Serial Transverse Enteroplasty (STEP) for Short Bowel Syndrome (SBS) in Children: A Multicenter Study on Long-term Outcomes.

OBJECTIVES: Short Bowel Syndrome (SBS), secondary to various underlying diseases, is one of the main causes of intestinal failure in children. Surgical management by serial transverse enteroplasty (STEP), is feasible in selected cases, but results on long-term follow-up are scarce. The aim of this study was to report long-term outcomes of the STEP procedure in children with SBS. METHODS: We performed a multicenter national retrospective study reviewing medical charts of children who underwent a STEP technique between 2000 and 2022 in 6 university hospitals. Collected data included demographics, SBS history, surgical procedures, nutritional support (enteral or parenteral), STEP management, complications, and outcomes (nutritional support, digestive symptoms, growth). RESULTS: STEP was performed in 36 SBS, resulting from 14 gastroschisis, 10 intestinal atresia, 8 necrotizing enterocolitis (NEC), 2 midgut volvulus, and 2 intestinal Hirschsprung disease. Median age at first STEP was 10.8 months [4.5; 63.8]. Bowel length significantly increased (47 vs 70 cm, p < 0.01), with a median gain of 16 cm [11; 25] or 34%. At surgery, 33 children had parenteral nutrition (PN). A second STEP was performed in 11 patients, at a median age of 4.2 years [2.8; 6.8]. One patient underwent a third STEP. Two children required intestinal transplantation. Median follow-up was 7 years [4.4;11.4]. Out of 33 children requiring PN before surgery, 14 children were weaned off PN. The remaining 19 children still required PN, but their dependency decreased by 19%. Out of 17 children presenting severe intestinal dilations and bacterial overgrowth, 12 became asymptomatic. Out of 20 with chronic obstructive symptoms, 8 patients still required supplementary PN whereas 12 achieved complete enteral autonomy, including 3 requiring supplementary enteral support. CONCLUSION: STEP technique remains a surgical option in the management of these children, enabling a decrease in PN dependency, resulting in weaning off PN in some cases, as well as an improvement of clinical symptoms. TYPE OF STUDY: Retrospective multicenter observational study. LEVEL OF EVIDENCE: Level 3.

Assessment of Anorectal Function and Related Quality of Life of 27 Patients with Bladder Exstrophy or Epispadias After Kelly Radical Soft Tissue Mobilisation.

INTRODUCTION: The Radical soft-tissue mobilisation (RSTM) described by J.H. Kelly for bladder exstrophy repair implies a detachment of levator ani muscle insertions from the pelvic wall. The aim of this controlled study was to evaluate the impact of this procedure on subsequent anorectal function. METHODS: Monocentric controlled study of prospectively collected data of children who underwent RSTM for BEEC from 2010 to 2017. Patients born after 2017 were not included, as they were below the theoretical age of continence acquisition at the time of the study. Anorectal function was assessed using the Childhood Bladder and Bowel Dysfunction Questionnaire, and quality of life (QoL) related to fecal continence using the CINCY FIS questionnaire. The control group was paired on age and sex with a 1:3 patient/control ratio. Answers to questionnaires were collected from September 2021 to January 2022. Univariate statistical analysis comparing two groups and subgroup analysis following age were also performed. RESULTS: During the period of study, 55 children with BEEC underwent Kelly RSTM. Twenty-seven (49%) were included and paired with 81 healthy children on age and sex. Median age at surgery was 15 months [0.5-93] and median follow-up was 10 years [4-13]. Patient's group median age at evaluation was 11 years [5-19]. There was no difference between patients and control group in anorectal function for both incontinence and constipation items. No significant difference was found in QoL related to fecal incontinence assessment. Subgroup analysis did not show difference. CONCLUSION: This study suggests that the levator ani detachment during Kelly procedure, realised in a paediatric population under the age of 8, did not impact anorectal function with a mid-term follow-up. LEVEL OF EVIDENCE: III.

The Use of Intraoperative Peripheral Extracorporeal Membrane Oxygenation in High-Risk Airways Tumor Removal Procedures in Neonates and Children: A Single-Center Case Series.

Airway surgery involving trachea or main stem bronchi in neonates and children is challenging. The use of extracorporeal support for such unusual indications is poorly described. Here, we report on three patients receiving peripheral extracorporeal membrane oxygenation (ECMO) to maintain adequate ventilation while improving surgical site exposure. Case 1 is a 9-year-old boy diagnosed with proximal left stem bronchus endoluminal tumor; cases 2 and 3 are a neonate and a young infant diagnosed with a subcarinal bronchogenic cyst. Planned ECMO use consisted in peripheral venoarterial cannulation through jugular and carotid access. There was no bleeding complication during and after surgical care. Hemodynamic and respiratory supports were optimized in all cases. Children were successfully weaned off ECMO immediately after surgery. Planned peripheral ECMO cannulation offers optimal conditions for high-risk airway surgery in neonates and children.

Rapidly growing mature retroperitoneal teratomas.

Retroperitoneal mature teratomas are rare benign tumors, most commonly found in neonates and young adults. We report 2 cases of fast-growing retroperitoneal mature teratomas. Both patients were girls operated on before the age of 6 months. In both cases, during the delay between diagnosis and surgery, the tumors practically doubled in size every 10 days. We believe the possibility of rapid growth of these tumors implies that treatment should be conducted as soon as possible.

Inflammatory pseudotumor of the ureter.

Inflammatory myofibroblastic pseudotumors are rare solid tumors found in most soft tissue locations although mainly in the lung. Their etiology is uncertain, and they are generally considered benign although some have a potential for recurrence and dissemination. Recent studies have suggested, however, that some of these tumors are in fact neoplastic processes that harbor chromosomal aberrations similar to those seen in certain lymphomas. The authors report a case of inflammatory pseudotumor of the ureter in a child and discuss recent reports.