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Summary: A 2-year-old girl presented with a left middle ear cholesteatoma. A CT scan showed a mass in the left middle ear starting from the mesotympanum extending through a bony canal defect into the parapharyngeal space, deep to the facial nerve and ending in the submandibular region. The patient underwent surgical excision of the first branchial cleft cyst by superficial parotidectomy with facial nerve dissection and mastoidectomy with middle ear exploration. This case illustrates a rare presentation of a first branchial cleft cyst with histologic and anatomical features of both types of Work''s classification of brachial cleft anomalies. [Copyright &y& Elsevier]

Background: Congenital cholesteatoma (CC) of middle ear is a rare entity that may go undiagnosed for years. Patients with CC who are diagnosed at a later stage of disease have poor outcome. There is controversy regarding the best way to delineate CC preoperatively. More specifically, the need to obtain preoperative computed tomography (CT) scan in all cases of CC is debated. Objectives: This study was conducted to determine factors that may influence the outcome of surgery in CC as well as the value of obtaining preoperative CT scan in CC. Method: A retrospective chart review of all patients with a diagnosis of middle ear cholesteatoma operated on between 1994 and 2000 was carried out. Patients with CC were identified using the criteria proposed by Levenson and Parisier. Results: Thirty-five patients with CC were identified. In 30 (86%) patients, the diagnosis was made during ear examination and the remaining five (14%) patients were diagnosed during myringotomies. Preoperative CT scans were available in 17 patients. The findings on CT scans were classified into four categories based on the ossicular chain and mastoid septae status as well as the presence or absence of middle ear and mastoid opacification. Intraoperatively, 22 (63%) patients were found to have extensive cholesteatomas with or without extension beyond the mesotympanum. Eleven of these 22 patients had ossicular chain erosion and five were later found to have recidivism. Preoperative CT scan accurately predicted the extent of the cholesteatoma seen during surgery in 14/17 (82%) and ossicular chain status in 15/17 (88%), while micro-otoscopy predicted the extent of the existing pathology in only 10/35 (29%). Intraoperative location and size of CC influenced the type of surgical approach, status of ossicular chain, postoperative hearing level and rate of recidivism. Conclusions: Children still present with late stage CC. Micro-otoscopy is insufficient to clearly delineate the extension of CC. Preoperative CT scan is essential in defining the extent of existing pathology. The intraoperative CC size and location influence the outcome of surgery. Early surgical intervention and long-term follow-up are essential. [Copyright &y& Elsevier]

Background: A recurrent tracheo-esophageal fistula can complicate esophageal atresia and tracheo-esophageal fistula (TEF) repair in children. Therapeutic approaches and the rate of recurrence vary widely. Most reports are limited by small cohorts and short-term follow-up, and rates of re-recurrence are substantial, making it difficult to select the treatment of choice. We aimed to review our experience with the treatment of recurrent TEF using posterior tracheopexy, focusing on operative risks and long-term outcomes.Study Design: We conducted a retrospective review of patients with esophageal atresia TEF with recurrent TEF treated at 2 institutions from 2011 to 2020. We approach recurrent TEFs surgically. Once the TEF is divided and repaired, the membranous trachea is sutured to the anterior longitudinal ligament of the spine (posterior tracheopexy) and the esophagus is rotated into the right chest (rotational esophagoplasty), separating the suture lines widely. To detect re-recurrence, patients undergo endoscopic surveillance during follow-up.Results: Sixty-two patients with a recurrent TEF were surgically treated (posterior tracheopexy/rotational esophagoplasty) at a median age of 14 months. All had significant respiratory symptoms. On referral, 24 had earlier failed endoscopic and/or surgical attempts at repair. Twenty-nine required a concomitant esophageal anastomotic stricturoplasty or stricture resection. Postoperative morbidity included 3 esophageal leaks, and 1 transient vocal cord dysfunction. We have identified no recurrences, with a median follow-up of 2.5 years, and all symptoms have resolved.Conclusions: The surgical treatment of recurrent TEFs that incorporates a posterior tracheopexy and rotational esophagoplasty is highly effective for preventing re-recurrence with low perioperative morbidity. [ABSTRACT FROM AUTHOR]

Introduction Tonsillectomy is the second most common procedure performed in the United States. Over 530,000 tonsillectomies are performed on children under 15 years of age in the United States, accounting for 16% of surgeries in this age group, resulting in missed school for patients of school-age and also resulting in missed work for caregivers. This study compared parent preferences for in-clinic follow-up (CFU) to telephone interview follow-up (TFU) after tonsillectomy. Materials and methods One hundred twenty-one parents of children who underwent a tonsillectomy and/or adenoidectomy were recruited to complete a survey about their child's post-operative visit. Results Statistical analyses were performed using t -test, Wilcoxon rank-sum, and Fischer's exact tests where appropriate. 60.3% of the surveys were completed as a TFU and the remainder were completed as a CFU. There were no statistical differences in the children's age, the time to follow-up, satisfaction with their follow-up, or the frequency of unresolved symptoms. Of parents receiving TFU, 91.8% disagreed they would have preferred a CFU, with 86.3% strongly disagreeing, and only 5.5% expressing that they would have preferred a CFU. Of the parents with CFU, 47.9% expressed a preference for a TFU. For CFU, 43.9% of parents missed work and 58.1% of their school-age children missed school. Conclusion Our study results indicate that parents receiving phone follow-up strongly preferred this method to an in-clinic follow-up, and that nearly half of all parents receiving in-clinic follow-up would have preferred a telephone follow-up. In select patients, telephone follow-up after tonsillectomy may increase patient satisfaction and decrease days of missed work and school. [ABSTRACT FROM AUTHOR]

Objective To review the clinical presentation, histology, staging, treatment modalities, and survival for pediatric head and neck rhabdomyosarcoma (non-orbital). Study design Retrospective chart review at a tertiary pediatric hospital of children treated over 18 years (1996–2014) for primary head and neck non-orbital rhabdomyosarcoma. Methods Medical charts were examined for clinical presentation, staging, histology, genetic abnormalities, treatment modalities, recurrence and complications from treatment. Results Our cohort was 17 children (7 male, 10 female) with rhabdomyosarcoma with a median age of 6.3 years (range <1–19). The majority of tumors were of parameningeal location (13/17) with embryonal histology (11/17). Twenty-nine percent (5/17) demonstrated advanced metastatic disease at initial referral. Fifty-three percent (9/17) had skull base erosion and/or cranial nerve deficits. PET CT scan was performed in 4 patients. The overall survival was 75% for the duration of the study. Primary surgical excision was performed in all 4 patients with nonparameningeal tumors as compared to only 1 patient with a parameningeal tumor. All received chemotherapy and radiotherapy, as none had completely resectable disease. Conclusion Pediatric non-orbital primary rhabdomyosarcoma of the head and neck usually has a rapid onset and presents with advanced disease. Our analysis found that the majority of patients in our series had a cranial neuropathy at presentation, which highlights how advanced the disease is in these patients at presentation. The first mode of surgical intervention should be directed toward biopsy in junction with a metastatic work-up. Primary excision with negative microscopic margins for nonparameningeal rhabdomyosarcoma is ideal to spare radiotherapy but was not achievable in our cohort. The benefits of second-look biopsy after chemotherapy and radiation are still unproven; however, we believe that it was beneficial in two patients in our review for further resection thus decreasing subsequent radiation. Fluorodeoxy- d -glucose positron emission tomography (PET) to evaluate disease post treatment may further define the role for second look surgery. [ABSTRACT FROM AUTHOR]

Abstract: Objective: To determine variables predictive of recovery room times in pediatric outpatient adenotonsillectomy. Study design: Retrospective case–control. Methods: One-hundred ninety consecutive patients undergoing outpatient adenotonsillectomy at an ambulatory surgery center of a tertiary-care free standing pediatric hospital were grouped into upper and lower deciles of recovery room times. Twenty-one variables were analyzed to determine which variables are predictive of prolonged recovery time. Univariate and multivariate analyses were performed. Results: Of the 190 patients, mean recovery room time was 103min (SD 53.1), 22 patients were in the lower decile (mean recovery room time of 63±6min) and 17 patients were in the upper decile (155±40min, P <0.0001). Of the 21 variables analyzed, post-anesthesia care unit (PACU) nursing staff was the only significant predictor of prolonged recovery room time. Compared with one PACU nurse, other nurses (N =5) predicted a longer recovery time (OR=10.8, 95% CI 2.0–59.5, P =0.0017). This association remained significant when controlling for anesthesiologist and surgeon (OR=8.8, 95% CI 1.5–50.9, P =0.0072). There were no complications in any patients. Conclusions: Recovery room times after outpatient adenotonsillectomy vary significantly (mean 103min (SD 53.1), range 50–241min). Of potential predictors, only the human factor (PACU nursing staff) was associated with prolonged recovery room times, independent of surgeon and anesthesiologist. Development of standardized protocols for nurses to use for discharge has the potential to increase throughput for adenotonsillectomy patients in an outpatient surgery center setting. [Copyright &y& Elsevier]

Summary: Objective: Epidermodysplasia verruciformis is a skin disease characterized by abnormal susceptibility to human papilloma viruses. Recently four mutations in the Epidermodysplasia verruciformis 1 gene (EVER1, also known as TMC6) have been associated with the disease. Because of the phenotypic similarity between Epidermodysplasia verruciformis and recurrent respiratory papillomatosis, we decided to investigate whether any of these mutations accounts for the susceptibility to human papilloma viruses in subjects with recurrent respiratory papillomatosis (RRP). Methods: Allele-specific PCR and restriction fragment length polymorphisms (RFLPs) were employed for genotyping a cohort of 101 patients with recurrent respiratory papillomatosis. Results: None of these four mutations were found in the studied subjects. Conclusion: The absence of these mutations in RRP patients might indicate that EVER 1 alleles are not associated with susceptibility to RRP, or that other, as yet unidentified, mutations in the Epidermodysplasia verruciformis 1 gene, might account for the susceptibility to RRP. [Copyright &y& Elsevier]