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Ali M Soltani, Cameron S Francis, Arash Motamed, Ashley L Karatsonyi, Jeffrey A Hammoudeh, Pedro A Sanchez-Lara, John F Reinisch, Mark M UrataDivision of Plastic and Maxillofacial Surgery at Children's Hospital Los Angeles, CA, USA; The Division of Plastic and Reconstructive Surgery at the Keck School of Medicine of the University of Southern California, Los Angeles, CA, USABackground: Although hypertrophic scar (HTS) formation following cleft lip repair is relatively common, published rates vary widely, from 1% to nearly 50%. The risk factors associated with HTS formation in cleft patients are not well characterized. The primary aim of this retrospective study of 180 cleft lip repairs is to evaluate the frequency of postoperative HTS among various ethnic groups following cleft lip repair.Methods: A retrospective chart view of patients undergoing primary cleft lip repair over a 16-year period (1990–2005) by the senior surgeon was performed. The primary outcome was the presence of HTS at 1 year postoperatively. Bivariate analysis and multivariable logistic regression were used to evaluate potential risk factors for HTS, including ethnicity, type and laterality of cleft, and gender.Results: One hundred and eighty patients who underwent cleft lip repair were included in the study. The overall rate of postoperative HTS formation was 25%. Ethnicity alone was found to be an independent predictor of HTS formation. Caucasian patients had the lowest rate of HTS formation (11.8%) and were used as the reference group. HTS rates were significantly higher in the other ethnicities, 32.2% in Hispanic patients (odds ratio [OR]: 3.51; 95% confidence interval [CI]: 1.53–8.85), and 36.3% for Asian patients (OR 4.27; 95% CI: 1.36–13.70). Sex, cleft type, and cleft laterality were not associated with increased rates of HTS.Conclusions: Differences in ethnic makeup of respective patient populations may be a major factor influencing the wide variability of reported HTS rates. Consideration should be given to potential prophylactic treatments for HTS in susceptible ethnic populations.Keywords: cleft lip, hypertrophic scarring, ethnicity, epidemiology

Nuclear protein of the testis (NUT) carcinoma is a rare, undifferentiated carcinoma that is characterized by NUTM1 gene rearrangements. Patients with NUT carcinoma have an overall survival of approximately 5% at 5 years despite a multimodal treatment approach. This report illustrates the management of mandibular NUT carcinoma in a pediatric patient, complemented by a systematic review of head and neck NUT carcinoma. A 5-year-old female presented with an enlarging jaw mass that was diagnosed as BRD4-NUTM1 carcinoma and was treated with hemimandibulectomy and chemoradiation. She remains disease-free 21 months after completion of therapy. A total of 63 patient cases reported in 34 articles were identified in the review. Only 26.9% (14/52) of tumors were correctly diagnosed initially as NUT carcinoma, whereas 73.1% (38/52) were incorrectly diagnosed as another malignancy; the initial diagnosis was not reported for 11 patients. The mandibular tumor subtype was among the rarest reported (n = 1; 1.6%). Combination therapy, including surgery and chemoradiation, was the most common treatment (55.2%). The patient case presented here is a novel case of pediatric mandibular NUT carcinoma. Due to the poor overall survival of patients with NUT carcinoma, aggressive upfront resection with 2-cm margins followed by adjuvant chemoradiation is advocated., Competing Interests: Competing interests None., (Copyright © 2022 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Congenital syngnathia is a very rare condition that presents with several unique challenges. There is no consensus on the best operative approach to manage this condition. Readhesion of surgically separated tissues is a relatively common occurrence. The authors propose that the use of a vertical distraction technique helps maintain adequate separation to prevent relapse of the bony fusion. Long-term follow-up will be required to follow mandibular growth and implications of this treatment option.

Preparation and planning for orthognathic surgery in late adolescence depends on the complexity of unresolved problems with which the patient presents. Different strategies are presented to address these unresolved problems in the adult patient with cleft lip and palate. Different surgical and orthodontic treatments are presented to correct the class III malocclusion in patients with cleft lip and palate in ranges that are analogous to the envelope of discrepancy. For complex cases, the principles of achievability, stability, and esthetics should guide the decision-making process for planning the preparation for orthognathic surgery., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: Management of obstructive sleep apnea in infants with Robin sequence (RS) includes prone positioning during sleep, which conflicts with safe infant sleep data. We examined changes in polysomnography (PSG) parameters for prone versus nonprone body positions in these infants., Design: Pre-post interventional, nonblinded study., Participants: Infants with RS referred for PSG were recruited from craniofacial clinic and inpatient units at Children's Hospital Los Angeles, a tertiary pediatric center. Fourteen infants were recruited, and 12 studies were completed on both body positions; 11 studies were used in the analysis., Interventions: The PSG was divided into nonprone and prone sleep, moving from their usual sleep position to the other position midway in the study., Main Outcome Measures: Data was collected in each position for obstructive apnea-hypopnea index (oAHI), central apnea index (CAI), sleep efficiency (SE), and arousal index (AI). Signed rank test was used to evaluate the change in body position., Results: All infants were term except 1, age 7 to 218 days (mean: 55 days; standard deviation: 58 days), and 8 (57%) of 14 were female. From nonprone to prone sleep position, the median oAHI (16.0-14.0), CAI (2.9-1.0), and AI (28.0-19.9) decreased ( P = .065); SE increased (67.4-85.2; P = .227)., Conclusions: Prone positioning may benefit some infants with RS. However, even those with significant improvement in obstructive sleep apnea did not completely resolve their obstruction. The decision to use prone positioning as a therapy should be objectively evaluated in individual infants.

Primary epithelioid sarcoma (ES) of bone is extremely rare with only 2 reported cases in the English literature. A previously healthy 18-year-old man presented with a 6-month history of right facial numbness and tingling and right eye diplopia. A computerized tomography scan revealed an ill-defined mass with dense osseous matrix centered in the right zygomatic bone. An outside biopsy was read as osteosarcoma. The resection specimen revealed large epithelioid and spindle cells embedded in a prominent hyalinized matrix with focal metaplastic bone formation. The tumor cells were strongly and diffusely positive for AE1/AE3 and epithelial membrane antigen, but a definitive diagnosis of ES was not immediately reached due to the presence of dense hyalinized matrix and weak expression of SAT2B by tumor cells. Deficient INI1 protein expression by immunohistochemistry and homozygous loss of the SMARCB1 gene by chromosomal microarray analysis ultimately justified this tumor's designation as ES.

Alveolar cleft reconstruction has historically relied on autologous iliac crest bone grafting (ICBG), but donor site morbidity, pain, and prolonged hospitalization have prompted the search for bone graft substitutes. The authors evaluated bone graft substitutes with the highest levels of evidence, and highlight the products that show promise in alveolar cleft repair and in maxillary augmentation. This comprehensive review guides the craniofacial surgeon toward safe and informed utilization of biomaterials in the alveolar cleft.A literature search was performed to identify in vitro human studies that fulfilled the following criteria: Level I or Level II of evidence, ≥30 subjects, and a direct comparison between a autologous bone graft and a bone graft substitute. A second literature search was performed that captured all studies, regardless of level of evidence, which evaluated bone graft substitutes for alveolar cleft repair or alveolar augmentation for dental implants. Adverse events for each of these products were tabulated as well.Sixteen studies featuring 6 bone graft substitutes: hydroxyapatite, demineralized bone matrix (DBM), β-tricalcium phosphate (TCP), calcium phosphate, recombinant human bone morphogenic protein-2 (rhBMP-2), and rhBMP7 fit the inclusion criteria for the first search. Through our second search, the authors found that DBM, TCP, rhBMP-2, and rhBMP7 have been studied most extensively in the alveolar cleft literature, though frequently in studies using less rigorous methodology (Level III evidence or below). rhBMP-2 was the best studied and showed comparable efficacy to ICBG in terms of volume of bone regeneration, bone density, and capacity to accommodate tooth eruption within the graft site. Pricing for products ranged from $290 to $3110 per 5 mL.The balance between innovation and safety is a complex process requiring constant vigilance and evaluation. Here, the authors profile several bone graft substitutes that demonstrate the most promise in alveolar cleft reconstruction.

Background: Recent studies indicate that recombinant human bone morphogenetic protein-2 (rhBMP-2) in a demineralized bone matrix scaffold is a comparable alternative to iliac bone autograft in the setting of secondary alveolar cleft repair. Postreconstruction occlusal radiographs demonstrate improved bone stock when rhBMP-2/demineralized bone matrix (DBM) scaffold is used but lack the capacity to evaluate bone growth in three dimensions. This study uses cone beam computed tomography to provide the first clinical evaluation of volumetric and density comparisons between these two treatment modalities., Methods: A prospective study was conducted with 31 patients and 36 repairs of the alveolar cleft over a 2-year period. Twenty-one repairs used rhBMP-2/DBM scaffold and 14 repairs used iliac bone grafting. Postoperatively, occlusal radiographs were obtained at 3 months to evaluate bone fill; cone beam computed tomographic images were obtained at 6 to 9 months to compare volumetric and density data., Results: At 3 months, postoperative occlusal radiographs demonstrated that 67 percent of patients receiving rhBMP-2/DBM scaffold had complete bone fill of the alveolus, versus 56 percent of patients in the autologous group. In contrast, cone beam computed tomographic data showed 31.6 percent (95 percent CI, 24.2 to 38.5 percent) fill in the rhBMP-2 group compared with 32.5 percent (95 percent CI, 22.1 to 42.9 percent) in the autologous population. Density analysis demonstrated identical average values between the groups (1.38 g/cc)., Conclusions: These data demonstrate comparable bone regrowth and density values following secondary alveolar cleft repair using rhBMP-2/DBM scaffold versus autologous iliac bone graft. Cone beam computed tomography provides a more nuanced understanding of true bone regeneration within the alveolar cleft that may contribute to the information provided by occlusal radiographs alone., Clinical Question/level of Evidence: Therapeutic, II.

Diprosopus (Greek; di-, "two" + prosopon, "face"), or craniofacial duplication, is a rare craniofacial anomaly referring to the complete duplication of facial structures. Partial craniofacial duplication describes a broad spectrum of congenital anomalies, including duplications of the oral cavity. This paper describes a 15 month-old female with a duplicated oral cavity, mandible, and maxilla. A Tessier type 7 cleft, midline meningocele, and duplicated hypophysis were also present. The preoperative evaluation, surgical approach, postoperative results, and a review of the literature are presented. The surgical approach was designed to preserve facial nerve innervation to the reconstructed cheek and mouth. The duplicated mandible and maxilla were excised and the remaining left maxilla was bone grafted. Soft tissue repair included closure of the Tessier type VII cleft. Craniofacial duplication remains a rare entity that is more common in females. The pathophysiology remains incompletely characterized, but is postulated to be due to duplication of the notochord, as well as duplication of mandibular growth centres. While diprosopus is a severe deformity often associated with anencephaly, patients with partial duplication typically benefit from surgical treatment. Managing craniofacial duplication requires a detailed preoperative evaluation as well as a comprehensive, staged treatment plan. Long-term follow up is needed appropriately to address ongoing craniofacial deformity., (Published by Elsevier Ltd.)

A Tessier no. 7 cleft is a lateral facial cleft which originates from the oral cavity and extends towards the tragus, involving both soft-tissue and skeletal components. A male patient presenting with both maxillary jaw duplication and bilateral Tessier no. 7 clefts, which has been reported only twice in the literature, is described. Bilateral facial clefts, macrostomia and chondro-cutaneous remnants were noted, which were repaired and resected. With further growth, facial asymmetry and asymmetric facial nerve dysfunction became apparent. Radiographic examination showed an accessory maxillary jaw and a flattened and hypoplastic right coronoid process. A maxillary alveolar cleft was also present between the left second bicuspid and the second permanent molar. This case may represent an under-recognized phenotype with an unusual combination of maxillary jaw duplication, macrostomia, Tessier no. 7 clefts, and chondro-cutaneous remnants. A long-term follow-up of these patients is recommended as they often develop craniofacial deformities later in life., (Copyright © 2012 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Ankyloblepharon-ectodermal defect-cleft lip and/or palate (AEC syndrome, also known as Hay-Wells syndrome) is an autosomal dominant disease caused by mutation in the p63 gene that is primarily characterized by facial clefting, presence of ankyloblepharon, ectodermal dysplasia, and scalp erosion. Scalp erosion is perhaps the most debilitating manifestation of AEC due to its problematic treatment that is fraught with failure given the underlying pathology of the p63 mutation causing dysfunctional wound healing. Management is often targeted in a stepwise fashion, beginning with daily baths, light debridement, and emollients and progressing to extensive skin excision. Skin grafting has limited success and, inevitably, infections requiring aggressive debridement and antibiotic therapy result from dysfunctional healing. The use of acellular dermal matrix for treatment of scalp erosion is a novel approach attempted in a patient with severe scalp disease. Here we report her case and the failure of treatment, along with possible explanations and suggestions for future therapy.

Context: Surgical management for severe obstructive sleep apnea has been tracheostomy, which has significant morbidity., Objective: To determine the efficacy of internal mandibular distraction in treating severe obstructive sleep apnea in infants and neonates., Design: Retrospective review of medical records of 29 patients who underwent internal mandibular distraction for obstructive sleep apnea secondary to micrognathia., Setting: Nonprofit, academic, pediatric medical center., Patients: A total of 29 infants with obstructive sleep apnea were studied. Nine were included in the respiratory failure group requiring intubation prior to distraction surgery. The other 20 were included in the respiratory distress group and underwent preoperative polysomnography that assessed the severity of obstructive sleep apnea as measured by the apnea-hypopnea index. One patient expired following surgery; the remaining 28 underwent postoperative polysomnography determining their postoperative apnea-hypopnea index., Interventions: Bilateral mandibular distraction with internal microdistractors., Main Outcome Measure: Improvement in the apnea-hypopnea index or extubation., Results: The nine respiratory failure patients avoided tracheostomy and were successfully extubated postdistraction. Eight in this group had postoperative polysomnographies showing a mean apnea-hypopnea index of 3.13 (range, 0 to 13.9). All 20 patients in the respiratory distress group underwent polysomnography and showed improved apnea-hypopnea indices (p < .001). The mean pre-op apnea-hypopnea index was 39.7 (range, 4.5 to 177), and the mean post-op apnea-hypopnea index was 5.8 (range, 0 to 34). Average improvement in the apnea-hypopnea index was 33.9. The mean follow-up period was 18.7 months (1.6 to 45.2 months)., Conclusions: Infants with micrognathia and obstructive sleep apnea may avoid tracheostomy and its inherent risks and complications by undergoing internal mandibular distraction, which is a viable alternative to tracheostomy.

Dentoalveolar infections represent a wide spectrum of conditions, from simple localized abscesses to deep neck space infections. The initial assessment of the patient with a dentoalveolar infection requires considerable clinical skill and experience, and determines the need for further airway management or emergent surgical therapy. Knowledge of head and neck fascial space anatomy is essential in diagnosing, understanding spread, and surgically managing these infections. Oral and maxillofacial surgeons must make use of their wide spectrum of clinical skill and knowledge to effectively evaluate and treat patients with dentoalveolar infections., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Recurrent giant cell tumors refractory to various treatment modalities are challenging dilemmas for the most experienced practitioner. We report a case of a multiply recurrent aggressive giant cell tumor diagnosed at 10 months of age with extensive involvement of the maxillae and mandibles unresponsive to multiple therapeutic modalities. The various treatments attempted in this patient including conventional therapies as well as the original use of bevacizumab (Avastin) are described.

The following case reports on the successful use of Integra® (Integra LifeSciences, Plainsboro, NJ) as an alternative for coverage of exposed tibia in a patient with necrotizing fasciitis and significant comorbidities. Necrotizing fasciitis is a generalized term that is used to describe soft tissue infections in which necrosis of the fascia and subcutaneous tissue occurs. Necrotizing fasciitis will often begin with disproportionate pain, localized erythema, and skin discoloration with vesicle formation. As the disease progresses along fascial planes, an area of deep necrosis develops with eventual signs of systemic toxicity and sepsis. Diagnosis of this condition can be quite challenging, and the diagnostic value of laboratory tests is limited. Aggressive treatment of necrotizing fasciitis is extremely important; the infection develops rapidly and can quickly lead to systemic toxicity and death. Traditionally, management of necrotizing fasciitis consists of resuscitation and stabilization of the patient, aggressive surgical debridement of all nonviable tissue, and the administration of broad-spectrum antibiotics. Reconstructive surgery is indicated for many patients who have recovered from necrotizing fasciitis. Integra, an artificial dermal regeneration template (DRT), has emerged as an adjunct to split-thickness grafts or tissue transfers. This case exemplifies the potential for DRT to provide another, more direct alternative for coverage of a complex, exposed wound. .

Background: The double-opposing Z-plasty palatal repair, as reported by Dr. Furlow, is one of the most popular methods of primary cleft palate repair. However, the repair as originally described is difficult to perform on wide palatal clefts. The authors have modified the original repair by altering the hard palatal flap design to allow for better mobilization and improved closure of the cleft., Methods: The authors performed a retrospective review of 500 consecutive children undergoing double-opposing Z-plasty cleft palate repairs with or without islandization of the hemipalate on its vascular pedicle over a 10-year period at Children's Hospital Los Angeles. Children were evaluated based on their age at the time of repair, extent of cleft, and occurrence of postoperative fistulas., Results: Three hundred thirty-two children underwent pedicle lengthening and 168 children did not. The overall fistula rate in this series was 5.0 percent. When the experience of the five cleft surgeons in this series was combined, patients undergoing pedicle lengthening had significantly lower fistula rates (2.1 percent) than patients undergoing palatoplasty without pedicle lengthening (10.6 percent). Patient gender, age, and extent of clefting did not correlate with the rate of fistula formation in this study. There was only one partial flap loss, early in the authors' series., Conclusion: The authors' data suggest that a double-opposing Z-plasty palate repair performed with islandization results in lower postoperative fistula rates when compared with repair performed without islandization.

Current techniques for model surgery and occlusal splint fabrication lack the ability to mark, measure and plan the position of the orbital rim for LeFort III and Monobloc osteotomies. This report describes a model surgery technique for planning the three dimensional repositioning of the orbital rims. Dual orbital pointers were used to mark the infraorbital rim during the facebow transfer. These pointer positions were transferred onto the surgical models in order to follow splint-determined movements. Case reports are presented to illustrate how the model surgery technique was used to differentiate the repositioning of the orbital rim from the occlusal correction in single segment and combined LeFort III/LeFort I osteotomies.