Extracapsular versus intracapsular tonsillectomy: Outcomes in children with a focus on developmental delay.

INTRODUCTION: Outcomes following intracapsular tonsillectomy (IT) have not been well established in children with developmental delays. The objective of this study was to compare outcomes and complications between intracapsular and extracapsular tonsillectomy (TT) in pediatric patients with developmental delay (DD) in comparison to non-developmentally delayed children. METHODS: This is a retrospective study of pediatric patients with DD undergoing tonsillectomy between 2016 and 2019 at a tertiary care hospital. This group included patients with Down Syndrome, Autism Spectrum Disorder, other genetic syndromes, and patients with a diagnosis of global developmental delay. Outcomes and complications were analyzed for IT and TT. RESULTS: 2267 charts were reviewed, and 320 patients were identified with DD. Of those, 72 patients underwent IT and 248 underwent TT. In the DD cohort, the IT group had a shorter length of stay (0.97 vs 1.7 days, p < .0001) and was less likely to receive post-operative narcotic medication (2.8% vs 35%, p < .0001) and corticosteroids (9.7% vs 64%, p < .0001) during their hospital stay. Reductions in emergency room (ER) visits (5.6% vs 10%, p = .21) and post-op bleeding (PTH) (1.4% vs 4.8%, p = .31) for IT vs TT were not statistically significant in the DD group. In the NDD group, fewer patients undergoing IT returned to the ER (11% vs 2.3%, p < .0001) or had PTH (4.8% vs 0.25%, p, 0.0001) as compared to those children undergoing TT. There was no difference between parental report of symptom improvement between the groups (39% vs 33%, p = .39). Analysis of 180 patients with preoperative and postoperative sleep study data revealed post-op Apnea Hypopnea Index (AHI) improved with both techniques (74% TT vs 79% IT, p = .7). There were no differences noted for persistent obstructive sleep apnea (OSA) among the two techniques for both study groups (p = .63). CONCLUSION: Children with DD undergoing IT have reduced length of stay and reduced inpatient administration of post-operative opioids and steroids. IT has comparable efficacy to TT in treating symptoms of pediatric sleep apnea with a better safety profile. Overall, children undergoing IT return to the operating room less frequently than those undergoing TT. Longer follow-up studies will be needed to evaluate rate of tonsil regrowth, risk of revision surgery and persistence of OSA in these patients.

Surgical Approach in a Patient With Agnathia-Otocephaly Complex: Three-Stage Mandibular Distraction Protocol.

Agnathia-otocephaly complex (AOC) is a rare malformation complex of the first pharyngeal arch that is characterized by agnathia/dysgnathia, microstomia, aglossia/hypoglossia and variable displacement of the ears. Only 11 post-infancy patients with severe AOC have been described in the literature, and the incidence of this malformation complex is estimated to be 1 per 70,000 births. In this brief clinical study, the authors describe the case of an 18-year-old female diagnosed with AOC who underwent a 3-step mandibular distraction protocol with an external distraction device. The surgical protocol the authors used was unique in that we first placed a tissue expander in the submental area to enlarge the skin envelope in an effort to mitigate skeletal relapse from soft tissue forces. Furthermore, the way in which the authors slowed the activation of the distraction device to allow for soft tissue healing behind the pins was a novel component of the patient's treatment. The 3-step mandibular distraction protocol the authors present in this study increased the length of the mandible by 20 mm, and nearly doubled the size of the patient's mandible from an initial volume of 3.62 cm to a post-operative volume of 6.89 cm. Future surgeries will aim to improve the function of our patient's expanded mandible. Most important of all, the surgical treatment authors are presenting led to a significant improvement in our patient's physical appearance and 3d quality of life.

Pediatric invasive fungal rhinosinusitis: An investigation of 17 patients.

PURPOSE: To investigate outcomes of pediatric patients at a single institution with invasive fungal rhinosinusitis (IFRS) and to determine variables that impact overall survival. METHODS: All pediatric patients at a large tertiary children's hospital diagnosed with IFRS confirmed by surgical pathology from 2009 to 2015 were retrospectively reviewed. Demographics, underlying diseases, symptoms, antifungal therapy, absolute neutrophil count (ANC), surgical management,and outcomes were analyzed. RESULTS: Seventeen patients were identified with IFRS with an average age of 8.7 years and 53% male. Hematologic malignancy was the most common (n = 13) underlying disease. The most common presenting symptoms were fever (82%) and congestion (41%). 15 patients had severe neutropenia (Absolute Neutrophil Count (ANC) < 500) within 2 weeks prior to diagnosis. The average ANC at time of diagnosis was 1420 cells/uL. 16 patients were treated with serial nasal endoscopy and debridement, while 1 patient was treated with an open approach. 16 received combination antifungals while 1 was treated with amphotericin monotherapy. The most common genus cultured was Fusarium (n = 6). The average number of surgical interventions was 3.4, with the average interval between interventions 6.2 days. 13 of 17 (76%) were cleared of IFRS. Overall survival at 6 months was 41%. CONCLUSION: Pediatric IFRS is a life-threatening disease that requires a coordinated surgical and medical approach. Despite a relatively high local control rate, overall mortality remains disappointingly high, reflecting the disease's underlying pathogenesis - lack of host defense and risk of disseminated fungal infection. Further investigation is necessary to reveal optimal management with regards to antifungal therapy, surgery, and utility of labs.

Intraneural perineurioma: a systematic review with illustrative cases.

CONTEXT: Intraneural perineurioma may be confused with other "onion bulb" Schwann cell entities (localized hypertrophic neuropathy, reactive/demyelinating processes, or inherited polyneuropathies of Charcot-Marie-Tooth/Dejerine Sottas) due to similar clinical, radiologic, and histologic features. Perineurial and Schwann cells can only be differentiated by ultrastructure and immunohistochemsitry. OBJECTIVE: To identify and summarize the clinicopathologic features of true cases of intraneural perineurioma from the English language literature. DATA SOURCES: A systematic review was performed on definitive intraneural perineuriomas identified through Medline. Baylor College of Medicine-affiliated hospitals' anatomic pathology databases yielded 2 illustrative intraneural perineurioma cases. STUDY SELECTION: Intraneural perineurioma inclusion criteria consisted of characteristic histology and confirmation of perineurial cell lineage by either immunohistochemistry (epithelial membrane antigen positive, S100 protein negative) and/or ultrastructural analysis (thin cytoplasmic processes with an incomplete basal lamina, poorly formed tight junctions, and pinocytotic vesicles). DATA EXTRACTION: Clinicopathologic data were extracted from all identified articles, with subsequent statistical analysis of the following parameters: age, sex, race, tumor location, tumor size, duration of symptoms prior to diagnosis, treatment modalities and outcomes measures, follow-up assessment for tumor recurrence and metastasis, clinical features (history of trauma, motor/sensory abnormalities, clinical/family history), and diagnostic workup (routine histology, immunohistochemistry, ultrastructural analysis, and molecular/cytogenetic characteristics). CONCLUSIONS: Intraneural perineurioma is a neoplastic proliferation of perineurial cells with unique immunohistochemistry and ultrastructural features, and it is distinct from other onion bulb Schwann cell-derived entities. Despite harboring molecular abnormalities of the long arm of chromosome 22, intraneural perineurioma has not been associated with neurofibromatosis. Intraneural perineurioma is a benign peripheral nerve sheath tumor that does not recur or metastasize.