Functional fecal and urinary outcomes after sacrococcygeal mass resection in pediatric patients.

BACKGROUND: Sacrococcygeal masses (SCM) are uncommon in children. The purpose of this study is to review the functional fecal and urinary outcomes following resection of SCM and to determine the impact of a multidisciplinary clinic (MDC) on these outcomes. METHODS: A retrospective review was performed of patients who underwent SCM resection between 1979 and 2019. Baylor Social Continence Scale (BCS), Vancouver Symptom Score (VSS) and Cleveland constipation score (CSS) surveys were used to assess fecal and urinary continence at time of most recent follow up. Age, tumor characteristics, histopathology, and type of anorectal malformations (ARM), if present, were also recorded. RESULTS: 75 patients were included. 51 (69%) patients were females and 23 (31%) had an associated ARM. The median age at resection was 8.5 months (IQR 0-26.8). 41 (56%) patients were followed in the MDC. 27 (82%) of patients seen in the MDC were clean for stool and 26 (87%) were dry for urine, while only 17 (59%) of patients not seen in the MDC were clean for stool and dry for urine (p<0.05). There was improvement in Baylor, Vancouver and Cleveland scores. CONCLUSIONS: A multidisciplinary approach to the care of patients following SCM resection may improve bowel and bladder outcomes.

Endosome-Mediated Epithelial Remodeling Downstream of Hedgehog-Gli Is Required for Tracheoesophageal Separation.

The trachea and esophagus arise from the separation of a common foregut tube during early fetal development. Mutations in key signaling pathways such as Hedgehog (HH)/Gli can disrupt tracheoesophageal (TE) morphogenesis and cause life-threatening birth defects (TEDs); however, the underlying cellular mechanisms are unknown. Here, we use mouse and Xenopus to define the HH/Gli-dependent processes orchestrating TE morphogenesis. We show that downstream of Gli the Foxf1+ splanchnic mesenchyme promotes medial constriction of the foregut at the boundary between the presumptive Sox2+ esophageal and Nkx2-1+ tracheal epithelium. We identify a unique boundary epithelium co-expressing Sox2 and Nkx2-1 that fuses to form a transient septum. Septum formation and resolution into distinct trachea and esophagus requires endosome-mediated epithelial remodeling involving the small GTPase Rab11 and localized extracellular matrix degradation. These are disrupted in Gli-deficient embryos. This work provides a new mechanistic framework for TE morphogenesis and informs the cellular basis of human TEDs.