Jerry W. Chao

Giant Congenital Sclerosing Blue Nevus of the Scalp Presenting with Rapidly Disseminated Fatal Metastases in a Pediatric Patient.

1 Michael V. DeFazio, MD Jerry W. Chao, MD Albert K. Oh, MD Gary F. Rogers, MD Division of Plastic and Reconstructive Surgery Children’s National Medical Center Wash. Sir: G congenital blue nevi (GCBN) are rare dermal melanocytic proliferations that are distinguished by their large size, aggressive behavior, and propensity for malignant degeneration.1 To date, only 18 cases of GCBN involving the scalp have been reported. Among these, local invasion into the calvaria/dura (n = 10), malignant transformation (n = 6), and disseminated metastases (n = 5) were observed.2 Although the precise nature of malignant change is unclear, adults with histopathologically cellular GCBN are most commonly affected.2 We report the first case of a sclerosing GCBN of the scalp presenting with rapidly progressive fatal metastases in a toddler. A 17-month-old girl presented for evaluation of a 15 × 20-cm blue-gray plaque of the scalp that had grown commensurately with the child since birth. The patient’s history was notable for posterior encephalocele repair during infancy, resulting in an occipital bone defect and chronic/stable ulceration at the vertex. Initial magnetic resonance imaging demonstrated a noninvasive parietooccipital mass with an underlying 12-cm2 calvarial defect. Multiple incisional biopsies from ulcerated and nonulcerated regions revealed histopathologic features of sclerosing GCBN with extensive dermal/subcutaneous infiltration (ie, spindle-shaped melanocytes/dense collagenous stroma). Given these benign findings, conservative observation/local wound care was pursued in the hopes that dural-induced calvarial regeneration would improve the safety of tumor resection from the underlying scarred dura. However, the patient presented 7 months later with rapidly enlarging subcutaneous nodules, necrosis, and new-onset pain, vomiting, and ataxia (Fig. 1). Repeated magnetic resonance imaging demonstrated interval development of 2 sublesional masses with intracranial extension and calvarial/dural invasion (Fig. 2). A histopathologic diagnosis of melanoma (ie, atypical epithelioid melanocytes, large/pleomorphic nuclei, necrosis, numerous mitoses) arising within the areas reminiscent of sclerotic blue nevus was confirmed. Immunostains for BRAF V600 were negative; however, homozygous deletion of the 9p21 locus (CDKN2A/P16 tumor suppressor) was identified in 40% of cells analyzed by fluorescent in situ hybridization. Total body positron emission tomography revealed disseminated visceral metastases. The patient subsequently died 3 weeks later from widespread metastatic disease. This case presents several unique findings that deviate from previously reported trends. Morphologically, the majority of GCBN display cellular histopathologic features, which correlate well with their proclivity for invasive growth and malignant degeneration.1,2 In contrast, sclerosing blue nevi are predominantly benign, solitary, well-circumscribed nodules. Focal malignant melanoma arising within this subtype has been reported only once; however,

Factors Predicting Hospital Length of Stay Following Pharyngeal Flap for Velopharyngeal Insufficiency

Objective: Routine hospital admission following pharyngeal flap (PF) to correct velopharyngeal insufficiency (VPI) is the standard at most hospitals. Nevertheless, there is increasing resistance from third-party providers to approve stays longer than a “short stay” (23-hour) observation period. The purpose of the current study was to evaluate length of stay (LOS) and document potential influencing factors following PF. Design: Retrospective chart review. Demographic and perioperative data were collected, and statistical analyses were performed to determine associations with hospital length of stay (LOS). Readiness for discharge was determined by oral intake, analgesic requirement, and respiratory status. Setting: Tertiary care children’s hospital Participants: All patients undergoing PF for VPI between 1990 and 2014. Outcome measures: (1) LOS, (2) % satisfying all discharge criteria within a 23-hour observational time frame. Results: Seventy-five patients were studied, with an average age of 6.8 years. Mean LOS was 65.4 hours. Only 11 patients (14.9%) met all discharge criteria by 23 hours. Multivariate predictors of shorter LOS were increasing patient age, male gender, lack of syndromic association, administration of an intraoperative antiemetic and steroids, and shorter anesthetic duration. Time to first oral intake correlated positively with LOS. Administration of intraoperative antiemetics increased the odds of meeting all discharge criteria within 23 hours by a factor of 12. Conclusions: Identification of factors associated with LOS after PF may allow providers to predict and potentially mitigate hospital stays. Nevertheless, most patients undergoing PF are not clinically ready for discharge within a short-stay (23-hour) observation period.

The “Double” Tessier 7 Cleft: An Unusual Presentation of a Transverse Facial Cleft

Congenital macrostomia, or Tessier number 7 cleft, is a rare craniofacial anomaly. We present a unique patient with bilateral macrostomia that consisted of a “double” transverse cleft on the left side and a single transverse cleft on the right side. A staged reconstructive approach was used to repair the “double” left-sided clefts. This staged technique produced a satisfactory aesthetic and functional outcome.

Efficacy of immediate replacement of cranial bone graft following drainage of intracranial empyema

OBJECTIVE Intracranial empyema is a life-threatening condition associated with a high mortality rate and residual deleterious neurological effects if not diagnosed and managed promptly. The authors present their institutional experience with immediate reimplantation of the craniotomy flap and clarify the success of this method in terms of cranial integrity, risk of recurrent infection, and need for secondary procedures. METHODS A retrospective analysis of patients admitted for management of intracranial empyema during a 19-year period (1997-2016) identified 33 patients who underwent emergency drainage and decompression with a follow-up duration longer than 6 months, 23 of whom received immediate bone replacement. Medical records were analyzed for demographic information, extent and location of the infection, bone flap size, fixation method, need for further operative intervention, and duration of intravenous antibiotics. RESULTS The mean patient age at surgery was 8.7 ± 5.7 years and the infections were largely secondary to sinusitis (52.8%), with the most common location being the frontal/temporal region (61.3%). Operative intervention involved removal of a total of 31 bone flaps with a mean surface area of 22.8 ± 26.9 cm2. Nearly all (96.8%) of the bone flaps replaced at the time of the initial surgery were viable over the long term. Eighteen patients (78.3%) required a single craniotomy in conjunction with antibiotic therapy to address the infection, whereas the remaining 21.7% required more than 1 surgery. Partial bone flap resorption was noted in only 1 (3.2%) of the 31 successfully replaced bone flaps. This patient eventually had his bone flap removed and received a split-calvaria bone graft. Twenty-one patients (91.3%) received postoperative CT scans to evaluate bone integrity. The mean follow-up duration of the cohort was 43.9 ± 54.0 months. CONCLUSIONS The results of our investigation suggest that immediate replacement and stabilization of the bone flap after craniectomy for drainage of intracranial empyemas has a low risk of recurrent infection and is a safe and effective way to restore bone integrity in most patients.

Pachyonychia Congenita in a Toddler.

1 Dear Sir: W report management of a 3-year-old boy with genetically confirmed pachyonychia congenita (PC), a rare (prevalence, 1:5,000–10,000) autosomal dominant genetic disorder characterized by excessive keratinization of the glabrous skin of the hands and feet and nail thickening that can mimic onychomycosis.1 Our patient was referred for treatment of painful, debilitating thickenings on the palms and selected nail beds of both hands and feet that has severely limited his ability to ambulate (Fig. 1). Prior temporizing measures, including sanding of the lesions, were ineffective in functional improvement. PC is caused by autosomal dominant mutations in selected keratin genes, has 2 principle subtypes: PC type 1 (Jadassohn-Lewandowski syndrome, KRT6A, and KRT16 genes) and PC type 2 (Jackson-Lawler syndrome, KRT6B, and KRT17 genes).2 Approximately 50% of PC cases are inherited. All subtypes of PC manifest with hypertrophic nail dystrophy, and focal, painful palmoplantar keratoderma, but other clinical symptoms can include palmoplantar blistering, oral leukokeratosis, follicular keratosis on the trunk and extremities, dystrophic toenails and fingernails, pilosebaceous cysts, palmoplantar hyperhidrosis, hoarseness, pili torti, and natal teeth.2 Treatment alternatives for painful hyperkeratoses have met with limited success due to short-term mitigation and high recurrence rates. Conservative measures include limiting friction and trauma to feet by minimizing walking or standing, though compliance in young children limits feasibility. Reducing hydration of the epidermis with wicking socks and ventilated footwear may help. Mechanical treatments such as filing, grinding, cutting, and clipping of affected nails are somewhat effective in reducing symptoms in mild cases.1 Reports of surgical management are sparse in the literature. Electrofulguration, deep curettage, and radical excision with autologous reconstruction have met with variable success due to recurrence and significant associated morbidity with more radical techniques.3–5 The most common pharmacological management employs retinoid agents that may cause thinning of the epidermis with subsequent painful blistering and secondary infections.1,2 Current medical research is examining the possible role of using mutationspecific small interfering RNA, rapamycin, anti-tumor necrosis factor biologics, and botulism toxins as treatment to reduce keratinization and patient symptoms.1,2 Our patient underwent blunt debridement of the affected skin and nails along a natural plane of dissection in the deep dermal layer (Fig. 2). This strategy was chosen by the parents, despite the risk of recurrence, to limit the morbidity of the procedure and shorten the recovery. The patient was discharged home with local wound care and healed uneventfully by 7 days. At 6-month follow-up, the toddler ambulates with minimal discomfort, and there is only minor thickening of the plantar and palmar skin.

Intradiploic Epithelial Inclusion Cyst of the Cranium Mimicking Fibrous Dysplasia.

Intradiploic inclusion cysts are exceedingly rare in the pediatric population. The authors present a 16-year-old male patient who presented with a large growing calvarial mass with a preoperative diagnosis of fibrous dysplasia based on radiologic imaging. Craniectomy followed by autogenous reconstruction was performed. Histopathological examination revealed a relatively small inclusion cyst of the intradiploic space, surrounded by reactive bone. This patient demonstrates a highly unusual presentation of a rare entity, and the authors discuss the diagnosis and management of intradiploic inclusion cysts.

Columellar Reconstruction After Nasal Continuous Positive Airway Pressure Associated Necrosis.

Abstract Though a life-saving modality in neonatal intensive care units, nasal continuous positive airway pressure (nCPAP) carries a small risk of irreversible ischemia and necrosis of the columella due to the configuration of the pressure delivery system. Iatrogenic injuries to the columella after nCPAP use result in a spectrum of disfigurement and functional airway obstruction. The authors performed a retrospective review of patients evaluated for nCPAP-related columellar deformities by the Division of Plastic and Reconstructive Surgery at the authors’ institution over a 10-year period to assess reconstructive outcomes. Of 7 patients evaluated, 3 underwent reconstruction using a combination of cartilaginous framework reshaping and local tissue flaps. After a mean follow-up period of 78 months, patients had satisfactory aesthetic and functional results. Based on the authors’ observations, columellar necrosis secondary to nCPAP can be divided into 3 categories: Type A demonstrates mild notching of the columella; Type B has an absent columella without notable nasal tip depression; Type C has an absent columella with nasal tip depression, with or without external nasal valve obstruction. Reconstructive needs should be individually tailored based on the degree of nasal tip depression, cartilaginous support, and soft tissue availability.