Surgery_Schwartz

Surgery_Schwartz. anteriorly, com-pletely displacing the incisor-bearing portion of the alveolus from the maxillary dental arch. Orbicularis continuity must be restored over an often protuberant premaxilla. The surgeon must carefully recreate the appearance of a symmetrical philtrum and median labial tubercle. Prototypical markings for bilateral cleft lip repair are demonstrated in Fig. 45-30A,B.20Any surgical approach to bilateral cleft lip repair would be incomplete without addressing the nasal stigmata, which include a short or absent columella, a poorly defined and underprojected nasal tip, and malpositioned lower lateral cartilages.25 Primary nasoplasty at the time of lip repair has become an increasingly common practice. Nasal skin and soft tissue are dissected free from the underlying cartilaginous framework, allowing for suture manipulation of lower lateral cartilages to improve tip symmetry, support, and projection.20Cleft Palate Repair The primary goal of palatoplasty is to enable normal


Surgery_Schwartz

Surgery_Schwartz. framework, allowing for suture manipulation of lower lateral cartilages to improve tip symmetry, support, and projection.20Cleft Palate Repair The primary goal of palatoplasty is to enable normal speech development. A successful palate repair is one that results in a robust, layered reconstruction of the cleft and restoration of functional velar anatomy. The two most com-mon techniques employed for soft palate repair are intravelar veloplasty (IVV) and Furlow double-opposing Z-plasty. Para-mount to each technique is the complete release of aberrant levator muscle insertions from the posterior edge of the hard palate. This maneuver untethers the velum anteriorly, enabling maximal levator muscle excursion in the superior and posterior directions postoperatively.21Brunicardi_Ch45_p1967-p2026.indd 198501/03/19 6:27 PM 1986SPECIFIC CONSIDERATIONSPART IIFigure 45-27. Variations in unilateral cleft lip morphology. Left unilateral incomplete cleft lip.Figure 45-26. Hallmarks of unilateral


Surgery_Schwartz

Surgery_Schwartz. 198501/03/19 6:27 PM 1986SPECIFIC CONSIDERATIONSPART IIFigure 45-27. Variations in unilateral cleft lip morphology. Left unilateral incomplete cleft lip.Figure 45-26. Hallmarks of unilateral cleft lip deformity include depression of the nasal tip and flaring of the alar base on the cleft side, deviation of the caudal septum and columella toward the non-cleft side, and deficient lip height (short philtral column) on the cleft side with cephalad rotation of the cleft side of cupid’s bow.ABIntravelar veloplasty requires meticulous dissection of the levator muscles with retropositioning and reconstruction of the sling mechanism in the posterior aspect of the soft palate. A Furlow double-opposing Z-plasty involves cleverly designed mirror image Z-plasties on the oral and nasal sides of the soft palate where the central limb of each Z-plasty is the cleft. The posteriorly based flap of mucosa on each surface of the palate incorporates the underlying levator muscle. Transposition of these


Surgery_Schwartz

Surgery_Schwartz. soft palate where the central limb of each Z-plasty is the cleft. The posteriorly based flap of mucosa on each surface of the palate incorporates the underlying levator muscle. Transposition of these flaps across the cleft lengthens the palate and, in a man-ner similar to IVV, corrects levator malposition. Lateral relax-ing incisions can be utilized to relieve tension on the closure, if necessary (Fig. 45-31A–C).21,31 In experienced hands, both techniques have demonstrated excellent speech outcomes and low fistula rates. However, direct comparison between the two methods has been difficult due to ongoing evolution of the IVV technique and wide variability in the extent of dissection between performing surgeons.26Clefts involving the hard palate (Veau II–IV) often require additional maneuvers for reconstruction. Wide undermining of the nasal floor mucosa in the subperiosteal plane facilitates the nasal-side repair. As palatal mucoperiosteum is thicker and less pliable, the oral-side


Surgery_Schwartz

Surgery_Schwartz. for reconstruction. Wide undermining of the nasal floor mucosa in the subperiosteal plane facilitates the nasal-side repair. As palatal mucoperiosteum is thicker and less pliable, the oral-side closure generally requires the use of relax-ing incisions along the lingual side of the alveolar ridge. Addi-tional medialization of the palatal soft tissue can be obtained by increasing isolation of the greater palatine neurovascular pedicle, which emerges from its foramen near the posterolateral aspect of the hard palate. Narrow Veau II clefts may be closed on the oral side by medialization of bilateral bipedicled muco-periosteal flaps (von Langenbeck palatoplasty), while wider clefts may require detachment of one or both flaps anteriorly for additional medialization (Bardach two-flap palatoplasty). Lateral relaxing incisions are left open, and typically heal by secondary intention within two weeks (Fig. 45-32).21,27Complications of palate repair include oronasal fistula, velopharyngeal


Surgery_Schwartz

Surgery_Schwartz. Lateral relaxing incisions are left open, and typically heal by secondary intention within two weeks (Fig. 45-32).21,27Complications of palate repair include oronasal fistula, velopharyngeal dysfunction, obstructive sleep apnea, and mid-face growth deficiency. Reported fistula rates vary widely in the literature, but increased incidence has been correlated with less experienced surgeons, wider clefts, and bilateral clefts.21,22 Few oronasal fistulae are amenable to closure with simple local tissue rearrangement. More commonly, a complete reelevation of palatal mucosa is required in order to obtain a tension-free layered closure. In the case of large or recurrent fistulae, there may be insufficient tissue available locally, and recruitment of regional healthy tissue from the buccal mucosa or tongue may be necessary.32Velopharyngeal dysfunction (VPD) is caused by incom-plete closure of the velopharyngeal port, which results in air leaking through the nose during speech. Approximately


Surgery_Schwartz

Surgery_Schwartz. or tongue may be necessary.32Velopharyngeal dysfunction (VPD) is caused by incom-plete closure of the velopharyngeal port, which results in air leaking through the nose during speech. Approximately 20% of patients develop VPD after primary palatoplasty. After insuring complete release and proper orientation of levator muscles, a posterior pharyngeal flap or a sphincter pharyngoplasty may be required to decrease the size of the velopharyngeal gap, allowing Brunicardi_Ch45_p1967-p2026.indd 198601/03/19 6:27 PM 1987PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45Figure 45-28. Left unilateral complete cleft lip.AponeurosisAHamulusTensor muscleLevator muscleUvulus muscleAponeurosisBHamulusTensor muscleAccessory muscleLevator muscleFigure 45-29. A. Normal anatomy: the levator veli palatini muscle forms a muscular sling in the posterior aspect of the soft palate. B. Cleft anatomy: the levator veli palatini muscles turn anteriorly, run along the cleft margin, and insert aberrantly into the


Surgery_Schwartz

Surgery_Schwartz. forms a muscular sling in the posterior aspect of the soft palate. B. Cleft anatomy: the levator veli palatini muscles turn anteriorly, run along the cleft margin, and insert aberrantly into the posterior edge of the hard palate. C. Rotation-advancement markings and repair for a unilateral complete cleft lip.ABCnasal air escape during speech.21 These operations carry a risk of obstructive sleep apnea, so preoperative polysomnography is indicated to rule out significant sleep-disordered breathing at baseline.Timeline for Repair The longstanding debate regarding opti-mal timing for lip and palate repair is ongoing. Central to this controversy is the impact of early surgical intervention on speech outcomes and midface growth. Current evidence sug-gests earlier palate repair is better for speech but more detri-mental to midface growth.21 Cleft care algorithms represent a compromise. Most experts perform lip repair between 3 and 6 months of age.33,34 Palate repair should be completed


Surgery_Schwartz

Surgery_Schwartz. speech but more detri-mental to midface growth.21 Cleft care algorithms represent a compromise. Most experts perform lip repair between 3 and 6 months of age.33,34 Palate repair should be completed prior to the onset of speech development, usually around 10 to 12 months of age. The alveolar cleft is often repaired secondarily with a can-cellous bone graft from the iliac crest. This operation provides bony support for the permanent teeth that will erupt adjacent to the cleft, and it is usually performed around 7 to 9 years of age. Orthognathic surgery and secondary rhinoplasty, if necessary, are delayed until skeletal maturity. The treatment timeline used at Nationwide Children’s Hospital can be seen in Fig. 45-33.Brunicardi_Ch45_p1967-p2026.indd 198701/03/19 6:28 PM 1988SPECIFIC CONSIDERATIONSPART IIABFigure 45-30. A. Bilateral cleft lip repair diagram. B. Bilateral cleft lip repair.ABCFigure 45-31. Furlow double opposing Z-plasty. A. Oral side markings. B. Nasal side markings.


Surgery_Schwartz

Surgery_Schwartz. 45-30. A. Bilateral cleft lip repair diagram. B. Bilateral cleft lip repair.ABCFigure 45-31. Furlow double opposing Z-plasty. A. Oral side markings. B. Nasal side markings. Note that the levator veli pala-tini muscle remains attached to the posteriorly based flap on each surface. C. Flap transposition and closure. The levator veli pala-tini muscle bundles, being attached to the posteriorly based flaps, are reoriented transversely and retrodisplaced as a result of flap transposition.Brunicardi_Ch45_p1967-p2026.indd 198801/03/19 6:28 PM 1989PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45Figure 45-34. The Tessier classification of craniofacial clefts. Numbered lines designate soft tissue manifestations (above) of the underlying skeletal clefts (below).Lip adhesion(1–2 months)Lip and primarynose repair(3–6 months)Orthognathicsurgery*(skeletal maturity)Definitiverhinoplasty*(after jaw surgery)Palate repair(10–12 months)Lip or noserevision*(> 6 years)VPD surgery*(4–7 years)Alveolar


Surgery_Schwartz

Surgery_Schwartz. primarynose repair(3–6 months)Orthognathicsurgery*(skeletal maturity)Definitiverhinoplasty*(after jaw surgery)Palate repair(10–12 months)Lip or noserevision*(> 6 years)VPD surgery*(4–7 years)Alveolar bonegrafting(7–11 years)Figure 45-32. Traditional von Langenbeck palatal repair with bilateral bipedicled mucoperiosteal flap.Figure 45-33. The treatment timeline used at Nationwide Children’s Hospital.The Importance of Team in Cleft Care Children born with CL/P require expertise of medical professionals from many different disciplines. In addition to experienced craniofacial surgeons, cleft teams typically consist of otolaryngologists, pediatricians, speech pathologists, feeding specialists, pediatric dentists, orthodontists, geneticists, psychologists, nurses, and social workers. Each member is an integral part of the team and absolutely essential for the delivery of comprehensive cleft care.21Atypical Craniofacial Clefts Beyond the familiar scope of clefts confined to the lip and


Surgery_Schwartz

Surgery_Schwartz. member is an integral part of the team and absolutely essential for the delivery of comprehensive cleft care.21Atypical Craniofacial Clefts Beyond the familiar scope of clefts confined to the lip and palate, there exist myriad forms of clefting that may affect the craniofacial skeleton. Sound epide-miologic studies of these atypical craniofacial clefts have been precluded by their extreme rarity, but rough estimates place them on the order of 100 times less common than CL/P. As a result, definitive causality has not been established. With the exception of some well-defined syndromes that include atypical craniofacial clefts, genetics does not appear to play a significant part in their pathogenesis. Some extrinsic factors that have been implicated include radiation, prenatal infections, early gesta-tional exposure to teratogenic drugs or chemicals, and amniotic bands. Metabolic derangements and vascular disturbances have also been hypothesized to play a role.27While CL/P can be


Surgery_Schwartz

Surgery_Schwartz. early gesta-tional exposure to teratogenic drugs or chemicals, and amniotic bands. Metabolic derangements and vascular disturbances have also been hypothesized to play a role.27While CL/P can be logically explained as an embryologic failure of fusion between facial processes, the location of the atypical craniofacial clefts is not well-accounted for by this theory. In the 1960s, Weston and Johnston used animal mod-els to demonstrate the vast contributions of neural crest cells to mesynchymal development of the face. They postulated that failure of these cells to penetrate into the developing face could lead to breakdown of the surrounding epithelia and result in atypical craniofacial clefts. The last 30 years has seen contin-ued refinement of this theory. Most recent evidence suggests that neural crest cells form developmental rests or ossification centers within the well-known facial processes. An abnormal number or impaired differentiation of these ossification centers may better


Surgery_Schwartz

Surgery_Schwartz. neural crest cells form developmental rests or ossification centers within the well-known facial processes. An abnormal number or impaired differentiation of these ossification centers may better explain the locations of clefts that seem to follow no known embryologic fusion plane.33In 1974, Paul Tessier published detailed anatomic obser-vations of a large series of children with atypical craniofacial clefts. He introduced a simple numbering system to classify these clefts based strictly on involved anatomy.28 Clefts were assigned numbers 0 to 14 as they radiate around the orbit. Num-bers 0 to 7 describe facial clefts, while 8 to 14 described cranial clefts. Fig. 45-34 illustrates the paths of soft tissue clefts (above) and their corresponding skeletal clefts (below).33,35A number 0 facial cleft and its number 14 cranial extension are midline clefts, which may be characterized by tissue defi-ciency or excess. Holoprosencephaly, a term used to describe a


Surgery_Schwartz

Surgery_Schwartz. (below).33,35A number 0 facial cleft and its number 14 cranial extension are midline clefts, which may be characterized by tissue defi-ciency or excess. Holoprosencephaly, a term used to describe a 10234568910111213141413121110987665432130334301122347Brunicardi_Ch45_p1967-p2026.indd 198901/03/19 6:28 PM 1990SPECIFIC CONSIDERATIONSPART IIfailed cleavage of the prosencephalon into two separate cere-bral hemispheres, presents as a midline tissue deficiency that causes variable degrees of hypotelorism and upper lip and nasal deformity. Mildly affected patients may have near-normal intel-ligence, while severely affected cases are incompatible with life. Representing the opposite end of the spectrum, patients with median cleft face dysmorphism typically present with a median clefts of the lip and/or premaxilla midline tissue excess, hypertelorism, bifid cranium, and a normal underlying CNS (Fig. 45-35A,B).33Tessier clefts 1, 2, and 3 originate at the cupids bow. All proceed cephalad


Surgery_Schwartz

Surgery_Schwartz. lip and/or premaxilla midline tissue excess, hypertelorism, bifid cranium, and a normal underlying CNS (Fig. 45-35A,B).33Tessier clefts 1, 2, and 3 originate at the cupids bow. All proceed cephalad through the piriform aperture and affect the nose. While number 1 and 2 clefts spare the orbit, number 3 clefts create continuity between the orbit, maxillary sinus, nasal and oral cavities. Clefts 4, 5, and 6 begin lateral to cupids bow, spare the nose, and pass cephalad to affect the orbit and lower eyelid. The number 7 cleft, otherwise known as craniofacial microsomia, extends transversely along a line from the oral com-missure to the auricular tragus. Underlying skeletal clefts can involve the mandible, maxilla, orbit, and cranium. Tessier clefts 8 through 10 continue to radiate laterally and superiorly around the orbit. Cranial extensions are numbered such that the sum of the facial cleft and its corresponding cranial extension is always 14. For example, the number 1 facial cleft


Surgery_Schwartz

Surgery_Schwartz. and superiorly around the orbit. Cranial extensions are numbered such that the sum of the facial cleft and its corresponding cranial extension is always 14. For example, the number 1 facial cleft continues as the number 13 cranial cleft, and the number 5 facial cleft continues as the number 9 cranial cleft.33,35 Clefts can be unilateral or bilateral and ABFigure 45-35. Tessier 0-14 clefts. A. Holoprosencephaly. Note the midline tissue deficiency, hypotelorism, and the rudimentary nose known as a “proboscis.” The degree of facial deformity in patients with holoprosencephaly typically reflects the degree to which the underlying CNS is affected. B. Median cleft face dysmorphism. Note the marked midline tissue excess and hypertelorism. Although this patient exhibits an obvious encephalocele, CNS function is usually normal.may occur in any combination. The constellation of bilateral Tes-sier clefts 6, 7, and 8 has been well-described within the context of Treacher Collins syndrome, in


Surgery_Schwartz

Surgery_Schwartz. CNS function is usually normal.may occur in any combination. The constellation of bilateral Tes-sier clefts 6, 7, and 8 has been well-described within the context of Treacher Collins syndrome, in which patients exhibit malar hypoplasia, lower eyelid colobomas, and downward-slanting palpebral fissures (Fig. 45-36A–C).33Treatment of atypical craniofacial clefts varies widely with each unique patient. Classical approaches to surgical man-agement involved excision of atrophic soft tissue along cleft margins with reconstruction by local tissue rearrangement, with or without underlying bone grafting. Unfortunately, this meth-odology gives little consideration to the aesthetic units of the face, and the resulting scars often cause postoperative deformi-ties of their own. Ortiz-Monasterio and Taylor proposed a new treatment philosophy based on the following tenants:1. Restoration of the craniofacial skeleton2. Reconstruction with skin and soft tissue with like color and texture3. Generous use


Surgery_Schwartz

Surgery_Schwartz. proposed a new treatment philosophy based on the following tenants:1. Restoration of the craniofacial skeleton2. Reconstruction with skin and soft tissue with like color and texture3. Generous use of tissue expanders4. Aesthetic unit and subunit reconstruction5. Scar location at limits of aesthetic subunits6. Symmetrical repositioning of key facial landmarksFig. 45-37 demonstrates the dramatic improvement in aes-thetic outcome that is attainable when abiding by this treatment philosophy.29Brunicardi_Ch45_p1967-p2026.indd 199001/03/19 6:28 PM 1991PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45ABCFigure 45-36. A child with Treacher Collins syndrome and the hallmark combination of Tessier clefts 6, 7, and 8. Note the downward-slanting palpebral fissures and profound malar hypoplasia due to complete absence of zygomas.Barring immediate danger to vital structures such as the eye, the timing of reconstruction can be determined on a case-by-case basis. Soft tissue clefts can be excised and


Surgery_Schwartz

Surgery_Schwartz. complete absence of zygomas.Barring immediate danger to vital structures such as the eye, the timing of reconstruction can be determined on a case-by-case basis. Soft tissue clefts can be excised and closed by classical measures within the first year of life. However, bony reconstruction should be delayed until at least 5 to 6 years of age to minimize iatrogenic impairment of facial growth. Serial tissue expansion of the cheek prior to this time may be necessary to excise unfavorable scars and reorient them along aesthetic subunit boundaries. Preoperative imaging, such as computed tomography (CT) or magnetic resonance imaging (MRI), is necessary to fully characterize the defects and plan the opera-tion. Additional preoperative workup should include anesthe-sia evaluation and labs, as these operations can be lengthy and accompanied by significant blood loss. Preparedness for blood transfusion is imperative.33,34Craniofacial clefts are typically approached through a combination of


Surgery_Schwartz

Surgery_Schwartz. these operations can be lengthy and accompanied by significant blood loss. Preparedness for blood transfusion is imperative.33,34Craniofacial clefts are typically approached through a combination of bicoronal and oral vestibular incisions. Various osteotomies have been described to reposition components of Brunicardi_Ch45_p1967-p2026.indd 199101/03/19 6:28 PM 1992SPECIFIC CONSIDERATIONSPART IIFigure 45-37. (left) Eight-year-old girl with significant deformity from local tissue rearrangement to reconstruct a right Tessier no. 4 cleft. (center) Schematic depicting current scars with a solid line and proper scars with a dotted line. (right) Same patient after serial tissue expan-sion and relocation of scars along borders of aesthetic units.the craniofacial skeleton, such as the orbits, maxilla, and man-dible. These may be used in conjunction with bone grafts from the calvarium, ribs or iliac crest, and fixation can be achieved with standard techniques using bioresorbable plates or


Surgery_Schwartz

Surgery_Schwartz. maxilla, and man-dible. These may be used in conjunction with bone grafts from the calvarium, ribs or iliac crest, and fixation can be achieved with standard techniques using bioresorbable plates or sutures.33Craniosynostosis. The term “craniosynostosis” refers to pre-mature fusion of one or more calvarial sutures. It occurs in up to 1 out of every 2000 live births, and single-suture, nonsyndromic patients account for 85% of cases. Of these, isolated sagittal cra-niosynostosis is the most common form, while lamdoidal is the least common. Normal suture maintenance is driven by underly-ing brain growth and a complex biochemical interplay between the suture and the underlying dura mater.30 Multiple genes have been implicated in the development of craniosynostosis, the most notable of which being FGFR and TWIST. Fifty percent of these present as de novo mutations, and most exhibit an autoso-mal dominant inheritance pattern. Environmental associations, such as maternal smoking, have been


Surgery_Schwartz

Surgery_Schwartz. being FGFR and TWIST. Fifty percent of these present as de novo mutations, and most exhibit an autoso-mal dominant inheritance pattern. Environmental associations, such as maternal smoking, have been postulated, but definitive causality has not been proven.31According to Virchow’s law, patients with craniosynosto-sis exhibit a predictable pattern of deformity that results from an arrest of cranial growth perpendicular to the prematurely fused suture, with a compensatory increase in growth parallel to the affected suture (Fig. 45-38). Isolated sagittal craniosynostosis, Patent suturesFused midline sutureFigure 45-38. (left) Patent sutures permit normal cranial growth in all directions. (right) Craniosynostosis results in restricted cranial growth across the synostotic suture with a compensatory increased growth parallel to the synostotic suture (Virchow’s law).for example, results in restricted cranial growth in the transverse direction and a compensatory increase in the


Surgery_Schwartz

Surgery_Schwartz. with a compensatory increased growth parallel to the synostotic suture (Virchow’s law).for example, results in restricted cranial growth in the transverse direction and a compensatory increase in the anterior-posterior diameter of the head with frontal and/or occipital bossing. This head shape is commonly referred to as “scaphocephaly.” Fig. 45-39 depicts various other isolated craniosynostoses and the patterns of deformity that ensue.36All patients with craniosynostosis should be screened for intracranial hypertension. It has been estimated that up to 17% of patients with single-suture involvement may develop elevated intracranial pressure (ICP). This risk approaches 50% in patients with multisuture craniosynostosis.36 Signs and symptoms of increased ICP may include headache, inconsolability, nausea, vomiting, lethargy, sleep apnea, developmental delay, bulging fontanelles, hydrocephalus, papilledema, or loss of vision.36,38 Facial dysmorphism and a strong family history should


Surgery_Schwartz

Surgery_Schwartz. nausea, vomiting, lethargy, sleep apnea, developmental delay, bulging fontanelles, hydrocephalus, papilledema, or loss of vision.36,38 Facial dysmorphism and a strong family history should raise suspicion for syndromic etiology, as seen in Apert, Crouzon, Pfeiffer, and Saethre-Chotzen syndromes, among others.Diagnosis of craniosynostosis begins with physical exam. A recent prospective multicenter study suggests 98% accu-racy of diagnosis based upon physical exam findings alone. Palpable ridges may be present on the cranium but are not pathognomonic for craniosynostosis. The much more reliable physical exam finding involves recognition of the distinct pat-terns of cranial growth that result from premature fusion of one or more sutures. Dysmorphic facies, suspicion for multisuture involvement, or any degree of uncertainty in the diagnosis can be clarified with adjunctive imaging. While skull plain films can provide useful information, 3D computed tomography has emerged as the new gold


Surgery_Schwartz

Surgery_Schwartz. or any degree of uncertainty in the diagnosis can be clarified with adjunctive imaging. While skull plain films can provide useful information, 3D computed tomography has emerged as the new gold standard imaging modality for diag-nosing craniosynostosis.37The goals of treatment for craniosynostosis are to achieve a more normalized head shape and to treat or prevent nega-tive impacts on development that may result from increased ICP.37 In general, two approaches exist: (a) strip craniectomy procedures and (b) remodeling procedures. Simply put, strip craniectomy procedures remove the synostotic suture in order to disinhibit cranial growth across the affected suture. Adjunc-tive techniques, such as cranial spring or distractor placement versus postoperative helmet therapy are frequently combined with strip craniectomies to improve aesthetic outcomes. Many surgeons who perform these procedures will do so as early as Brunicardi_Ch45_p1967-p2026.indd 199201/03/19 6:28 PM 1993PLASTIC


Surgery_Schwartz

Surgery_Schwartz. with strip craniectomies to improve aesthetic outcomes. Many surgeons who perform these procedures will do so as early as Brunicardi_Ch45_p1967-p2026.indd 199201/03/19 6:28 PM 1993PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45NormocephalyScaphocephalyTrigonocephaly AnteriorplagiocephalyBrachycephalyPosteriorplagiocephalyFigure 45-39. Patterns of single-suture cranio-synostosis. Scaphocephaly results from sagittal synostosis. Trigonocephaly results from metopic synostosis. Anterior plagiocephaly results from unilateral coronal synostosis. Brachycephaly results from bilateral coronal synostosis. Posterior plagiocephaly results from unilateral lambdoidal synostosis.6 to 12 weeks of life to take advantage of early rapid brain growth, which helps drive cranial expansion after release of the synostotic suture. In addition, younger patients have a better capacity to heal the resulting cranial defects due to the high osteogeneticity of the underlying dura, which decreases substan-tially


Surgery_Schwartz

Surgery_Schwartz. synostotic suture. In addition, younger patients have a better capacity to heal the resulting cranial defects due to the high osteogeneticity of the underlying dura, which decreases substan-tially with age.37 Remodeling procedures go further to normalize head shape by complete removal, rearrangement, and replace-ment of abnormal areas of the calvarium. Given the limited efficacy of the aforementioned strip craniectomy techniques in patients older than 6 months of age, cranial vault remodeling is generally accepted as the definitive treatment for craniosynos-tosis in this age group.36Advantages of strip craniectomy procedures include shorter operative times, less blood loss, and shorter hospital stays, while disadvantages include an inability to treat complex deformities from multisuture involvement, inability to treat areas of compensatory increased cranial growth, and the neces-sity for secondary hardware removal procedures. Remodeling procedures offer a more definitive correction of


Surgery_Schwartz

Surgery_Schwartz. inability to treat areas of compensatory increased cranial growth, and the neces-sity for secondary hardware removal procedures. Remodeling procedures offer a more definitive correction of head shape in a single surgical procedure at the cost of increased operative times, higher rate of blood transfusions, and increased length of hospital stays.37The complexity of patients with syndromic craniosynosto-ses, such as Crouzon or Apert syndrome, mandates multidisci-plinary care from an experienced team of subspecialists. These patients may present with urgent airway obstruction, danger-ously elevated ICP, and/or vision-threatening globe protrusion (Fig. 45-40A–C).23 Early surgical interventions, such as strip craniectomy or posterior cranial vault distraction, are designed to increase cranial volume and therefore decrease ICP. Although optimal timing of definitive reconstruction is debatable, results of cranial vault remodeling and midface advancement surgeries appear more stable and


Surgery_Schwartz

Surgery_Schwartz. volume and therefore decrease ICP. Although optimal timing of definitive reconstruction is debatable, results of cranial vault remodeling and midface advancement surgeries appear more stable and demonstrate less relapse when delayed.32 Hearing, speech, and feeding difficulties are common among patients with syndromic craniosynostoses. As always, the psy-chosocial implications of such profound facial differences make social workers and psychologists indispensable members of the team.23Atrophy and Hypoplasia. Two conditions that exemplify the atrophy and hypoplasia class of craniofacial anomalies are progressive hemifacial atrophy and Robin sequence. Progres-sive hemifacial atrophy, otherwise known as Parry-Romberg syndrome, is a rare, acquired, idiopathic atrophy of the skin, subcutaneous tissue, muscle, and occasionally bone affecting one side of the face (Fig. 45-41). With a typical onset during the first or second decade of life, this self-limiting condition progresses with an


Surgery_Schwartz

Surgery_Schwartz. tissue, muscle, and occasionally bone affecting one side of the face (Fig. 45-41). With a typical onset during the first or second decade of life, this self-limiting condition progresses with an indolent course for 2 to 10 years before sta-bilizing, or “burning out.” The pathogenesis of Parry-Romberg syndrome is not well understood. Autoimmune processes such as scleroderma, chronic neurotropic viral infections, trigeminal neuritis, intracerebral vascular malformations, and increased sympathetic nerve activity have all been postulated to play a role. After progression of atrophy ceases, the mainstay of treat-ment is volume and contour restoration with autologous fat grafting. More severe cases may require microvascular transfer of free tissue, such as the parascapular fasciocutaneous flap.33Robin sequence is defined as the triad of micrognathia, glossoptosis, and airway obstruction (Fig. 45-42).23 Cleft palate is present in up to 90% of affected patients, though it is not an obligatory


Surgery_Schwartz

Surgery_Schwartz. sequence is defined as the triad of micrognathia, glossoptosis, and airway obstruction (Fig. 45-42).23 Cleft palate is present in up to 90% of affected patients, though it is not an obligatory component of the diagnosis. The cause of this condi-tion is not known, but many believe mandibular hypoplasia to be the inciting event. According to this theory, micrognathia (small jaw) prevents forward migration of the tongue during gestational development. Glossoptosis results, where the tongue remains flipped dorsally into an obstructive position within the oropharyngeal airway. The first step in management is prone positioning, which utilizes gravity to bring the mandible and tongue base forward and alleviate the upper airway obstruction. More severely affected babies may require emergent endotra-cheal intubation at the time of delivery in order to secure the airway.34A diagnosable syndrome can be expected in over 50% of patients born with Robin sequence. Stickler syndrome (congeni-tal


Surgery_Schwartz

Surgery_Schwartz. intubation at the time of delivery in order to secure the airway.34A diagnosable syndrome can be expected in over 50% of patients born with Robin sequence. Stickler syndrome (congeni-tal ocular, orofacial, auditory, and articular anomalies), which is the leading cause of childhood blindness due to retinal detach-ment, is the most commonly associated syndrome. For this reason, ophthalmology and genetics evaluations are indicated in all patients with Robin sequence. Additionally, a thorough airway evaluation by an otolaryngologist is necessary to con-firm obstruction at the level of the tongue base and to rule out intrinsic airway anomalies or obstruction at lower levels of the respiratory tract.41Babies who are mildly affected can often be managed nonsurgically with prone positioning alone. Close monitoring is required because obstructive symptoms do not always fol-low a linear course to resolution. High caloric expenditure on Brunicardi_Ch45_p1967-p2026.indd 199301/03/19 6:28 PM


Surgery_Schwartz

Surgery_Schwartz. Close monitoring is required because obstructive symptoms do not always fol-low a linear course to resolution. High caloric expenditure on Brunicardi_Ch45_p1967-p2026.indd 199301/03/19 6:28 PM 1994SPECIFIC CONSIDERATIONSPART IIABCFigure 45-40. A and B. Frontal and lateral views of a young girl affected by Crouzon syndrome. Brachycephaly is appreciable on the lateral view, which results from bicoronal craniosynostosis. This patient also exhibits exorbitism and significant midface hyposplasia. C. A patient with Crouzon syndrome whose severe exorbitism has led to exposure keratitis.Brunicardi_Ch45_p1967-p2026.indd 199401/03/19 6:29 PM 1995PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45Figure 45-41. Child with progressive hemifacial atrophy, other-wise known as Parry-Romberg syndrome.Figure 45-42. An infant with Robin sequence. Marked microgna-thia and glossoptosis cause respiratory distress due to upper airway obstruction at the level of the tongue base. Note the presence of sternal


Surgery_Schwartz

Surgery_Schwartz. 45-42. An infant with Robin sequence. Marked microgna-thia and glossoptosis cause respiratory distress due to upper airway obstruction at the level of the tongue base. Note the presence of sternal retraction during inspiration.increased work of breathing, in combination with reflux and feeding difficulties that are ubiquitous in this population, may manifest as poor weight gain over time. Persistent failure to thrive indicates a failure of conservative management.41Robin sequence patients with single-level obstruction at the tongue base who have failed conservative measures should be considered for surgical airway management.41 Tongue-lip adhesion (TLA) is designed to bring the tongue base forward and out of the airway by temporarily sewing the under-surface of the tongue to the mucosal surface of the lower lip. Adhesions are typically reversed within the first year of life as significant mandibular growth and improved muscle tone of the tongue result in a stable airway.35Another


Surgery_Schwartz

Surgery_Schwartz. surface of the lower lip. Adhesions are typically reversed within the first year of life as significant mandibular growth and improved muscle tone of the tongue result in a stable airway.35Another option to treat upper airway obstruction in patients with Robin sequence is mandibular distraction osteogenesis (MDO). In this procedure, osteotomies are made in bilateral mandibular rami, and distractor devices are applied that enable a gradual (1–2 mm/day) lengthening of the mandible. As the mandible is brought forward, the tongue base follows, result-ing in enlargement of the oropharyngeal airway. Specific risks include injury to tooth buds, inferior alveolar or marginal man-dibular nerves, and disruption of mandibular growth potential.41In Robin sequence, patients who fail or are not candidates for less invasive surgical maneuvers, tracheostomy remains the definitive option for airway control. Figure 45-43 represents an algorithm for management of children with Robin sequence proposed on


Surgery_Schwartz

Surgery_Schwartz. for less invasive surgical maneuvers, tracheostomy remains the definitive option for airway control. Figure 45-43 represents an algorithm for management of children with Robin sequence proposed on the basis that TLA is less invasive and does not preclude subsequent MDO in the event of failure.42 However, 4one option has not been proven to be significantly better than the other, and many surgeons prefer MDO as a first-line intervention.Hypertrophy, Hyperplasia, and Neoplasia. Numerous hypertrophic, hyperplastic, or neoplastic processes can affect the craniofacial region. The presence of certain vascular anomalies in the face can result in hypertrophy of surrounding bone or soft tissue.19 Patients with neurofibromatosis-1 may similarly present with hemifacial hypertrophy related to the presence of an underlying plexiform neurofibroma.36 Fibrous dysplasia is a focal error in osteoblast differentiation that leads to replacement of normal bone with a disorganized mass of bony trabeculae


Surgery_Schwartz

Surgery_Schwartz. of an underlying plexiform neurofibroma.36 Fibrous dysplasia is a focal error in osteoblast differentiation that leads to replacement of normal bone with a disorganized mass of bony trabeculae and fibrous tissue. Seventy percent of lesions are monostotic, and MandibulardistractionosteogenesisLaryngotrachealanomaly?Treat anomaly +/– tracheostomyPronepositioningObservationTongue-lip adhesionObservationFigure 45-43. Algorithm for management of children with Robin sequence.Brunicardi_Ch45_p1967-p2026.indd 199501/03/19 6:29 PM 1996SPECIFIC CONSIDERATIONSPART IIthe remaining 30% are polyostotic. In the craniofacial region, fibrous dysplasia typically presents in childhood with pain and progressive asymmetry. Patients with McCune-Albright syn-drome have polyostotic fibrous dysplasia, café au lait spots, and hyperfunctioning endocrinopathies, which classically manifest as precocious puberty. Lesions have a distinct “ground glass” appearance on CT scan. Small, monostotic fibrous dysplasia


Surgery_Schwartz

Surgery_Schwartz. lait spots, and hyperfunctioning endocrinopathies, which classically manifest as precocious puberty. Lesions have a distinct “ground glass” appearance on CT scan. Small, monostotic fibrous dysplasia lesions can occasionally be resected completely and recon-structed with bone grafts. More commonly, surgical debulking and contouring is the treatment of choice.37Vascular Anomalies. Vascular anomalies affect approxi-mately 5.5% of the population. They can be broadly categorized as either tumors or malformations.38 Vascular tumors are char-acterized histologically by endothelial cell proliferation, with or without luminal structure. In contrast, vascular malformations are collections of abnormally developed vessels without signifi-cant endothelial cell turnover.39Hemangiomas Hemangiomas are the most common vascular tumor in children, presenting in up to 20% of premature infants. Females are four times as likely to be affected as males, and darker-skinned individuals are rarely affected.


Surgery_Schwartz

Surgery_Schwartz. the most common vascular tumor in children, presenting in up to 20% of premature infants. Females are four times as likely to be affected as males, and darker-skinned individuals are rarely affected. These benign tumors are believed to be collections of primitive blood vessels formed from angioblasts. Hemangiomas can occur anywhere throughout the body, with the liver being the most common extracutaneous site.46The natural history of hemangiomas is highly predict-able depending on the timing of presentation and early clinical course. Infantile hemangiomas appear shortly after birth, usu-ally between 2 weeks and 2 months of life. Cutaneous infantile hemangiomas may initially resemble a red scratch or bruise, while subcutaneous or visceral lesions go unnoticed. Rapid growth ensues over the next 9 to 12 months (“the proliferative phase”). During this time, cutaneous lesions become bright red and tense, while subcutaneous lesions may present as deep soft tissue masses with a


Surgery_Schwartz

Surgery_Schwartz. over the next 9 to 12 months (“the proliferative phase”). During this time, cutaneous lesions become bright red and tense, while subcutaneous lesions may present as deep soft tissue masses with a bluish/purplish hue. After plateau of the proliferative phase, infantile hemangiomas reliably undergo a slow regression (“involution”), which is usually complete by 4 years of age. History alone can help differentiate a congenital hemangioma, which is fully formed at birth, from an infantile one. Congenital hemangiomas may exhibit rapidly involuting (RICH), noninvoluting (NICH), or partially involuting (PICH) clinical courses. History and physical is often sufficient to diagnose a hemangioma. Doppler ultrasound has become the imaging modality of choice, while MRI is typically reserved to confirm the diagnosis in cases of uncertainty.40Most hemangiomas can be observed and allowed to invo-lute spontaneously. High-risk lesions that may require early intervention include ulcerated and bleeding


Surgery_Schwartz

Surgery_Schwartz. the diagnosis in cases of uncertainty.40Most hemangiomas can be observed and allowed to invo-lute spontaneously. High-risk lesions that may require early intervention include ulcerated and bleeding hemangiomas; periocular hemangiomas, which can occlude the visual axis and lead to blindness; hemangiomas in the beard distribution, which place the patient at risk for upper airway obstruction (Fig. 45-44); and posterior midline lumbosacral hemangiomas, which may indicate underlying spinal dysraphism and cause cord compression. Patients with three or more hemangiomas should be screened by ultrasound for involvement of abdomi-nal viscera, as large hepatic lesions may lead to high-output heart failure. Large segmental hemangiomas in the cranial nerve V distribution (Fig. 45-45) should raise suspicion for PHACES association (Posterior fossa malformations, Heman-giomas, Arterial anomalies, Cardiac defects, Eye anomalies, Sternal defects).46 The LUMBAR association (Lower body Figure


Surgery_Schwartz

Surgery_Schwartz. raise suspicion for PHACES association (Posterior fossa malformations, Heman-giomas, Arterial anomalies, Cardiac defects, Eye anomalies, Sternal defects).46 The LUMBAR association (Lower body Figure 45-44. Hemangiomas in the beard distribution.hemangiomas, Urogenital anomalies, Myelopathy, Bony defor-mities, Anorectal/Arterial malformations, Renal anomalies) should be considered in patients with large infantile hemangio-mas of the lumbosacral region or lower extremities.41Oral propranolol therapy has emerged as the first-line treatment for complicated or high-risk infantile hemangio-mas. When administered during the proliferative phase, this nonselective beta adrenergic receptor blocker causes rapid invo-lution of the hemangioma. Several randomized, controlled trials have demonstrated oral propranolol to cause a greater decrease in lesion size compared to placebo and steroid therapy.42 In addition, many clinicians believe the side effect profile of pro-pranolol (hypoglycemia, sleep


Surgery_Schwartz

Surgery_Schwartz. propranolol to cause a greater decrease in lesion size compared to placebo and steroid therapy.42 In addition, many clinicians believe the side effect profile of pro-pranolol (hypoglycemia, sleep disturbances, hypotension, bra-dycardia, bronchospasm) to be more favorable than that of systemic steroids.43While hemangioma involution may result in no visible sequelae, up to 50% of patients are left with a residual fibrofatty mass with atrophic, hypopigmented and/or telangiectatic over-lying skin (Fig. 45-46A,B). If the residual deformity is troubling to the patient, surgical excision may be indicated.46Vascular Malformations Vascular malformations are collec-tions of abnormally formed vessels that demonstrate minimal endothelial cell turnover. They are present at birth and grow slowly in proportion with the patient. Vascular malformations are classified on the basis of anatomic origin of the abnormal vessels: capillary malformations (CM), venous malformations (VM), lymphatic


Surgery_Schwartz

Surgery_Schwartz. in proportion with the patient. Vascular malformations are classified on the basis of anatomic origin of the abnormal vessels: capillary malformations (CM), venous malformations (VM), lymphatic malformations (LM), and arteriovenous mal-formations (AVM). These classes can be further categorized into “slow-flow” or “fast-flow” lesions (Table 45-4).46Capillary malformations, formerly known as “port wine stains,” present at birth as flat, pink patches of skin. They typi-cally darken with age and may develop a thickened or “cob-blestoned” appearance. CMs may be found anywhere on the body, and overgrowth of underlying soft tissue or bone can occur. History and physical is sufficient to diagnose isolated CMs, but syndromic associations do exist that would warrant 5Brunicardi_Ch45_p1967-p2026.indd 199601/03/19 6:29 PM 1997PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45Figure 45-45. Large segmental hemangiomas in the cranial nerve V distribution.Figure 45-46. Twenty-year-old female with a


Surgery_Schwartz

Surgery_Schwartz. 199601/03/19 6:29 PM 1997PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45Figure 45-45. Large segmental hemangiomas in the cranial nerve V distribution.Figure 45-46. Twenty-year-old female with a capillary malformations of the right cheek. A. Before and (B) after pulsed-dye laser treatment.ABTable 45-4Classification of vascular malformationsSLOW FLOWFAST FLOWCapillary malformationsVenous malformationsLymphatic malformationsArteriovenous malformationsfurther work-up.46 Sturge-Weber syndrome often presents with CMs in the V1/V2 nerve distributions of the face and may be accompanied by vascular malformations of the underlying lep-tomeninges or globe. Patients are at high risk for seizure, stroke, and glaucoma, for which pharmacologic prophylaxis may be indicated.44 The mainstay of treatment of CMs is pulsed-dye laser therapy (Fig. 45-47A, pre procedure; Fig. 45-47B post pro-cedure). Other surgical interventions, if necessary, are aimed at addressing soft tissue or bony overgrowth.46Venous


Surgery_Schwartz

Surgery_Schwartz. CMs is pulsed-dye laser therapy (Fig. 45-47A, pre procedure; Fig. 45-47B post pro-cedure). Other surgical interventions, if necessary, are aimed at addressing soft tissue or bony overgrowth.46Venous malformations are lobulated collections of dilated veins that typically involve skin, mucosa, or subcutaneous tis-sue, although 50% demonstrate deeper involvement. Lesions may or may not be noted at the time of birth. VMs generally grow in proportion to the patient but may undergo accelerated growth during puberty or pregnancy. Swelling of the mass may occur with dependent positioning or Valsalva maneuvers, such as crying. On exam, superficial VMs are soft, compressible masses with a bluish hue. Firm, tender nodules may be present, which represent calcifications known as phleboliths. Deeper, intramuscular VMs may present with pain or increased extrem-ity circumference, while lesions of the GI tract may simply pres-ent with bleeding. MRI with contrast is the imaging modality of choice,


Surgery_Schwartz

Surgery_Schwartz. intramuscular VMs may present with pain or increased extrem-ity circumference, while lesions of the GI tract may simply pres-ent with bleeding. MRI with contrast is the imaging modality of choice, although ultrasound can be used in infants and young children to avoid sedation. Observation is indicated for asymp-tomatic lesions. Compression of involved extremities helps alleviate pain and swelling and prevent thrombosis and phlebo-lith formation. Due to the high risk of recurrence after surgi-cal excision, the first line of treatment for symptomatic VMs is sclerotherapy. Surgery is reserved for small, well-localized lesions amenable to complete resection; extremity lesions near major peripheral nerves; or residual deformities after sclero-therapy (Fig. 45-48A, before laser; Fig. 45-48B, after laser; and Fig. 45-48C, after limited resection).46Brunicardi_Ch45_p1967-p2026.indd 199701/03/19 6:29 PM 1998SPECIFIC CONSIDERATIONSPART IIABABCFigure 45-47. A. A 3-year-old patient with an


Surgery_Schwartz

Surgery_Schwartz. laser; and Fig. 45-48C, after limited resection).46Brunicardi_Ch45_p1967-p2026.indd 199701/03/19 6:29 PM 1998SPECIFIC CONSIDERATIONSPART IIABABCFigure 45-47. A. A 3-year-old patient with an involuting hem-angioma of the right cheek. B. The same patient at 8 years of age showing minimal sequelae after completion of involution.Figure 45-48. A 5-year-old boy with venous malformation of the lower lip. A. Initial presentation. B. After three sclerotherapy treat-ments. C. Six weeks after surgical debulking of residual fibrotic tissue.Brunicardi_Ch45_p1967-p2026.indd 199801/03/19 6:29 PM 1999PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45Figure 45-49. A. Lymphatic malformation of the neck. B. After sclerotherapy with significant skin excess. C. Seven months after resection of excess skin.Lymphatic malformations, previously referred to as “cys-tic hygromas,” are collections of abnormal lymph channels that may cross multiple tissue planes and cause swelling, pain, bleeding, or bony


Surgery_Schwartz

Surgery_Schwartz. malformations, previously referred to as “cys-tic hygromas,” are collections of abnormal lymph channels that may cross multiple tissue planes and cause swelling, pain, bleeding, or bony overgrowth. LMs are classified as macrocys-tic, microcystic or combined. Large, macrocystic lesions can alter form and impair function locally through mass effect. Cuta-neous components of LMs present as vesicles that may bleed or become infected. While superficial lesions can be diagnosed by history and physical exam alone, deeper lesions require MRI ABCto confirm the diagnosis and assess the extent of the disease. Asymptomatic LMs can be observed. Sclerotherapy is the treat-ment of choice for all macrocysts. Symptomatic microcystic LMs have been treated with oral sirolimus, and draining cutane-ous vesicles have been successfully ablated with CO2 laser ther-apy. Recurrence after surgery is common; therefore, excision is reserved for severely symptomatic lesions no longer amenable to sclerotherapy or


Surgery_Schwartz

Surgery_Schwartz. have been successfully ablated with CO2 laser ther-apy. Recurrence after surgery is common; therefore, excision is reserved for severely symptomatic lesions no longer amenable to sclerotherapy or small, well-localized lesions where excision can be curative (Fig. 45-49A–C).46Brunicardi_Ch45_p1967-p2026.indd 199901/03/19 6:30 PM 2000SPECIFIC CONSIDERATIONSPART IIArteriovenous malformations are abnormal vascular con-nections between arteries and veins without intervening capil-lary beds. AVMs involving the skin appear pink and are warm to the touch. A palpable pulse or thrill may be present from the fast-flow shunting of blood from arterial to venous circu-lation. Lack of local capillaries can cause a painful, ischemic ulceration of the skin. Patients with large AVMs are at risk for development of congestive heart failure. Doppler ultrasound is the imaging modality of choice, but MRI is often obtained to provide additional information on the extent of the lesion. Observation is


Surgery_Schwartz

Surgery_Schwartz. development of congestive heart failure. Doppler ultrasound is the imaging modality of choice, but MRI is often obtained to provide additional information on the extent of the lesion. Observation is appropriate for asymptomatic AVMs. For symp-tomatic AVMs, embolization is frequently employed 24 to 72 hours prior to excision to minimize operative blood loss. Excision or embolization alone is rarely curative and highly likely to recur. Indications for surgery include small, well-localized AVMs; focal deformities that result from an AVM; or symptomatic AVMs not amenable to embolization.46When multiple types of vascular malformations cohabi-tate, they are collectively referred to as combined malforma-tions. Patients with Klippel-Trenaunay syndrome demonstrate a combined capillary, venous, and lymphatic malformation of an extremity resulting in bony and/or soft tissue overgrowth (Fig. 45-50).45Figure 45-50. A patient with Klippel-Trenaunay syndrome involv-ing the right lower extremity.


Surgery_Schwartz

Surgery_Schwartz. lymphatic malformation of an extremity resulting in bony and/or soft tissue overgrowth (Fig. 45-50).45Figure 45-50. A patient with Klippel-Trenaunay syndrome involv-ing the right lower extremity. The combined capillary, venous, and lymphatic malformations result in generalized overgrowth of the extremity.Table 45-5Classification of CMN’sPROJECTED ADULT DIAMETERCMN CLASSIFICATION<1.5 cmSmall≥1.5 cm and <11 cmMedium≥11 cm and ≤20 cmLarge>20 cmGiantCongenital Melanocytic Nevi. Congenital melanocytic nevi (CMN) are hyperpigmented lesions present at birth that result from ectopic rests of melanocytes within the skin. They can be distinguished histologically from acquired nevi by their exten-sion into the deep dermis, subcutaneous tissue, or muscle.46 Depending on their size and location, CMNs may cause severe disfigurement and accompanying psychologic distress. Classi-fication is based on projected diameter of the largest dimension on the fully-grown adult (Table 45-5)47. While CMNs are


Surgery_Schwartz

Surgery_Schwartz. may cause severe disfigurement and accompanying psychologic distress. Classi-fication is based on projected diameter of the largest dimension on the fully-grown adult (Table 45-5)47. While CMNs are gener-ally common (1% incidence), only 1 in 20,000 children are born with a giant lesion. At birth, CMNs often appear flat, brown and hairless. They grow in proportion with the patient and may develop color variegation, verrucous thickening, hypertrichosis, erosions, or ulcerations over time. CMNs carry an estimated 0.7% to 2.9% lifetime risk of melanoma, with the majority of cases presenting before puberty. Patients with giant CMNs, multiple satellite lesions, or trunk lesions appear to be at higher risk for malignancy. Melanomas can develop within the CMN itself, but they may also present as primary cancers at distant, extra-cutaneous sites, such as the GI tract or the central nervous system. Patients with CMNs require regular skin surveillance by a dermatologist. A biopsy is indicated


Surgery_Schwartz

Surgery_Schwartz. primary cancers at distant, extra-cutaneous sites, such as the GI tract or the central nervous system. Patients with CMNs require regular skin surveillance by a dermatologist. A biopsy is indicated for concerning changes in color or shape, nodularity, or ulceration. If melanoma is diag-nosed, management should proceed in accordance with standard melanoma treatment guidelines.55CMNs with multiple (>20) satellite lesions or midline CMNs over the trunk or calvaria should raise suspicion for neu-rocutaneous melanosis, a condition resulting from melanoblast proliferation in the central nervous system (CNS). In addition to the risk of CNS melanoma, patients with neurocutaneous melanosis may suffer from developmental delay, seizures, intracranial hemorrhages, hydrocephalus, cranial nerve palsies, or tethered spinal cord. High-risk patients should be evaluated by MRI between 4 and 6 months of age. While asymptomatic patients may be followed with serial MRI, patients with symp-tomatic


Surgery_Schwartz

Surgery_Schwartz. palsies, or tethered spinal cord. High-risk patients should be evaluated by MRI between 4 and 6 months of age. While asymptomatic patients may be followed with serial MRI, patients with symp-tomatic neurocutaneous melanosis often succumb to their dis-ease within 2 to 3 years of diagnosis.54The goals in surgical management of CMN are (a) to decrease cancer risk, (b) to reduce symptoms, (c) to improve appearance, (d) to improve psychosocial health, and (e) to maintain function.54 It is important to note that the risk of mela-noma is not eliminated even with complete excision of a CMN. Indeed, a definitive cancer risk reduction from surgical excision of CMNs has yet to be proven. Management paradigms have therefore shifted from complete excision and reconstruction to maximal excision and reconstruction without compromis-ing function or aesthetic outcome.55 From serial excisions or skin grafting, to tissue expansion or free tissue transfer, plastic surgeons have drawn from the entire


Surgery_Schwartz

Surgery_Schwartz. reconstruction without compromis-ing function or aesthetic outcome.55 From serial excisions or skin grafting, to tissue expansion or free tissue transfer, plastic surgeons have drawn from the entire armamentarium in meet-ing the substantial reconstructive challenges posed by giant CMNs. Treatment plans must be grounded in principle: “tissue Brunicardi_Ch45_p1967-p2026.indd 200001/03/19 6:30 PM 2001PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45losses should be replaced in kind,” and “reconstruct by units.”48 Figure 45-51A–C shows an infant with a giant CMN of the pos-terior trunk and right flank preoperatively; at end of the first round of tissue expansion; and at the culmination of three rounds of tissue expansion, excision, and closure by local tissue rearrangement.49Figure 45-51. A. An infant with a giant CMN of the posterior trunk and right flank. B. Tissues expanders were placed under adjacent normal skin in preparation for first-stage excision. C. The same patient at 11 years


Surgery_Schwartz

Surgery_Schwartz. An infant with a giant CMN of the posterior trunk and right flank. B. Tissues expanders were placed under adjacent normal skin in preparation for first-stage excision. C. The same patient at 11 years of age after three rounds of tissue expansion and excision.ABCRECONSTRUCTIVE SURGERY IN ADULTSReconstructive surgery applies techniques that modify tissues in order to restore a normal function and appearance in a patient with congenital or acquired deformities. The most common causes of acquired deformities are traumatic injuries and cancer.Brunicardi_Ch45_p1967-p2026.indd 200101/03/19 6:30 PM 2002SPECIFIC CONSIDERATIONSPART IIWe will focus first on trauma. Although any anatomic region can be subjected to injuries that might require reconstruc-tive surgery, traumatic fractures, and soft tissue damage in the head and neck and extremities are most common. The manner in which these reconstructive steps are conducted is criti-cal. Reconstructive surgery involves the coordination of many


Surgery_Schwartz

Surgery_Schwartz. damage in the head and neck and extremities are most common. The manner in which these reconstructive steps are conducted is criti-cal. Reconstructive surgery involves the coordination of many specialties and must occur according to a particular time-line, involving complex system-based practice.Maxillofacial Injuries and FracturesManagement of maxillofacial injuries typically occurs in the context of multiple trauma. Concomitant injuries beyond the face are the rule rather than the exception. The first phase of care is activation of the advanced trauma life support proto-cols. The most common life-threatening considerations in the facial trauma patient are airway maintenance, control of bleed-ing, identification and treatment of aspiration, assessment for closed head injuries, and identification of other injuries. Once the patient’s condition has been stabilized and life-threatening injuries managed, attention is directed to diagnosis and manage-ment of craniofacial


Surgery_Schwartz

Surgery_Schwartz. and identification of other injuries. Once the patient’s condition has been stabilized and life-threatening injuries managed, attention is directed to diagnosis and manage-ment of craniofacial injuries.Physical examination of the face focuses first on assess-ment of soft tissue injuries as manifested by surface contusions and lacerations. Part of this process is intranasal and intraoral examination. Associated injuries to the underlying facial skel-eton are determined by observation, palpation, and digital bone examination through open lacerations. Signs of a facial frac-ture include contour abnormalities, irregularities of normally smooth contours such as the orbital rims or inferior border of the mandible, instability, tenderness, ecchymosis, facial asym-metry, or displacement of facial landmarks. Traditional plain radiographs have largely been replaced by high-resolution CT, which is widely available at emergency centers that typically receive these patients. Reformatting raw scans


Surgery_Schwartz

Surgery_Schwartz. landmarks. Traditional plain radiographs have largely been replaced by high-resolution CT, which is widely available at emergency centers that typically receive these patients. Reformatting raw scans into coronal, sag-ittal, and 3D views is a valuable method to elucidate and plan treatment for complex injuries.The facial skeleton can be divided into the upper third, middle third, and lower third. The upper third is comprised bounded inferiorly by the superior orbital rim and is formed by the frontal bone. The middle third is the most complex and is formed primarily by the maxilla, nasal bones, and zygoma. The lower third is inferior to the oral cavity and is formed by the mandible. The functional structure of the midface may be understood as a system of buttresses formed by the frontal, maxillary, zygomatic, and sphenoid bones. These buttresses are oriented vertically and horizontally and distribute forces applied to the bones in order to maintain their shape and position with-out


Surgery_Schwartz

Surgery_Schwartz. maxillary, zygomatic, and sphenoid bones. These buttresses are oriented vertically and horizontally and distribute forces applied to the bones in order to maintain their shape and position with-out fracturing. There are three paired vertical buttresses called the nasomaxillary, zygomaticomaxillary, and pterygomaxillary buttresses. The horizontal buttresses of the midface pass through the superior and inferior orbital rims and hard palate. A guiding principle of facial facture management is to restore the integrity of these buttresses.Mandible FracturesMandibular fractures are common injuries that may lead to permanent disability if not diagnosed and properly treated. The mandibular angle, ramus, coronoid process, and condyle are points of attachment for the muscles of mastication, including the masseter, temporalis, lateral pterygoid, and medial pterygoid muscles (Fig. 45-52). Fractures are frequently multiple. Altera-tions in dental occlusion usually accompany mandible fractures.


Surgery_Schwartz

Surgery_Schwartz. the masseter, temporalis, lateral pterygoid, and medial pterygoid muscles (Fig. 45-52). Fractures are frequently multiple. Altera-tions in dental occlusion usually accompany mandible fractures. Malocclusion is caused by forces exerted on the mandible of the 6CoronoidprocessRamusAngleBodySymphysisCondyleFigure 45-52. Mandibular anatomy.many muscles of mastication on the fracture segments. Den-tal occlusion is perhaps the most important basic relationship to understand about fracture of the midface and mandible. The Angle classification system describes the relationship of the maxillary teeth to the mandibular teeth. Class I is normal occlu-sion, with the mesial buccal cusp of the first maxillary molar fitting into the intercuspal groove of the mandibular first molar. Class II malocclusion is characterized by anterior (mesial) posi-tioning, and class III malocclusion is posterior (distal) posi-tioning of the maxillary teeth with respect to the mandibular teeth (Fig. 45-53). These


Surgery_Schwartz

Surgery_Schwartz. is characterized by anterior (mesial) posi-tioning, and class III malocclusion is posterior (distal) posi-tioning of the maxillary teeth with respect to the mandibular teeth (Fig. 45-53). These occlusal relationships guide clinical management.The goals of surgical treatment include restoration of den-tal occlusion, fracture reduction and stable fixation, and soft Figure 45-53. Angle classification. Class I: The mesial buccal cusp of the maxillary first molar fits into the intercuspal groove of the mandibular first molar. Class II: The mesial buccal cusp of the maxillary first molar is mesial to the intercuspal groove of the mandibular first molar. Class III: The mesial buccal cusp of the maxillary first molar is distal to the intercuspal groove of the man-dibular first molar.IIIIIIBrunicardi_Ch45_p1967-p2026.indd 200201/03/19 6:30 PM 2003PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45tissue repair. Nonsurgical treatment may be used in situations in which there is minimal


Surgery_Schwartz

Surgery_Schwartz. 200201/03/19 6:30 PM 2003PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45tissue repair. Nonsurgical treatment may be used in situations in which there is minimal displacement, preservation of the pretraumatic occlusive relationship, normal range of motion, and no significant soft tissue injury. Operative repair involves first establishing and stabilizing dental occlusion and holding in place with maxillomandibular fixation to stabilize the relation-ships between the mandible and maxilla. The simplest method for this is to apply arch bars to the maxillary and mandibular teeth then use secure them together using interdental wires. Alternatives are sometimes indicated (e.g., screws placed into the bone of the maxilla and mandible that serve as posts for spanning the maxilla and mandible with wires), especially for patients with poor dentition. Once the dental relationships are established, then the fractures can then be reduced and fixed using wire or plates and screws that are specially


Surgery_Schwartz

Surgery_Schwartz. wires), especially for patients with poor dentition. Once the dental relationships are established, then the fractures can then be reduced and fixed using wire or plates and screws that are specially designed for this purpose. The fracture is surgically exposed using multiple incisions, depending on the location of the fracture and condi-tion of the soft tissues. The fracture is visualized and manually reduced. Fixation may be accomplished using traditional inter-fragment wires, but plating systems are generally superior. The mandibular plating approach follows two schools of thought: rigid fixation as espoused by the Association for Osteosynthe-sis/Association for the Study of Internal Fixation and less rigid but functionally stable fixation (Champy technique). Regardless of the approach, it is important to release maxillomandibular fixation and begin range of motion as soon as possible to pre-vent temporomandibular joint ankylosis. Fractures immediately inferior to the mandibular


Surgery_Schwartz

Surgery_Schwartz. it is important to release maxillomandibular fixation and begin range of motion as soon as possible to pre-vent temporomandibular joint ankylosis. Fractures immediately inferior to the mandibular condyles, called subcondylar frac-tures, are unique in that there is ordinarily minimal displace-ment because the fragments are less subject to displacement from muscle forces and there is little bone available across the ClosedOpenYesYesNoNoAnteriortable onlyAnterior andposteriortables ObservationAnterior ORIFAnterior ORIFAnterior ORIFCranialization of sinusObliteration of NF ductbone grafting orificefat/fascial grafting orificeflap coverage of cavityremoval of posterior tableburring of mucosa-----ExplorationEstablish DiagnosisPhysical examCT scanDepressed?CSF leak ordisplacedposterior wall?Figure 45-54. Algorithm for the treatment of frontal sinus fracture. CSF = cerebrospinal fluid; CT = computed tomography; NF = nasofrontal; ORIF = open reduction, internal fixation.fracture line to permit


Surgery_Schwartz

Surgery_Schwartz. 45-54. Algorithm for the treatment of frontal sinus fracture. CSF = cerebrospinal fluid; CT = computed tomography; NF = nasofrontal; ORIF = open reduction, internal fixation.fracture line to permit fixation. These are most often treated with maxillomandibular fixation alone.Important considerations in postoperative management are release from maxillary-mandibular fixation and resumption of range of motion as soon as possible to minimize the risk of tem-poromandibular joint ankylosis. Complications to be avoided include infection, nonunion, malunion, malocclusion, facial nerve injury, mental nerve injury, and dental fractures.Frontal Sinus FracturesThe frontal sinus is located in the upper third of the face. It is actually a paired structure ordinarily fused in the midline imme-diately superior to the orbital rims. It has an anterior bony table that defines the contour of the forehead and a posterior table that separates the sinus cavity from the underlying dura of the intra-cranial


Surgery_Schwartz

Surgery_Schwartz. to the orbital rims. It has an anterior bony table that defines the contour of the forehead and a posterior table that separates the sinus cavity from the underlying dura of the intra-cranial frontal fossa. The anterior table is a relatively weak and subject to fracture when it sustains a direct forceful blow, mak-ing frontal sinus fractures relatively common in facial trauma. Each sinus drains through the medial floor into its frontonasal duct, which empties into the middle meatus within the nose.Treatment of a frontal sinus fracture depends on the frac-ture characteristics as shown in the algorithm (Fig. 45-54). The diagnosis is established by physical examination and confirmed by CT scan. Closed fractures that are not depressed and caus-ing a visible deformity may be observed. Depressed or open fractures must be explored. Fractures that involve only the anterior table are reduced and fixed using interosseous wires or miniature plates and screws. Fractures of the posterior table


Surgery_Schwartz

Surgery_Schwartz. or open fractures must be explored. Fractures that involve only the anterior table are reduced and fixed using interosseous wires or miniature plates and screws. Fractures of the posterior table without disruption of the dura evidenced by leaking cerebro-spinal fluid can be treated in similar fashion. When the dura is disrupted, excising the bone and mucosa or the posterior table Brunicardi_Ch45_p1967-p2026.indd 200301/03/19 6:30 PM 2004SPECIFIC CONSIDERATIONSPART IIand obliterating the nasofrontal duct with a local graft or flap converts with frontal sinus into the anterior frontal fossa of the cranial vault, “cranializing” it.Orbital FracturesTreatment of all orbital injuries begins with a careful examina-tion of the globe, which often is best completed by a specialist to assess visual acuity and ocular mobility and to rule out globe injury. Fractures may involve the orbital roof, the orbital floor, or the lateral or medial walls (Fig. 45-55). The most common fracture involves


Surgery_Schwartz

Surgery_Schwartz. acuity and ocular mobility and to rule out globe injury. Fractures may involve the orbital roof, the orbital floor, or the lateral or medial walls (Fig. 45-55). The most common fracture involves the floor because this is the weakest bone. This type of fracture is referred to as an orbital a “blow-out” frac-ture because the cause is usually direct impact to the globe that results in a sudden increase in intraorbital pressure with failure of the orbital floor. The typical history is either a direct blow Figure 45-55. Facial bone anatomy.FrontalTemporalSphenoidZygomaMaxillaSphenoidFrontalZygomaMaxillaTemporalABduring an altercation or a sports-related event with a small ball directly striking the orbit. Because the medial floor and inferior medial wall are made of the thinnest bone, fractures occur most frequently at these locations. These injuries may be treated with observation only if they are isolated and small without signs of displacement or limitation of mobility of the globe.


Surgery_Schwartz

Surgery_Schwartz. occur most frequently at these locations. These injuries may be treated with observation only if they are isolated and small without signs of displacement or limitation of mobility of the globe. However, surgical treatment is generally indicated for large fractures or ones associated with enophthalmos (retrusion of the globe), which suggests increased intraorbital volume and restriction of upward gaze on the injured side, with entrapment of inferior orbital tissues or double vision (diplopia) persisting greater than 2 weeks.28 There are a variety of options for surgical exposure of the orbital floor, including the transconjunctival, subciliary, and lower blepharoplasty incisions. All provide good access for accurate diagnosis and treatment, which involves reducing orbital contents and repairing the floor with either autologous bone or synthetic materials. Late complications include per-sistent diplopia, enophthalmos, or displacement of the lower eyelid ciliary margin inferiorly


Surgery_Schwartz

Surgery_Schwartz. repairing the floor with either autologous bone or synthetic materials. Late complications include per-sistent diplopia, enophthalmos, or displacement of the lower eyelid ciliary margin inferiorly (ectropion) or rolling inward (entropion). Entropion causes the eyelashes to brush constantly against the cornea and is very uncomfortable. Each of these sequelae has procedures for repair should they occur.Orbital floor fractures can be associated with fractures of the lateral or inferior orbital rim. These are typically a compo-nent of facial fractures that extend beyond the orbit involving the zygomatic and maxillary bones and are discussed in more detail in the next section.It is important to be aware of two adverse associated con-ditions seen at times in patients with orbital fractures. The first is superior orbital fissure syndrome. Cranial nerves III (oculo-motor nerve), IV (trochlear nerve), and VI (abducens nerve), and the first division of cranial nerve V (VI, trigeminal nerve)


Surgery_Schwartz

Surgery_Schwartz. The first is superior orbital fissure syndrome. Cranial nerves III (oculo-motor nerve), IV (trochlear nerve), and VI (abducens nerve), and the first division of cranial nerve V (VI, trigeminal nerve) pass into the orbit from the base of the skull and into the orbit through the superior orbital fissure. Direct fractures of the pos-terior orbit or localized swelling caused by a fracture nearby can cause compression of these nerves. Symptoms include eyelid ptosis, protrusion of the globe (proptosis), paralysis of the extra-ocular muscles, and anesthesia supraorbital and trochlear nerve distributions. The second condition to remember is orbital apex syndrome. This is the most severe circumstance in which supe-rior orbital fissure syndrome is combined with signs of optic nerve (cranial nerve II) compression manifested visual changes ranging up to complete blindness. This is a medical emergency that requires immediate treatment to prevent permanent loss of function.Zygomaticomaxillary


Surgery_Schwartz

Surgery_Schwartz. II) compression manifested visual changes ranging up to complete blindness. This is a medical emergency that requires immediate treatment to prevent permanent loss of function.Zygomaticomaxillary Complex FracturesThe zygoma defines the lateral contour of the middle third of the face and forms the lateral and inferior borders of the orbit. It articulates with the sphenoid bone in the lateral orbit, the maxilla medially and inferiorly, the frontal bone superiorly, and the temporal bone laterally. It forms the anterior portion of the zygomatic arch, articulating with the zygomatic projection of the temporal bone. The temporalis muscle, a major muscle of mastication, passes beneath the zygomatic arch and inserts on the coronoid process of the mandible.Fractures of the zygomatic bone may involve the zygo-matic arch alone or any of its other portions and bony relation-ships. Isolated arch fractures manifest as a flattened, wide facial appearance with edema and ecchymosis. Typically, they


Surgery_Schwartz

Surgery_Schwartz. the zygo-matic arch alone or any of its other portions and bony relation-ships. Isolated arch fractures manifest as a flattened, wide facial appearance with edema and ecchymosis. Typically, they are also associated with pain or limited mobility of the mandible. Nondisplaced fractures may be treated without surgery, but Brunicardi_Ch45_p1967-p2026.indd 200401/03/19 6:30 PM 2005PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45displaced or comminuted fractures should be reduced and stabi-lized. This can be accomplished using an indirect approach from above the hairline in the temporal scalp, the so-called “Gilles approach,” or directly through a coronal incision in severe fractures.A common fracture pattern is called the zygomaticomaxil-lary complex (ZMC) fracture. This involves the zygomatic arch, the inferior orbital rim, the zygomaticomaxillary buttress, the lateral orbital wall, and the zygomaticofrontal buttress. Muscle forces acting on the fracture segment tend to rotate it


Surgery_Schwartz

Surgery_Schwartz. arch, the inferior orbital rim, the zygomaticomaxillary buttress, the lateral orbital wall, and the zygomaticofrontal buttress. Muscle forces acting on the fracture segment tend to rotate it laterally and inferiorly, thereby expanding the orbital volume, limiting mandibular excursion, creating an inferior cant to the palpebral fissure, and flattening the malar eminence. ZMC fractures are almost always accompanied on physical examination by altered sensation in the infraorbital nerve distribution and a subconjunc-tival hematoma.Treatment of displaced ZMC fractures is surgical. Each fracture site is exposed through incisions strategically placed to gain access but minimize disfiguring facial scars afterwards. These include an incision in the upper eyelid, exposing the zygomaticofrontal buttress and lateral orbital wall; a subtarsal or transconjunctival incision in the lower eyelid, exposing the orbital floor and infraorbital rim; and a maxillary gingivobuc-cal sulcus incision, exposing


Surgery_Schwartz

Surgery_Schwartz. and lateral orbital wall; a subtarsal or transconjunctival incision in the lower eyelid, exposing the orbital floor and infraorbital rim; and a maxillary gingivobuc-cal sulcus incision, exposing the zygomaticomaxillary buttress. Severe fractures involving the arch require wide exposure through a coronal incision.Nasoorbitalethmoid and Panfacial FracturesNasoorbitalethmoid (NOE) fractures are defined anatomically by a combination of injuries that involve the medial orbits, the nasal bones, the nasal processes of the frontal bone, and the frontal processes of the maxilla. If improperly treated, these injuries cause severe disfigurement and functional deficits from nasal airway collapse, medial orbital disruption, displacement of medial canthus of the eyelids, and nasolacrimal apparatus dysfunction. Telecanthus is abnormally wide separation of the medical canthus of the eyelids and is produced by a splaying apart of the nasomaxillary buttresses to which the medial can-thal ligaments are


Surgery_Schwartz

Surgery_Schwartz. Telecanthus is abnormally wide separation of the medical canthus of the eyelids and is produced by a splaying apart of the nasomaxillary buttresses to which the medial can-thal ligaments are attached. NOE fractures require surgical man-agement with open reduction and internal fixation. At times, the thin bones are so comminuted that they are not salvageable and must be replaced or augmented using autologous bone grafts or synthetic materials. Each fragment is carefully identified, returned to a normal anatomic position, and fixed in place using plates and screws or interosseous wiring all bone fragments meticulously, potentially with primary bone grafting, to restore their normal configuration. The key to the successful repair of NOE fractures is to carefully reestablish the nasomaxillary buttress and to restore the normal points of attachment of the medial canthal ligaments.NOE fractures are typically caused by such extreme forces that they are frequently associated with intracranial


Surgery_Schwartz

Surgery_Schwartz. buttress and to restore the normal points of attachment of the medial canthal ligaments.NOE fractures are typically caused by such extreme forces that they are frequently associated with intracranial injuries and multiple other facial bone fractures in a presentation referred to as a panfacial fracture. These may involve any combination of the fractures described previously. The challenge of these injuries is to reestablish normal relationships of key anatomic landmarks. A combination of salvable bone fragments, autolo-gous bone grafting, and synthetic materials accomplishes this.Posttraumatic Extremity ReconstructionThe primary goal in posttraumatic extremity reconstruction is to maximize function. When structural integrity, motor function, and sensation can be reasonably preserved, then extremity salvage may be attempted. Otherwise, severe injuries require amputation best performed following reconstructive surgery principals that set the stage for maximizing function with


Surgery_Schwartz

Surgery_Schwartz. then extremity salvage may be attempted. Otherwise, severe injuries require amputation best performed following reconstructive surgery principals that set the stage for maximizing function with pros-thetics and minimizing chronic pain and risk of tissue break-down. Microvascular surgical techniques are an essential part of extremity trauma surgery, allowing replantation of amputated parts or transfer of vascularized bone and soft tissue when tis-sue in zone of injury cannot be salvaged. Soft tissue techniques combined with advances in bone fixation and regeneration with distraction have proven tremendous benefit for patients with severe limb-threatening extremity trauma. Current state-of-the-art techniques require multidisciplinary cooperation between orthopedic, vascular, and plastic surgeons as presented in the algorithm (Fig. 45-56). Reconstructive techniques include the use of vascularized bone, bone distraction techniques, external fixation, nerve grafts and transfers, composite


Surgery_Schwartz

Surgery_Schwartz. as presented in the algorithm (Fig. 45-56). Reconstructive techniques include the use of vascularized bone, bone distraction techniques, external fixation, nerve grafts and transfers, composite tissue flaps, and functioning muscle transfers tailored to the given defect. The future promises further advances with routine application of vascularized composite allografts, engineered tissue replace-ments, and computer animated prosthetics controlled intuitively by patients via sensors that are placed on the amputation stump and able to detect impulses transmitted through undamaged peripheral nerves remaining in the extremity.Common causes of high-energy lower extremity trauma include road traffic accidents, falls from a height, direct blows, sports injuries, and gunshots. As with maxillofacial trauma, the first phase of care is activation of the advanced trauma life support protocols. The most common life-threatening consider-ations are airway maintenance, control of bleeding, and


Surgery_Schwartz

Surgery_Schwartz. trauma, the first phase of care is activation of the advanced trauma life support protocols. The most common life-threatening consider-ations are airway maintenance, control of bleeding, and identi-fication of other injuries. Once the patient’s condition has been stabilized and life-threatening injuries managed, attention is directed to diagnosis and management of the extremity. Tetanus vaccine and antibiotics should be provided as soon as possible for open wounds.Systematic evaluation of the traumatized extremity helps to ensure no important findings are missed. Physical examina-tion to assess the neurovascular status, soft tissue condi-tion, and location of bone fractures forms the foundation of ordering imaging studies to provide details of bone and vas-cular injuries. Evidence of absent pulses is an indication to con-sider Doppler ultrasound examination followed by angiography to detail the exact nature of the injury. The blood supply must be immediately restored to devascularized


Surgery_Schwartz

Surgery_Schwartz. pulses is an indication to con-sider Doppler ultrasound examination followed by angiography to detail the exact nature of the injury. The blood supply must be immediately restored to devascularized extremities. Crush injuries might be associated with compartment syndrome, in which tissue pressure due to swelling in the constricted facial compartments exceeds capillary perfusion pressure and causes nerve and muscle ischemia. In the early stages of compartment syndrome, findings include pain on passive stretch of the com-partment’s musculature in a pale, pulseless extremity without evidence of direct vascular injury. Neurologic changes consist-ing of paresthesias followed by motor paralysis are late signs. Once recognized, decompressive fasciotomies must be per-formed as soon as possible to prevent permanent tissue loss. Compartment syndrome can be a late event after fracture reduc-tion and fixation (either internal or external), so the extremity must be reevaluated regularly in the


Surgery_Schwartz

Surgery_Schwartz. to prevent permanent tissue loss. Compartment syndrome can be a late event after fracture reduc-tion and fixation (either internal or external), so the extremity must be reevaluated regularly in the early postoperative period. This is especially true in situations where there has been a period of ischemia prior to successful revascularization.Several scoring systems for extremity trauma severity have been suggested to aid in treatment planning. Open fractures can be classified according to a system devised by Gustilo and 7Brunicardi_Ch45_p1967-p2026.indd 200501/03/19 6:30 PM 2006SPECIFIC CONSIDERATIONSPART IIReconstructableKnee functionalAdequate soft tissueDirty woundDirty woundClean woundFoot availableFoot not availableClean woundInadequate soft tissueKnee irreparableUnreconstructableTraumaticbelow kneeinjuryAmputationLimbreconstruction/replantationDelayedclosurePrimaryclosureFoot filetfree flapParascapularfree flapImmediatefree flapDelayedfree flapPrimaryreconstructionBelow


Surgery_Schwartz

Surgery_Schwartz. kneeinjuryAmputationLimbreconstruction/replantationDelayedclosurePrimaryclosureFoot filetfree flapParascapularfree flapImmediatefree flapDelayedfree flapPrimaryreconstructionBelow kneesalvageBelow kneesalvageAbove kneeamputationFigure 45-56. Algorithm of posttraumatic extremity reconstruction.colleagues. Grades I and II are open fractures with minimal soft tissue disruption. Grade III injuries most often require consider-ation of soft tissue reconstruction. Grade IIIA are open fractures with severe soft tissue injury but adequate soft tissues to repair. Grade IIIB involves a loss of soft tissue that will require some technique for tissue replacement. Grade IIIC involves a vascular injury requiring reconstruction. For the most severe injuries, the most important decision is whether to attempt extremity salvage or proceed with amputation. Patients with extensive fracture comminution, bone or soft tissue loss, wound contamination, and devascularization have a poor prognosis. Extremity


Surgery_Schwartz

Surgery_Schwartz. extremity salvage or proceed with amputation. Patients with extensive fracture comminution, bone or soft tissue loss, wound contamination, and devascularization have a poor prognosis. Extremity salvage requires multiple operations and a prolonged period of rehabili-tation and physical therapy. The loss of plantar sensation histori-cally favored below-knee amputation, but this is no longer an absolute recommendation. A final decision to attempt salvage must be made within the context of comorbidities, socioeco-nomic considerations, patient motivation, and overall rehabilita-tive potential.The first step in surgical management is complete debride-ment of all devitalized tissue. Early one-stage wound coverage and bony reconstruction is generally advocated and should be performed jointly by extremity trauma orthopedic and plastic surgical teams.50 It is acceptable for reconstruction to be deferred briefly if the adequacy of debridement is certain. Negative pres-sure wound therapy is


Surgery_Schwartz

Surgery_Schwartz. by extremity trauma orthopedic and plastic surgical teams.50 It is acceptable for reconstruction to be deferred briefly if the adequacy of debridement is certain. Negative pres-sure wound therapy is useful between debridement and defini-tive reconstruction to control the wound drainage and prevent bacterial contamination. When there is segmental bone loss, it is advisable to achieve soft tissue closure prior to performing osse-ous reconstruction. Preparation for later restoration of the bone requires steps to prevent the soft tissue from collapsing into the space where bone is needed. A common technique for this is to fill the space with antibiotic-impregnated beads or an antibiotic spacer at the time of soft tissue restoration until definitive bony reconstruction is possible. An external fixation may be needed, if there is segmental bone loss (Fig. 45-57A,B).The sequence for reconstruction is meticulous debride-ment of nonviable tissue, fracture reduction and stabilization, vascular


Surgery_Schwartz

Surgery_Schwartz. fixation may be needed, if there is segmental bone loss (Fig. 45-57A,B).The sequence for reconstruction is meticulous debride-ment of nonviable tissue, fracture reduction and stabilization, vascular repair if necessary, and finally restoration of the soft tissue coverage. A multidisciplinary team of specialists works together to perform these procedures in order to obtain the best outcomes. Orthopedic and plastic surgeons perform wound debridement. Orthopedic surgeons then reduce and stabilize the fractures. Vascular surgeons reconstruct damage major vessels. Finally, plastic and reconstructive surgeons perform soft tissue coverage. Ideally, each operating team completes their part of the procedure sequentially during the same anesthetic.Choices for soft tissue coverage of open fractures include split-thickness skin grafts, temporary skin substitutes fol-lowed later by skin grafting, local rotation flaps, or free tissue transfers. Selecting the most appropriate option depends on the


Surgery_Schwartz

Surgery_Schwartz. include split-thickness skin grafts, temporary skin substitutes fol-lowed later by skin grafting, local rotation flaps, or free tissue transfers. Selecting the most appropriate option depends on the quality of the local tissues and location of the soft tissue defect relative to the underlying fracture and fixation hard-ware. The guiding principle is to be certain that the source of tissue transferred into the defect is outside of the zone of injury. When flaps are selected, either fasciocutaneous or muscular flaps may be indicated depending on tissue avail-ability, wound bed contours, and surgeon preferences. Uneven wound surface contours are more reliably obliterated with a Brunicardi_Ch45_p1967-p2026.indd 200601/03/19 6:30 PM 2007PLASTIC AND RECONSTRUCTIVE SURGERYCHAPTER 45Figure 45-57A, B. An external fixation for segmental bone loss.Figure 45-58. A. Defect ulnar side of the forearm, with an external fixator. B. Propeller flap. C. Flap is inset. D. Six weeks post


Surgery_Schwartz

Surgery_Schwartz. 45Figure 45-57A, B. An external fixation for segmental bone loss.Figure 45-58. A. Defect ulnar side of the forearm, with an external fixator. B. Propeller flap. C. Flap is inset. D. Six weeks post operation.ABpliable muscle flap. Fasciocutaneous flaps may provide more durable coverage in areas subject to abrasion or pressure from footwear, for example, on the foot or around the ankle. Some defects can be covered with flaps containing both skin and muscle if indicated. Ideal coverage for weight-bearing areas should be able to resist pressure and shear and provide sensa-tion. Split-thickness skin grafts are reasonable for coverage of exposed healthy muscle or soft tissue. Local flaps may be used to cover smaller defects as long as uninjured tissue is located nearby. These may be designed as traditional random or axial ABCDflaps, but the most advanced techniques are based on under-lying perforators that allow extremely versatile flap designs customized to the defect. These flaps are


Surgery_Schwartz

Surgery_Schwartz. as traditional random or axial ABCDflaps, but the most advanced techniques are based on under-lying perforators that allow extremely versatile flap designs customized to the defect. These flaps are designed with a per-forating vessel at the base near to the defect and a long axis extending an equal distance opposite. The flap is elevated and rotated into the defect in a motion reminiscent of an airplane propeller, which gives rise to the designation “propeller flap” for this kind of reconstruction (Fig. 45-58A, defect ulnar side of the forearm, with an external fixator; Fig. 45-58B, propel-ler flap; Fig. 45-58C, flap is inset; Fig. 45-58D, 6 weeks after Brunicardi_Ch45_p1967-p2026.indd 200701/03/19 6:31 PM 2008SPECIFIC CONSIDERATIONSPART IIthe operation). The advantages of this technique are that it does not impair muscle function and it can often complete a complex reconstruction without the need for microvascular surgery.When requirements exceed the potential for skin grafts or


Surgery_Schwartz

Surgery_Schwartz. are that it does not impair muscle function and it can often complete a complex reconstruction without the need for microvascular surgery.When requirements exceed the potential for skin grafts or local flaps, tissue must be transferred from distant sites. The reconstructive choices differ based on the anatomic location of the defect and the extent of damage. This is often the case for major injuries in the middle or lower third of the leg where bones are covered with thin soft tissue and less donor tissue is available. A traditional method is to obtain tissue by creating a pedicled flap from the opposite, uninjured extremity. Cross-leg flaps remain effective, but indications are limited to circum-stances where microsurgery is not possible or in young children who are less prone to risks associated with prolonged immobi-lization necessary for these flaps, such as joint stiffness or deep vein thrombosis. Free tissue transfer is the preferred alternative. The general principles of


Surgery_Schwartz

Surgery_Schwartz. associated with prolonged immobi-lization necessary for these flaps, such as joint stiffness or deep vein thrombosis. Free tissue transfer is the preferred alternative. The general principles of reconstructive microsurgery in lower extremity trauma are to select recipient vessels outside of the zone of injury, select donor tissue suitable for the defect with minimal risk of donor site morbidity, and ensure there is bone stability before reconstruction using either internal or external fixation. For example, a latissimus dorsi muscle flap provides a large amount of tissue for reconstruction, but loss of the latis-simus function can make it more difficult for the patient to use crutches for ambulation during rehabilitation. Muscle or fascio-cutaneous flaps each have a role in selected circumstances.51 Bone can also be added to help fracture repair.52 Free flaps can also be designed as “flow-through” flaps, which reconstruct missing segments of major vessels and provide soft tissue or


Surgery_Schwartz

Surgery_Schwartz. Bone can also be added to help fracture repair.52 Free flaps can also be designed as “flow-through” flaps, which reconstruct missing segments of major vessels and provide soft tissue or bone coverage.53After wound healing, proper physical and/or occupational therapy and rehabilitation is essential for the best long-term out-comes. This often requires many months of consistent retrain-ing and conditioning in order to return to the functional status enjoyed by the patient before injury. Properly fitted orthotic appliances and footwear provide essential protection against pressure-related complications and can improve function. Late complications such as osteomyelitis may appear, evidenced by signs of infection months or even years after reconstruction. Very often this is caused by inadequate debridement at the time of initial surgery.Tumor locationPrimaryreconstructive optionSecondaryreconstructive optionLower-extremity bone sarcomacomposite resectionDistal femur/proximal tibiaPedicled


Surgery_Schwartz

Surgery_Schwartz. debridement at the time of initial surgery.Tumor locationPrimaryreconstructive optionSecondaryreconstructive optionLower-extremity bone sarcomacomposite resectionDistal femur/proximal tibiaPedicled gastrocnemius ±soleusDistally-based pedicledALT; anterior bipedicledfasciocutaneous flap; pedicledsural artery flap; free flapMid/distal tibiaPrimary closurePedicled gastrocneumius± soleus; propeller,keystone flaps; free flapProximal/mid-femurPrimary closurePedicled ALT;Pedicled rectusabdominis; free flapWhen limb salvage either is not possible or is not in the best interest of the patient, amputation is indicated. Maxi-mizing limb length, providing durable soft tissue coverage, and managing peripheral nerves to avoid chronic pain help to ensure good functional recovery using extremity prosthet-ics. Ideally, local tissues are used; however, when they are unavailable or inadequate, the amputated part can be a use-ful source of skin grafts or tissues for microvascular free transfers to the