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Beckwith-Wiedemann Syndrome (BWS) is a pediatric overgrowth disorder commonly associated with macroglossia. Speech/feeding difficulties and airway compromise often warrant surgical intervention through reduction glossectomy. Given the complexity of pediatric patients with congenital anomalies, concurrent diagnoses of coexisting conditions may be delayed. We present a pediatric patient with BWS who presented with chronic respiratory distress and stridor in the setting of obstructive sleep apnea (OSA) and macroglossia. We seek to highlight the concurrent presence and treatment approach of a congenital vascular ring and macroglossia as there are no reported cases of these coexisting anomalies in the literature.

Background Microvascular hepatic artery reconstruction (MHAR) is associated with decreased rates of hepatic artery thrombosis (HAT) in living donor liver transplantation (LDLT). There is a paucity of literature describing the learning points and initiation of this technique at the institutional level. The objective of this study is to report our institutional experience using MHAR in adult LDLT with a focus on technique and outcomes. Methods A retrospective review of adult patients who underwent LDLT from January 2012 to December 2020 was conducted. Patients were divided into two groups, those who underwent LDLT without MHAR and with MHAR. We analyzed cases for technical data including donor and recipient artery characteristics, anastomotic techniques, intraop events, and postop complications. A Mann–Whitney test was performed to compare outcomes between non-MHAR and MHAR patients. Results Fifty non-MHAR and 50 MHAR patients met inclusion criteria. Median age at transplantation was 58 (interquartile range [IQR] 11.8) and 57.5 years (IQR 14.5), respectively. Median follow-up for MHAR patients was 12.8 months (IQR 11.6). The most common recipient arteries were the right hepatic artery (HA) (58%) and left HA (20%). Median size of recipient and donor arteries were 3.3 mm (IQR 0.7) and 3.1 mm (IQR 0.7), resulting in a median mismatch size of 0.3 mm (IQR 0.4). Median microanastomosis time was 44 minutes (IQR 0). HAT, graft failure, and mortality rates were higher in the non-MHAR cohort (6% vs. 0%, 8% vs. 0%, and 16% vs. 6%, respectively); however, these did not reach statistical significance. Conclusion This study found lower rates of HAT and graft failure after implementing MHAR, though statistical significance was not achieved. Larger cohort studies are needed to further assess the potential benefit of MHAR in adult LDLT. From our experience, MHAR requires cooperation between the transplant and microsurgical teams, with technical challenges overcome with appropriate instrumentation and planning.

Cleft lip and/or palate affects approximately one in 700 live births. Optimal timing for repair of cleft lip has yet to be objectively validated. Earlier repair takes advantage of a high degree of plasticity within the nasal cartilage and maxilla. The authors present patients enrolled in an early cleft lip repair protocol facilitating effective repair of the cleft lip and nostril.American Society of Anesthesiologists class I to II patients with unilateral cleft lip and/or palate undergoing repair before 3 months of age were enrolled over 5 years. Perioperative data, surgical and anesthetic complications, preoperative and postoperative nostril breadth, nostril width, nasal angle, lip length, frontal nasal breadth, and commissure length measured as ratios between the cleft and noncleft sides were abstracted. Early cleft lip repair and nasoalveolar molding patients were matched for cleft lip severity using the cleft width ratios and compared.The surgical and anesthetic complication rate for 100 early cleft lip repair patients was 2 percent. Operative and anesthetic times were 123 ± 37 minutes and 177 ± 34 minutes, respectively. Hospital length of stay was 1 ± 0 day. Age at repair between early cleft lip repair and nasoalveolar molding was 33 ± 15 days and 118 ± 33 days, respectively. After early cleft lip repair, preoperative to postoperative distance from symmetry for all anthropomorphic measurements improved ( p0.001). Comparing severity-matched early cleft lip repair to nasoalveolar molding patients, similar improvements were observed, suggesting equivalent results ( p0.05).Early cleft lip repair provides an efficacious method for correcting the cleft lip and nasal deformity that simulates nasoalveolar molding.Therapeutic, III.

BACKGROUND Midface hypoplasia dramatically affects the normative facial cascade. Simultaneous Le Fort III and Le Fort I procedures (Le Fort III/I) provide a powerful tool for achieving significant midface advancement. This study presents the authors' approach for addressing midface hypoplasia in the setting of class III malocclusion using Le Fort III/I advancement. METHODS This was an institutional review board-approved retrospective review of patients who underwent Le Fort III/I advancement at the authors' institution from 2009 to 2019. Demographic, surgical, and postoperative data were recorded. The authors' operative technique and surgical pearls are described. RESULTS Twenty-five patients met inclusion criteria, 15 male patients (60 percent) and 10 female patients (40 percent). Patient age ranged from 14.9 to 21.6 years. Diagnoses included Crouzon syndrome, nonsyndromic developmental skeletal dysplasia, cleft lip/palate, Klippel-Feil syndrome, Apert syndrome, Van den Ende-Gupta syndrome, and Pfeiffer syndrome. Le Fort III advancements averaged 6.18 ± 1.38 mm and Le Fort I advancements averaged 6.70 ± 2.48 mm. Thirteen patients underwent simultaneous bilateral sagittal split osteotomy with average movement of 5.85 ± 1.21 mm. Average follow-up was 1.3 ± 1.0 years. One patient experienced intraoperative cerebrospinal fluid leak that resolved with expectant management. Three patients experienced major complications (12 percent) postoperatively necessitating repeated orthognathic operations. Ten patients experienced minor complications (40 percent). Average length of stay was 10 days, with all patients achieving improvement of their facial profile. CONCLUSIONS The authors' experience reaffirms the relative safety of simultaneous Le Fort III/I advancement. This technique should be considered in select patients with global midface retrusion and class III malocclusion. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, IV.

Objective: To evaluate characteristics of congenital heart disease (CHD) in patients with cleft lip and/or palate (CL/P) and assess potential associations with cleft outcomes. Design: Retrospective review of all patients with CL/P who underwent primary cleft treatment from 2009 to 2015. Setting: Children’s Hospital Los Angeles, a tertiary hospital. Patients: Exclusion criteria included microform cleft lip diagnosis, international patients, and patients presenting for secondary repair or revision after primary repair at another institution. Main Outcomes Measured: Patient demographics, prenatal and birth characteristics, CL/P characteristics, syndromic status, postoperative complications, and other outcomes were analyzed relative to CHD diagnoses and management. Patients with CL/P with (+CHD) were compared to those without (−CHD) CHD using χ2 tests and analysis of variance. Results: Among 575 patients with CL/P, 83 (14.4%) had CHD. Congenital heart disease rates were significantly higher in patients with cleft palate (CP) compared to other cleft types (χ2, P = .009). Eighty-one (97.6%) out of 83 +CHD patients were diagnosed prior to initial CL/P surgical assessment. Twenty-three (27.7%) +CHD patients required surgical repair of 10 cardiac anomalies prior to cleft care. Congenital heart disease was associated with delayed CP repair and increased rates of fistula in isolated patients with CP. Conclusions: Congenital heart disease is known to be more prevalent in patients with CL/P. These data suggest the condition is particularly increased in patients with CP. Severe forms of CHD are diagnosed and treated prior to cleft care however postoperative fistula may be more common in patients with CHD. Therefore, careful attention is required for patient optimization and palatal flap dissection in patients with coexisting CHD and CL/P.

Background Although combined monobloc facial bipartition with distraction (MFBD) may simultaneously correct multiple facial dimensions in patients with syndromic craniosynostoses, complication risks limit its use. This study reassesses MFBD complications and outcomes to compare safety and efficacy to monobloc distraction (MD) and facial bipartition (FB) alone. Methods A retrospective review of MFBD, MD, and FB cases for 10 years at a tertiary children's hospital was performed. Patient demographics and surgical variables were compared between cohorts. Distraction distance was compared between MFBD and MD. Correction of interdacryon distance in MFBD compared with FB was measured on preoperative and postoperative computed tomographic scans. SPSS 17 was used for data analysis. Results Twenty-two total patients, 11 MFBD, 4 MD, and 7 FB, met the inclusion criteria. Three MFBD (27.3%) patients experienced complications, including 1 osteomyelitis and 2 hardware displacements. One MD patient (25%) experienced a postoperative complication consisting of a wound infection. Three FB patients (42.9%) experienced either cerebrospinal fluid leak, seroma, mucocele, hardware exposure, and/or orbital dystopia (n = 1 each). Patients with MFBD had significantly longer intensive care unit stay (P ≤ 0.05), but no difference in hospital stay (P = 0.421). Mean distraction length was similar between MFBD and MD (P = 0.612). There was no significant difference in final (P = 0.243) or change (P = 0.189) in interdacryon distance between MFBD and FB patients. Conclusions In our experience, MFBD has similar complication rates compared with MD and FB alone. Given equivalent safety and postoperative correction of facial dimensions, MFBD could be more widely considered for select patients.

BACKGROUND Cranial CT is routinely taught to be the gold standard for diagnosis of craniosynostosis and used by craniofacial teams for suspected nonsyndromic single suture craniosynostosis. Given the risks associated with infant CTs, do these scans provide significantly enhanced diagnostic accuracy compared to the physical exam when performed by an experienced clinical provider? METHOD A retrospective chart review was performed for children who underwent corrective surgery for nonsyndromic, single-suture craniosynostosis over an 11 year period by a single craniofacial team. Ages at presentation and surgery, preoperative clinical diagnosis and imaging, co-existing radiographic findings, and correlation with the intraoperative diagnosis were analyzed. RESULTS A total of 138 patients were included in this study. The mean age was 4.2 months at initial craniofacial evaluation, and 8.0 months at time of surgery. Twenty-seven patients received imaging prior to our clinic. Of those, 21 had plain radiography and 6 had CT scans. Of the remaining 111 patients referred without imaging, craniosynostosis was clinically diagnosed in 102 (92%), whereas 9 (8%) had an unclear clinical diagnosis. Of these 9, 1 (1%) was diagnosed clinically at follow-up exam, and the remaining 8 (7%) were diagnosed using radiography (3 CT scans, 5 plain radiographs). In all patients, the preoperative diagnosis was confirmed during intraoperative assessment. CONCLUSIONS Cranial CT was not needed by experienced craniofacial providers in 93% of nonsyndromic, single-suture craniosynostosis. Imaging obtained before craniofacial clinic referral may have been unnecessary. These findings question the classic teaching that preoperative cranial CT is the gold standard for diagnosis in infants with nonsyndromic, single-suture craniosynostosis.

Excisions of scalp nevus sebaceous (NS) presents a unique challenge due to limited soft tissue laxity, hair-bearing skin, and convex surfaces which often leave the surgeon and patient underwhelmed with the reconstructive outcome. In this study, the authors conducted an institutional review board-approved retrospective review of patients who underwent excision of pathologically proven scalp primary NS from 2003 to 2017 at our institution to better define the reconstructive outcomes and options for treatment of pediatric scalp NS. 92 patients were included in the study, 54 males (58.7%) and 38 females (41.3%). The average age at surgery was 7.24 years (0.5-16.0; SD 4.7). Local tissue undermining/galeal scoring with primary closure (LTUGS) was used for lesions with average surface area of 3.6 cm, rotational or transposition flaps (RF/TF) for lesions averaging 4.3 cm, completed serial excision for lesions averaging 13.9 cm, and tissue expansion (TE) for lesions averaging 21 cm (P

Purpose Secondary alveolar bone grafting (SABG) during mixed dentition is the standard of care for alveolar clefts. However, early SABG at 4 to 7 years of age before the eruption of lateral incisors versus late SABG at 8 to 12 years of age before the eruption of maxillary permanent canines is still debated. The purpose of this study was to perform a systematic review of the literature to evaluate the outcomes of early SABG in residual bone volume or degree of bone resorption, maxillary canine movement or impaction rate, complications, and esthetic outcomes and to compare outcomes of early versus late SABG. Materials and Methods A comprehensive search of the PubMed database was performed according to PRISMA guidelines. Keywords for alveolar cleft repair and inclusion criteria were used to screen articles for final review. Results The initial search yielded 6,278 articles, of which 4 retrospective and 4 prospective studies were chosen for final review. Of these, 7 studies compared outcome variables between early and late SABG and 1 looked at bone formation of patients who underwent only early SABG. For outcome variables, 3 studies assessed bone volume with radiographic evaluation, 2 estimated maxillary permanent canine impaction rate, 1 evaluated surgical complications, operative time, and length of hospital stay, and 1 looked at anterior incisor crown length. Overall, 6 studies concluded that early SABG provides a better outcome than late SABG and 2 found comparable results across the aforementioned variables. Conclusions This review suggests that the existing data support the concept of early SABG; however, the data are not sufficient for a definitive conclusion. A well-planned prospective study is needed to further investigate the rationale for early SABG.

Over the last 3 years a shift at our institution has taken place in which patients who would have been offered nasoalveolar molding (NAM) as an adjunct to cleft lip repair (repair after 3 months) have instead undergone early cleft lip repair (ECLR) (2-5 weeks of life) without NAM. This study sought to examine the financial and social impact of the transition away from NAM to ECLR. The efficacy of NAM is limited by patient compliance to a rigorous treatment schedule requiring weekly visits for appliance adjustments. Nasoalveolar molding patients required an average of 11 dental visits, accounting for $2132 in indirect lost income per family. Average direct charges for NAM totaled $12,290 for the hospital, physician, and appliance costs. Over the entire study period, the cumulative direct cost of NAM separate from the surgical repair of the lip was $970,910. Following the introduction of ECLR as an alternative to NAM with standard lip repair, NAM usage decreased by 48% and unilateral cleft lip patients undergoing NAM decreased by 86%. Those diverted from NAM to ECLR resulted in a decreased healthcare cost burden of $368,700 ($111,727 per year). In addition to the time burden, the financial burden of NAM is significant. Early cleft lip repair without NAM is more cost effective. Nasoalveolar molding has significantly decreased utilization since the implementation of ECLR. We believe that ECLR, with increased experience, long-term data, and increased awareness, has the potential to decrease the burden of health care costs in the United States.

Objective At our institution, cleft lip repair is performed at or before 8 weeks of age, and tympanostomy tubes (TT) have been inserted concurrently in some of these patients. Our objective was to determine the feasibility and present the preliminary results of TT placement at this early age. Methods A retrospective review was conducted of 22 patients born with cleft lip and/or palate who had TT placement in the first 8 weeks of life. Data collected included complications and audiologic function post TT placement. Results Subjects had a mean age of 3.9 weeks at the time of TT placement and cleft lip repair. Thirteen patients (59%) reported otorrhea during the follow-up period. Eighteen of 21 (86%) patients who underwent auditory evaluation had normal audiological function following TT placement. One patient had sensorineural hearing loss and 2 patients had mild conductive hearing loss due to bilateral extruded TT and OME at 15.9 and 39.2 months after surgery. Conclusion Exceptionally early TT is feasible. Placing TT at or before 8 weeks of age did not result in any major complications but demonstrated a high incidence of otorrhea. A randomized, prospective study with a larger sample size is necessary to determine the benefits of TT placement at this age.

Background In severe cases of maxillary hypoplasia, Le Fort I distraction may be required for treatment. This study describes our experience with internal distraction devices and assesses our outcomes in patients with a negative overjet on average >15 mm. Methods A retrospective review of patients with a history of cleft lip and/or palate who underwent Le Fort I distraction at our institution from 11/2007-11/2017 was performed. Data regarding demographics, procedural details and outcomes were collected and analyzed. Results Twenty patients, 13 (65.0%) male and 7 (30.0%) female, were included. All (100%) patients had a history of cleft lip and/or palate and 2 (10.0%) of them had associated syndromes. All 20 patients underwent internal distraction. The average age at surgery was 17.8 years (range 15.2-20.7, SD 1.6 years). The average preoperative negative overjet was 19.27 mm (range 10-30, SD 5.63 mm). The mean total distraction length was 15.3 mm (range 0-30, SD 6.72 mm). There were no intraoperative complications, however, there were a total of 4 (20.0%) postoperative complications, 2 of which required reoperation due to device malfunction or displacement. 14 (70.0%) patients had repeat procedures to complete occlusal correction or correct relapse. Conclusions Maxillary distraction alone was insufficient for correction of maxillary discrepancies averaging >15 mm. Instead, it's utility may be in positioning the maxilla for a more viable final advancement and fixation procedure, rather than being solely responsible for achieving normal occlusion during distraction.

BACKGROUND Fat grafting is a common adjunct procedure used to treat temporal fat hollowing in children with craniofacial anomalies. The goal of this study was to assess the surgical and aesthetic outcomes of this procedure. METHODS We retrospectively reviewed patients who underwent temporal fat grafting at a single tertiary pediatric craniofacial center. The surgical outcome was assessed based on intraoperativeand 30-day postoperative complication rates. The aesthetic outcome was assessed by 3 independent investigators using a 3-point grading scale for preoperative temporal hollowing severity (1 = mild, 2 = moderate, 3 = severe) and a 5-point scale for postoperative improvement (0 = no, 1 = mild, 2 = moderate, 3 = significant, and 4 = complete improvement). RESULTS Forty-three patients met inclusion criteria. Twenty-seven (63%) were male, 39 (91%) had a history of craniosynostosis, and 18 (42%) had associated syndromes. The mean age at fat grafting was 9.9 years (2.7-20.4, SD = 5.5) with an average follow-up time of 1.6 years (0-5.8, SD = 1.8). The average volume of fat grafted was 8.6 mL (0-30, SD = 5.9) to the right temporal region and 8.6 mL (0-30, SD = 5.8) to the left. There were no intraoperative or postoperative complications. The mean improvement score was 2.9 (1-4, SD = 0.7), demonstrating that most patients experienced moderate to significant improvement. Multiple linear regression analysis demonstrated that syndromic status had a negative impact on the aesthetic outcome (P < 0.001). CONCLUSIONS These findings demonstrate that fat grafting is an effective method to treat temporal hollowing in children with craniofacial anomalies with no perioperative complications.

Purpose Mandibular distraction osteogenesis (MDO) has been shown to be successful in treating upper airway obstruction caused by micrognathia in pediatric patients. The purpose of this study was to assess the success rate of MDO and possible predictors of failure. Patients and Methods The records of all neonates and infants who underwent MDO from 2008 to 2015 were retrospectively reviewed. Procedural failure was defined as patient death or the need for tracheostomy postoperatively. Details of distraction, length of stay, and failures were captured and elucidated. Results Of the 82 patients, 47 (57.3%) were male; 46 (56.1%) had sporadic Pierre Robin sequence; 33 (40.3%) had syndromic Pierre Robin sequence; and 3 (3.7%) had micrognathia, not otherwise specified. The average distraction length was 27.5 mm (range, 15 to 30 mm; SD, 4.4 mm), the average age at operation was 63.3 days (range, 3 to 342 days; SD, 71.4 days), and the average length of post-MDO hospital stay was 43 days (range, 9 to 219 days; SD, 35 days) with an average follow-up period of 4.3 years (range, 1.1 to 9.6 years; SD, 2.6 years). There were 7 failures (8.5%) (5 tracheostomies and 2 deaths) resulting in a 91.5% success rate. Regression analysis showed that the predicted probability of the need for tracheostomy was 45% (P = .02) when the patient had a central nervous system (CNS) anomaly. The predicted probability of the need for tracheostomy and death combined was 99.6% when the patient had laryngomalacia and a CNS anomaly and was preoperatively intubated (P Conclusions This review confirms that MDO is an effective method of treating the upper airway obstruction caused by micrognathia with a high success rate. In our sample the presence of CNS abnormalities, laryngomalacia, and preoperative intubation had a significant impact on the failure rate.

Bone wax is a commonly used hemostatic agent with minimal complications. Some of the known complications include inflammation, granuloma formation, infection, and impaired osteogenesis. Several clinical reports of bone wax migration have also been reported. In this paper, the authors present a rare patient of bone wax migration intracranially in a 6-year-old patient who initially underwent craniotomy for the evacuation of subdural hematoma and repair of depressed skull fracture. The patient then underwent craniotomy scalp scar revision several months later. Postoperatively he developed short-term memory loss, apraxia, and word finding difficulties. The imaging findings were consistent with the presence of a foreign body centered in the posterior aspect of the left middle temporal gyrus, which was surgically removed and found to be bone wax. The patient recovered well with complete improvement of his neurologic symptoms.

Objective:The workup of patients with Pierre Robin sequence (PRS) consists of a physical examination, O2saturation, and polysomnography to determine the severity of respiratory obstruction and need for surgery. We suggest that capillary blood gas (CBG) may be a better physiologic representation of airway obstruction and should be routinely used in the management of patients with PRS.Design:This is a multicenter study based on a retrospective review of medical records.Setting:The study was performed at tertiary care centers.Interventions:Patients with PRS Main Outcome Measure:Using successful treatment outcome as a reference standard, receiver operating characteristic (ROC) curve was used to determine the accuracy of the diagnostic test and values for the best sensitivity and specificity to determine the need for surgical intervention.Results:Of 73 patients, 48 had sporadic PRS, 23 had syndromes, 2 had micrognathia, not otherwise specified. Mandibular distraction osteogenesis was performed in 62 patients at a mean age of 39 days. The mean initial Apnea-Hypopnea Index (AHI) in nonsurgical versus surgical groups was 10 versus 31 ( P = .063), pH 7.41 versus 7.34 ( P = .003), pCO243 versus 56 ( P < .001), and HCO327 versus 30 ( P = .022). The ROC curve showed that pCO2of 49.5 has the best specificity (100%) and sensitivity (72.6%) profile in terms of need for definitive airway.Conclusion:A simple CBG heel stick may better predict the physiologic effects of obstructive apnea; therefore, it should be added to the algorithm of PRS workup.

Ameloblastic carcinomas are rare malignant lesions with 3 mandibular pediatric cases reported in the literature. We present a case of ameloblastic carcinoma in situ in a 15-year-old male with a right mandibular cystic lesion on computed tomography. The incisional biopsy revealed plexiform ameloblastoma. Due to the infiltrating and aggressive nature of the tumor, the patient underwent hemimandibulectomy and immediate reconstruction with a vascularized osteocutaneous fibula free flap. The final pathology was read as ameloblastic carcinoma in situ. Given the rarity of this disease in the pediatric population, this case report may be a valuable addition to the current literature.

Background Of U.S. craniofacial and neurosurgeons, 94 percent routinely admit patients to the intensive care unit following cranial vault remodeling for correction of sagittal synostosis. This study aims to examine the outcomes and cost of direct ward admission following primary cranial vault remodeling for sagittal synostosis. Methods An institutional review board-approved retrospective review was undertaken of the records of all patients who underwent primary cranial vault remodeling for isolated sagittal craniosynostosis from 2009 to 2015 at a single pediatric hospital. Patient demographics, perioperative course, and outcomes were recorded. Results One hundred ten patients met inclusion criteria with absence of other major medical problems. Average age at operation was 6.7 months, with a mean follow-up of 19.8 months. Ninety-eight patients (89 percent) were admitted to a general ward for postoperative care, whereas the remaining 12 (11 percent) were admitted to the intensive care unit for preoperative or perioperative concerns. Among ward-admitted patients, there were four (3.6 percent) minor complications; however, there were no major adverse events, with none necessitating intensive care unit transfers from the ward and no mortalities. Average hospital stay was 3.7 days. The institution's financial difference in cost of intensive care unit stay versus ward bed was $5520 on average per bed per day. Omitting just one intensive care unit postoperative day stay for this patient cohort would reduce projected health care costs by a total of $540,960 for the study period. Conclusion Despite the common practice of postoperative admission to the intensive care unit following cranial vault remodeling for sagittal craniosynostosis, the authors suggest that postoperative care be considered on an individual basis, with only a small percentage requiring a higher level of care. Clinical question/level of evidence Therapeutic, III.

Background Despite advances in the field of tissue expansion, the face is especially difficult to reconstruct using this technique due to its dynamic nature and high incidence of distortional scarring. This article aimed to review complications seen in pediatric facial tissue expansion, specifically ectropion, as well as its restorative treatment. Methods A retrospective chart review of pediatric patients treated by tissue expansion for congenital facial lesions, trauma, or burns at Children's Hospital Los Angeles from January 2000 to present was performed. Patients were analyzed for preoperative diagnosis, reconstruction area, tissue expander location, number and fill volume of expanders, incidence of complications, including ectropion, and type of revision surgery. Results A total of 88 patients with 150 expander reconstructions were examined. The total complication rate was 43.1% with an 11.3% rate of ectropion. Of the 10 cases of ectropion, 9 were treated with canthoplasty, whereas 1 was managed conservatively. In addition to canthoplasty, full-thickness skin graft was preformed in 1 patient, Z-plasty in 1, and lid switch in 2. Discussion Tissue expansion is a safe and effective method of reconstruction for facial defects in the pediatric population despite complication rates being higher than other areas of the body. Specifically, ectropion can be a devastating complication, often requiring surgical correction. As such, careful planning should go into orientation and design of the reconstruction, and staged procedures should be strongly considered. Additionally, and possibly the most important, is setting patient and parental expectation about the possibility of ectropion and the necessity often for multiple corrective surgeries.

The lower extremity is often involved in trauma or other disease processes requiring reconstructive procedures in an attempt to maintain its anatomic and functional integrity. It also serves as a donor site for free flaps to reconstruct other body parts. Therefore, understanding the complex anatomy of the lower extremity is fundamental for every reconstructive surgeon to learn and understand. The aim of this chapter is to review the essentials of skeletal, muscular, fascial, and important neurovascular anatomic structures of the thigh, leg, and foot.

An intracranial approach to the craniomaxillofacial skeleton can be effective for correcting complex craniofacial dystoses such as frontofacial hypoplasia, hypertelorism, and orbital dystopia. However, the significant morbidity resulting from the high complication rates has limited intracranial use. Given the need for intracranial approaches for certain clinical indications, the present study reevaluated intracranial frontofacial procedures to determine their safety and outcomes.A retrospective review was performed of all frontofacial procedures completed between 2007 and 2017 at a single institution. Patients who had undergone monobloc distraction alone or with facial bipartition, facial bipartition alone, or box osteotomy were included in the intracranial cohort. Those who had undergone Le Fort III distraction, advancement, or a combination with Le Fort I were included in the subcranial cohort. The recorded data included demographics, previous craniofacial surgery, and operative events. The complications rates were compared between the 2 cohorts.The present study included 65 patients-35 subcranial and 30 intracranial. The rates of previous craniomaxillofacial (P = .193) and intracranial (P = .340) surgery were equivalent between the 2 cohorts. Of the 30 intracranial and 35 subcranial patients, 26.7% and 34.3% experienced complications (P = .218). The intracranial patients experienced more dural tears (53.3 vs 5.7%; P .0001); however, no significant differences were observed in cerebrospinal fluid leakage. Reintubations (n = 3; 4.5%) occurred exclusively in the subcranial group. No significant differences in the major and minor complication rates were observed between the 2 cohorts.Intracranial and subcranial frontofacial procedures are associated with an equal risk of major and minor complications. Given the lack of an increase in risk, intracranial frontofacial procedures should be considered for the management of complex craniofacial dystoses.

Background Congenital melanocytic nevi (CMN) have a 1% to 5% lifetime risk for malignant transformation, with 50% of transformations occurring before the age of 5 years.The aim of this study is to assess the risk of melanoma development in pediatric patients with facial CMN involving the eyebrows, eyelid margins, and nasal alae where a margin of CMN was not excised to preserve these structures. Methods A retrospective chart review of all pediatric patients with CMN from 1986 to 2014 was performed to review demographic information, diagnosis, and number of surgeries. Patients' clinical photographs were evaluated for residual nevi after completion of the treatment. Results More than 950 medical charts of patients with CMN of the face area were reviewed. We identified 32 patients (13; 41% male) that met the study criteria with pathology-confirmed diagnosis of facial CMN with an average age of 4.4 years (3.3 months-15.8 years) at the time of initial surgery. The CMNs were classified into small (1; 3%), medium (14; 44%), large (14; 44%), and giant (3; 9%) based on their projected adult sizes. No patients developed melanoma within the small residual lesions left over the eyebrows and eyelids and inside nostrils at an average follow-up time of 5.6 (1.0-14.4) years and average age of 9.6 (1.8-19.2) years at the time of last follow-up. Conclusions Although a lifelong risk of malignant transformation of the residual CMN cannot be concluded, our results found no transformation in follow-up visits at an average age beyond the highest risk of melanoma development in childhood. We feel that leaving residual lesions on the face in areas of important anatomic structures for better cosmetic outcome is an acceptable risk.

Congenital and pediatric nasal lesion resection and their reconstructive outcomes are not well studied. A surgeon must consider the site, size, depth, etiology, age, and effect on future function (including growth). As such, it is important to contrast the differences between the adult's and child's nose. The authors propose that more conservative resection and reconstructive methods may better serve congenital and pediatric nasal lesions. An Internal Review Board approved study of congenital and pediatric nasal lesions using a defect only approach from 2005 to 2017 was performed. Lesions, type of surgeries, complications, aesthetic outcome, and additional interventions were reviewed. One hundred twenty-seven patients met the study criteria with a median age at surgery of 5.4 years with follow-up of 1.4 years (1 week-11.3 years). The most common diagnosis was congenital melanocytic nevus (47, 37%). The lesions were located on more than 1 subunit in 34 (27%) patients with an average surface area of 3.7 (0.04-32) cm. The most common primary procedure was excision and primary closure with adjacent tissue undermining/rearrangement (73, 57.4%) followed by full-thickness skin graft (23, 18.1%). The aesthetic outcome was considered acceptable in a high number of patients 117 (92%), while 10 (8%) patients had unacceptable aesthetic outcomes, mostly due to scarring. The authors' data supports the concept of minimal healthy tissue excision or lesion only excision when treating pediatric and congenital nasal lesions.