and is the leading cause of death among injured children. In the toddler age group, nonaccidental trauma is the most common cause of serious head injury. Findings suggestive of abuse include the presence of retinal hemorrhage on fundo-scopic evaluation and intracranial hemorrhage without evidence of external trauma (indicative of a shaking injury) and fractures at different stages of healing on skeletal survey. In older children, CNS injury occurs most commonly after falls and bicycle and motor vehicle collisions. The initial head CT can often underesti-mate the extent of injury in children. Criteria for head CT include any loss of consciousness or amnesia to the trauma, or inabil-ity to assess the CNS status as in the intubated patient. Patients with mild, isolated head injury (GCS 14-15) and negative CT scans can be discharged if their neurologic status is normal after 6 hours of observation. Young


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Surgery_Schwartz. as in the intubated patient. Patients with mild, isolated head injury (GCS 14-15) and negative CT scans can be discharged if their neurologic status is normal after 6 hours of observation. Young children and those in whom there is multisystem involvement should be admitted to the hospital for observation. Any change in the neurologic status warrants neu-rosurgical evaluation and repeat CT scanning. In patients with severe head injury (GCS 8 or less), urgent neurosurgical consulta-tion is required. These patients are evaluated for intracranial pres-sure monitoring and for the need to undergo craniotomy.Thoracic InjuriesThe pediatric thorax is pliable due to incomplete calcification of the ribs and cartilages. As a result, blunt chest injury com-monly results in pulmonary contusion, although rib fractures are infrequent. Diagnosis is made by chest radiograph and may be associated with severe hypoxia requiring mechanical ventila-tion. Pulmonary contusion usually resolves with careful


Surgery_Schwartz

Surgery_Schwartz. rib fractures are infrequent. Diagnosis is made by chest radiograph and may be associated with severe hypoxia requiring mechanical ventila-tion. Pulmonary contusion usually resolves with careful venti-lator management and judicious volume resuscitation. Children who have sustained massive blunt thoracic injury may develop traumatic asphyxia. This is characterized by cervical and facial petechial hemorrhages or cyanosis associated with vascular engorgement and subconjunctival hemorrhage. Management includes ventilation and treatment of coexisting CNS or abdomi-nal injuries. Penetrating thoracic injuries may result in damage to the lung or to major disruption of the bronchi or great vessels.Abdominal InjuriesIn children, the small rib cage and minimal muscular coverage of the abdomen can result in significant injury after seemingly minor trauma. The liver and spleen in particular are relatively unprotected and are often injured after direct abdominal trauma. Duodenal injuries are


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Surgery_Schwartz. can result in significant injury after seemingly minor trauma. The liver and spleen in particular are relatively unprotected and are often injured after direct abdominal trauma. Duodenal injuries are usually the result of blunt trauma, which may arise from child abuse or injury from a bicycle handlebar. Duodenal hematomas usually resolve without surgery. Brunicardi_Ch39_p1705-p1758.indd 175212/02/19 11:27 AM 1753PEDIATRIC SURGERYCHAPTER 39Small intestinal injury usually occurs in the jejunum in the area of fixation by the ligament of Treitz. These injuries are usually caused by rapid deceleration in the setting of a lap belt. There may be a hematoma on the anterior abdominal wall caused by a lap belt, the so-called seat belt sign (Fig. 39-41A). This should alert the caregiver to the possibility of an underlying small bowel injury (Fig. 39-41B), as well as to a potential lumbar spine injury (Chance fracture).The spleen is injured relatively commonly after blunt abdominal trauma in


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Surgery_Schwartz. of an underlying small bowel injury (Fig. 39-41B), as well as to a potential lumbar spine injury (Chance fracture).The spleen is injured relatively commonly after blunt abdominal trauma in children. The extent of injury to the spleen is graded (Table 39-6), and the management is governed by the injury grade. Current treatment involves a nonoperative approach in most cases, even for grade 4 injuries, assuming the patient is hemodynamically stable. This approach avoids surgery in most cases. All patients should be placed in a monitored unit, and type-specific blood should be available for transfusion. When nonoperative management is successful, as it is in most cases, an extended period of bed rest is prescribed. This optimizes the chance for healing and minimizes the likelihood of reinjury. A typical guideline is to keep the children on extremely restricted activity for 2 weeks longer than the grade of spleen injury (i.e., a child with a grade 4 spleen injury receives 6 weeks of


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Surgery_Schwartz. A typical guideline is to keep the children on extremely restricted activity for 2 weeks longer than the grade of spleen injury (i.e., a child with a grade 4 spleen injury receives 6 weeks of restricted activity). In children who have an ongoing fluid requirement, BAFigure 39-41. Abdominal computed tomography of patient who sustained a lapbelt injury. A. Bruising is noted across the abdomen from the lapbelt. B. At laparotomy, a perforation of the small bowel was identified.or when a blood transfusion is required, exploration should not be delayed. At surgery, the spleen can often be salvaged. If a splenectomy is performed, prophylactic antibiotics and immuni-zations should be administered to protect against overwhelming post splenectomy sepsis. The liver is also commonly injured after blunt abdominal trauma. A grading system is used to character-ize hepatic injuries (Table 39-7), and nonoperative management is usually successful (Fig. 39-42). Recent studies have shown that associated


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Surgery_Schwartz. abdominal trauma. A grading system is used to character-ize hepatic injuries (Table 39-7), and nonoperative management is usually successful (Fig. 39-42). Recent studies have shown that associated injuries are more significant predictors of out-come in children with liver injuries than the actual injury grade. Criteria for surgery are similar to those for splenic injury and primarily involve hemodynamic instability. The intraoperative considerations in the management of massive hepatic injury are similar in children and adults. Renal contusions may occur after significant blunt abdominal trauma. Nonoperative management is usually successful, unless patients are unstable due to active renal bleeding. It is important to confirm the presence of a nor-mal contralateral kidney at the time of surgery.FETAL INTERVENTIONOne to the most exciting developments in the field of pediatric surgery has been the emergence of fetal surgery. In general terms, performance of a fetal intervention may be


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Surgery_Schwartz. surgery.FETAL INTERVENTIONOne to the most exciting developments in the field of pediatric surgery has been the emergence of fetal surgery. In general terms, performance of a fetal intervention may be justified in the setting where a defect is present that would cause devastating consequences to the infant if left uncorrected. For the vast majority of congenital anomalies, postnatal surgery is the preferred modality. However, in specific circumstances, fetal surgery may offer the best possibility for a successful outcome. Table 39-6Grading of splenic injuriesGrade I: Subcapsular hematoma, <10% surface area capsular tear, <1 cm in depthGrade II: Subcapsular hematoma, nonexpanding, 10%–50% surface area; intraparenchymal hematoma, nonexpanding, <2 cm in diameter; capsular tear, active bleeding, 1–3 cm, does not involve trabecular vesselGrade III: Subcapsular hematoma, >50% surface area or expanding; intraparenchymal hematoma, >2 cm or expanding; laceration >3 cm in depth or involving


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Surgery_Schwartz. 1–3 cm, does not involve trabecular vesselGrade III: Subcapsular hematoma, >50% surface area or expanding; intraparenchymal hematoma, >2 cm or expanding; laceration >3 cm in depth or involving trabecular vesselsGrade IV: Ruptured intraparenchymal hematoma with active bleeding; laceration involving segmental or hilar vessels producing major devascularizatrion (>25% of spleen).Grade V: Shattered spleen; hilar vascular injury that devascularizes spleenTable 39-7Liver injury grading systemGrade I: Capsular tear <1 cm in depthGrade II: Capsular tear 1–3 cm in depth, <10 cm lengthGrade III: Capsular tear >3 cm in depthGrade IV: Parenchymal disruption 25%–75% of hepatic lobe or 1–3 Couinaud’s segmentsGrade V: Parenchymal disruption >75% of hepatic lobe or >3 Couinaud’s segments within a single lobe, injury to retrohepatic vena cavaReproduced with permission from Moore EE, Cogbill TH, Malangoni MA, et al: Organ injury scaling, Surg Clin North Am. 1995


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Surgery_Schwartz. >3 Couinaud’s segments within a single lobe, injury to retrohepatic vena cavaReproduced with permission from Moore EE, Cogbill TH, Malangoni MA, et al: Organ injury scaling, Surg Clin North Am. 1995 Apr;75(2):293-303.Brunicardi_Ch39_p1705-p1758.indd 175312/02/19 11:27 AM 1754SPECIFIC CONSIDERATIONSPART IIFigure 39-43. The EXIT procedure (ex utero intrapartum treat-ment) in a 34-week gestation age baby with a large cervical tera-toma. Intubation is being performed while the fetus is on placental support.Figure 39-42. Abdominal computed tomography in a child dem-onstrating a grade 3 liver laceration (arrows).Fetal Surgery for MyelomeningoceleMyelomeningocele refers to a spectrum of anomalies in which portions of the spinal cord are uncovered by the spinal column. This leaves the neural tissue exposed to the injurious effects of the amniotic fluid, as well as to trauma from contact with the uterine wall. Nerve damage ensues, resulting in varying degrees of lower extremity paralysis


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Surgery_Schwartz. tissue exposed to the injurious effects of the amniotic fluid, as well as to trauma from contact with the uterine wall. Nerve damage ensues, resulting in varying degrees of lower extremity paralysis as well as bowel and bladder dys-function. Initial observations indicated that the extent of injury progressed throughout the pregnancy, which provided the ratio-nale for fetal intervention. The current in utero approach for the fetus with myelomeningocele has focused on obtaining cover-age of the exposed spinal cord. The efficacy of in utero treat-ment versus postnatal repair was recently compared in a large multicenter trial as described earlier and showed that prenatal surgery for myelomeningocele reduced the need for shunting and improved motor outcomes at 30 months but was associ-ated with maternal and fetal risks. The results of this study have paved the way for the acceptance of in utero repair of myelome-ningocele in certain centers with the experience and expertise to perform this


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Surgery_Schwartz. maternal and fetal risks. The results of this study have paved the way for the acceptance of in utero repair of myelome-ningocele in certain centers with the experience and expertise to perform this procedure safely.The EXIT ProcedureThe EXIT procedure is an abbreviation for ex utero intrapar-tum treatment. It is utilized in circumstances where airway obstruction is predicted at the time of delivery due to the pres-ence of a large neck mass, such as a cystic hygroma or teratoma (Fig. 39-43), or congenital tracheal stenosis. The success of the procedure is dependent upon the maintenance of utero-placen-tal perfusion for a sufficient duration to secure the airway. To achieve this, deep uterine relaxation is obtained during a cae-sarian section under general anesthesia. Uterine perfusion with warmed saline also promotes relaxation and blood flow to the placenta. On average, between 20 and 30 minutes of placental perfusion can be achieved. The fetal airway is secured either by placement


Surgery_Schwartz

Surgery_Schwartz. warmed saline also promotes relaxation and blood flow to the placenta. On average, between 20 and 30 minutes of placental perfusion can be achieved. The fetal airway is secured either by placement of an orotracheal tube or performance of a tracheos-tomy. Once the airway is secured, the cord is cut, and a defini-tive procedure may be performed to relieve the obstruction in the postnatal period. In general terms, cystic neck masses such as lymphangiomas have a more favorable response to an EXIT procedure as compared to solid tumors, such as teratomas, par-ticularly in premature infants.The decision to perform a fetal intervention requires careful patient selection, as well as a multidisciplinary center that is dedicated to the surgical care of the fetus and the mother. Patient selection is dependent in part upon highly accurate prenatal imaging that includes US and MRI. Significant risks may be associated with the performance of a fetal surgical procedure, to both the mother and the


Surgery_Schwartz

Surgery_Schwartz. is dependent in part upon highly accurate prenatal imaging that includes US and MRI. Significant risks may be associated with the performance of a fetal surgical procedure, to both the mother and the fetus. From the maternal viewpoint, open fetal surgery may lead to uterine bleeding due to the uterine relaxation required during the procedure. The long-term effects on subsequent pregnancies remain to be established. For the fetus, in utero surgery carries the risk of premature labor and amniotic fluid leak. As a result, these procedures are performed only when the expected benefit of fetal intervention outweighs the risk to the fetus of standard postnatal care. Currently, open fetal intervention may be efficacious in certain instances of large congenital lung lesions with hydrops, large teratomas with hydrops, twin-twin transfusion syndrome, certain cases of congenital lower urinary tract obstruction, and myelomeningocele. The Management of Myelomeningocele Study, which was funded by


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Surgery_Schwartz. with hydrops, twin-twin transfusion syndrome, certain cases of congenital lower urinary tract obstruction, and myelomeningocele. The Management of Myelomeningocele Study, which was funded by the NIH, compared prenatal with postnatal repair of myelomeningocele, and determined that prenatal repair was associated with improved motor skills and independent walking. There are ongoing trials for the evaluation of fetal tracheal occlusion in the setting of severe congenital diaphragmatic hernia, from which early results are very promising. The field has undertaken a rigorous evaluation of the potential benefit of prenatal as compared to postnatal management of many of these conditions, given the significant risk that may be associated with fetal therapy.Fetal Surgery for Lower Urinary Tract ObstructionLower urinary tract obstruction refers to a group of diseases characterized by obstruction of the distal urinary system. Com-mon causes include the presence of posterior urethral valves and


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Surgery_Schwartz. urinary tract obstruction refers to a group of diseases characterized by obstruction of the distal urinary system. Com-mon causes include the presence of posterior urethral valves and urethral atresia, as well as other anomalies of the urethra and bladder. The pathologic effects of lower urinary tract obstruc-tion lie in the resultant massive bladder distention that occurs, which can lead to reflux hydronephrosis. This may result in oligohydramnios, and cause limb contractures, facial anoma-lies (Potter sequence), and pulmonary hypoplasia. Carefully selected patients with lower urinary tract obstruction may ben-efit from vesicoamniotic shunting. By relieving the obstruction and improving renal function, fetal growth and lung develop-ment may be preserved.Brunicardi_Ch39_p1705-p1758.indd 175412/02/19 11:27 AM 1755PEDIATRIC SURGERYCHAPTER 39BIBLIOGRAPHYEntries highlighted in bright blue are key references.Adzick NS, Thom EA, Spong CY, et al. A randomized trial of prenatal versus


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Surgery_Schwartz. 175412/02/19 11:27 AM 1755PEDIATRIC SURGERYCHAPTER 39BIBLIOGRAPHYEntries highlighted in bright blue are key references.Adzick NS, Thom EA, Spong CY, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011;364:993-1004.Adzick NS, Thom EA, Spong CY, et al. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med. 2011;364:993-1004.American Academy of Pediatrics Section on Orthopaedics; American Academy of Pediatrics Committee on Pediatric Emergency Medicine; American Academy of Pediatrics Section on Critical Care, et al. Management of pediatric trauma. Pediatrics. 2008;121(4):849-854.Andersen B, Kallehave F, Andersen HK. Antibiotics versus placebo for prevention of postoperative infection after appendicectomy. Cochrane Database Syst Rev. 2003;(2):CD001439.Anderson KD, Rouse TM, Randolph JG. A controlled trial of corticosteroids in children with corrosive injury of the esophagus. N Engl J Med.


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Surgery_Schwartz. Cochrane Database Syst Rev. 2003;(2):CD001439.Anderson KD, Rouse TM, Randolph JG. A controlled trial of corticosteroids in children with corrosive injury of the esophagus. N Engl J Med. 1990;323(10):637-640.Azarow K, Messineo A, Pearl R, Filler R, Barker G, Bohn D. Congenital diaphragmatic hernia—a tale of two cities: the Toronto experience. J Pediatr Surg. 1997;32(3):395-400.Ballance WA, Dahms BB, Shenker N, Kliegman RM. Pathology of neonatal necrotizing enterocolitis: a ten-year experience. J Pediatr. 1990;117(1 pt 2):S6-S13.Barraco RD, Cheng JD, Bromberg WJ, et al. Child passenger safety: an evidence-based review. J Trauma. 2010;69(6):1588-1590.Barthel ER, Pierce JR, Goodhue CJ, Burke RV, Ford HR, Upperman JS. Can a pediatric trauma center improve the response to a mass casualty incident? J Trauma Acute Care Surg. 2012;73(4):885-889.Bell MJ, Ternberg JL, Feigin RD, et al. Neonatal necrotizing enterocolitis. Therapeutic decisions based upon clinical staging. Ann Surg.


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Surgery_Schwartz. incident? J Trauma Acute Care Surg. 2012;73(4):885-889.Bell MJ, Ternberg JL, Feigin RD, et al. Neonatal necrotizing enterocolitis. Therapeutic decisions based upon clinical staging. Ann Surg. 1978;187(1):1-7.Bezerra JA, Spino C, Magee JC, et al. Use of corticosteroids after hepatoportoenterostomy for bile drainage in infants with biliary atresia: the START randomized clinical trial. JAMA. 2014;311:1750-1759.Bohn D. Congenital diaphragmatic hernia. Am J Respir Crit Care Med. 2002;166(7):911-915.Boloker J, Bateman DA, Wung JT, Stolar CJ. Congenital diaphragmatic hernia in 120 infants treated consecutively with permissive hypercapnea/spontaneous respiration/elective repair. J Pediatr Surg. 2002;37(3):357-366.Bouchard S, Johnson MP, Flake AW, et al. The EXIT procedure: experience and outcome in 31 cases. J Pediatr Surg. 2002; 37(3):418-426.Branstetter BF, Weissman JL, Kennedy TL, Whitaker M. The CT appearance of thyroglossal duct carcinoma. AJNR Am J Neuroradiol.