Clinical relevance of inflammation on rectal biopsy for Hirschsprung disease: An outcomes analysis.

OBJECTIVES: Inflammation on diagnostic rectal biopsy for children with suspected Hirschsprung disease (HSCR) is reported on pathology, and its significance is unknown. We describe the management and outcomes of a cohort with inflammation on rectal biopsy compared to those without. Specifically, to address the hypothesis that inflammation on diagnostic biopsy is associated with increased complication rates irrespective of intervention type and timing. METHODS: A single institution retrospective review of children with HSCR who underwent biopsy and endorectal pull-through (ERPT) from 2010 to 2020 was performed. The primary outcome was overall complications at 30-days following ERPT. Secondary outcomes included timing and type of operative intervention as well as postoperative enterocolitis diagnosed within 6-months of ERPT. RESULTS: Forty-nine children were identified; inflammation was present on diagnostic biopsy for 17 children. Those with inflammation were more likely to have clinical evidence of enterocolitis at the time of biopsy (p = 0.001) and were more likely to undergo leveling colostomy before ERPT (p = 0.01). Children with inflammation had a higher anastomotic leak rate (p = 0.04). Subgroup analysis of patients with inflammation undergoing primary ERPT versus leveling colostomy demonstrated no significant difference in outcomes following definitive ERPT. CONCLUSIONS: Our study suggests inflammation on diagnostic rectal biopsy for HSCR is associated with increased anastomotic leak rates. While additional prospective studies are indicated, attention to methods of mitigating inflammation and confirming its resolution before definitive pull-through may be of benefit for improving clinical outcomes in patients found with inflammation on diagnostic rectal biopsy.

A single institution's experience with the management of peripheral bronchial atresia.

PURPOSE: Peripheral bronchial atresia is a pulmonary abnormality diagnosed on postnatal computed tomography after prenatal imaging reveals a congenital lung lesion. Debate regarding management of this abnormality prompted us to review our institution's practice patterns and outcomes. METHODS: All patients diagnosed with bronchial atresia were assessed from 6/2014 to 7/2020. Pediatric radiologists were surveyed to delineate computed tomography criteria used to diagnose peripheral bronchial atresia. Criteria were applied in an independent blinded review of postnatal imaging. Data for patients determined to have peripheral bronchial atresia and at least an initial pediatric surgical evaluation were analyzed. RESULTS: Twenty-eight patients with bronchial atresia received at least an initial pediatric surgical evaluation. Expectant management was planned for 22/28 (79%) patients. Two patients transitioned from an expectant management strategy to an operative strategy for recurrent respiratory infections; final pathology revealed bronchial atresia in both. Six patients were initially managed operatively; final pathology revealed bronchial atresia (n = 3) or congenital lobar overinflation (n = 3). CONCLUSIONS: Peripheral bronchial atresia can be safely managed expectantly. A change in symptoms is suspicious for alternate lung pathology, warranting further workup and consideration for resection. LEVEL OF EVIDENCE: Level IV.

Current status of subspecialization in pediatric surgery: A focus on fetal surgery.

BACKGROUND/PURPOSE: Fetal surgery is a growing field within pediatric surgery. We sought to understand practice patterns of pediatric surgeons who subspecialize in fetal surgery. METHODS: A survey was sent to all active non-trainee surgeons within the American Pediatric Surgical Association. Respondents were stratified based on self-reported fetal affiliations. RESULTS: Of 1015 surveys, we received 405 responses (40%). Fetal surgery practices were described by 77 self-designated fetal center members. Centers provide prenatal consultation (99%), diagnostic imaging (84%), care/delivery coordination (83%), and/or fetal surgery/procedures (52%). The majority (56%) of fetal programs are directed by surgery and maternal fetal medicine. Pediatric surgeons are represented on the fetal team in 96% of centers. Prenatal consultations are primarily seen by any pediatric surgeon in the group (53%), with the pediatric surgeon on call operating on/caring for the baby postnatally in the majority (64%), regardless of who performed the prenatal consultation. Only 29% of fetal center members performed a fetal operation in the last year. Yearly fetal case numbers vary widely per procedure, with the most common being complex twin procedures and needle-based sclerotherapy. CONCLUSIONS: Fetal centers vary by services offered, team composition, and interventions performed, with few surgeons performing a wide range of fetal surgery. LEVEL OF EVIDENCE: Level III.

Surgical Management of Wilms Tumor (Nephroblastoma) and Renal Cell Carcinoma in Children and Young Adults.

This article reviews the epidemiology, pathophysiology, clinical presentation, and multimodality management of Wilms tumors and renal cell carcinoma in pediatric and young adults. Key renal Société Internationale d'Oncologie Pédiatrique and Children Oncology Group studies are presented. The article reviews the common staging systems and risk-adapted treatment strategies with particular attention to the surgical management.