The performance of ultrasound and upper gastrointestinal study in diagnosing malrotation in children, with or without volvulus.

BACKGROUND: Rapid diagnosis is crucial for pediatric patients with midgut volvulus and malrotation to prevent serious complications. While the upper gastrointestinal study (UGIS) is the traditional method, the use of ultrasound (US) is gaining prominence. OBJECTIVES: To assess the diagnostic sensitivity and specificity of US compared to UGIS for malrotation and midgut volvulus. METHODS: A cross-sectional study was performed on 68 pediatric patients who underwent US and/or UGIS before surgery for suspected midgut volvulus or malrotation in Kuala Lumpur (PPUKM and HTA), referencing surgical outcomes as the gold standard. RESULTS: US demonstrated a higher specificity (100%) than UGIS (83%) for diagnosing malrotation, with a slightly lower sensitivity (97% vs. 100%). For midgut volvulus, US surpassed UGIS in sensitivity (92.9% vs. 66.7%) while maintaining comparable specificity. The SMA/SMV criteria showed better sensitivity (91.1%) than the D3 assessment (78.9%) on US, though both had high specificity. CONCLUSION: US is equivalent to UGIS for identifying malrotation and is more sensitive for detecting midgut volvulus, supporting its use as a primary diagnostic tool. The study advocates for combined US and UGIS when either yields inconclusive results, optimizing diagnostic precision for these conditions.

Biliary complications post liver resection for pediatric liver tumors.

Objective: To review biliary complications following liver resection for liver tumors in children and their associated risk factors. Methods: Retrospectively, we reviewed children who underwent liver resection for liver tumors from 2010 to 2019. Demographic data, operative details, types of complications, interventions and outcomes were studied. Results: Eighty-six out of 108 liver resections were included in this study. The median age of patients was 1.8 years old, and 55% were male. The majority (95%) were malignant tumors, of which 87% were hepatoblastoma (n=71). The most common procedure performed was extended right hepatectomy (37%, n=32). Twelve (14%) patients had primary biliary complications: nine bile leakages and three biliary obstructions. Six cases of bile leakage were treated non-operatively with drainage only; however, one developed bilothorax. Three bile leakages underwent early operative intervention. Four patients underwent biliary reconstruction. Biliary complications were not significantly associated with age, sex, ethnicity or pathology of the tumor. Ten of them (83%) developed following extended hepatectomies (five right, five left), in which the left side had a higher rate of complications (63% vs 16%). None of the central hepatectomies had biliary complications. Biliary complication rates were significantly higher among those who had segmentectomy 1 (p=0.023). Conclusions: Biliary complication is a significant morbidity following liver resection in children. Surgery is eventually required for complicated bile leakage and primary biliary strictures. Follow-up is mandatory since secondary biliary complications may occur after the initial resolution of bile leakage. The groups at high risk of developing biliary complications are extended left hepatectomies and segmentectomy 1.

Diagnostic dilemma and challenges in management: Hirschsprung's disease, anal stenosis and reduced interstitial cells of Cajal enteric mesenchymopathy.

Hirschsprung's disease (HD) is one of the most well-known gastrointestinal motility disorders. Diagnosis and management of other lesser-known motility disorders are often challenging and tedious. We describe a teenager who was severely constipated from birth and needed intensive care admissions for life-threatening enterocolitis. She also had concomitant anal stenosis. Several rectal biopsies were unable to yield a conclusive diagnosis. Surgical level of resection had to be identified based on the motility of the bowel as determined by transit studies using oral ingestion of a milk feed labelled with Technetium-99m colloid. After completion of all operative stages, histopathological examination of the excised specimens concluded that she had short-segment HD associated with reduced interstitial cells of Cajal in the large bowel. She is currently continent, evacuating voluntarily approximately four times a day and is relieved of all her symptoms.

Gastrointestinal and oncologic outcomes of pediatric gastrointestinal lymphoma following upfront resection or biopsy of bowel masses.

PURPOSE: Complete upfront resection of pediatric gastrointestinal lymphomas is recommended over biopsy whenever feasible, but either approach may have adverse sequelae. We sought to compare gastrointestinal and oncological outcomes of pediatric gastrointestinal lymphomas who underwent attempted upfront resection or biopsy of the presenting bowel mass. METHODS: We retrospectively reviewed charts of children with gastrointestinal lymphomas treated on LMB89 and LMB96 protocols from 2000 to 2019 who underwent upfront gastrointestinal surgery, and compared resection and biopsy groups. RESULTS: Of 33 children with abdominal lymphomas, 20 had upfront gastrointestinal surgery-10 each had resection or biopsy. Patients with attempted upfront resections had fewer postoperative gastrointestinal complications compared to biopsies (10% vs. 60%, p = 0.057), but longer time to chemotherapy initiation (median 11.5 vs. 4.5 days, p < 0.001). Three resection patients were surgically down-staged. Second surgeries were required in 30% and 40% of resected and biopsied patients, respectively, at median 4.6 months. Survival was similar in both groups, but better in patients on LMB96 protocol and stage II/III disease. CONCLUSIONS: Children with upfront attempted resection had low rates of surgical down-staging, greater delay in chemotherapy initiation, but fewer gastrointestinal complications and subsequent surgeries than biopsies. Survival was similar regardless of upfront surgery, likely reflecting beneficial effects of newer protocols.

Pediatric Solid Tumor Care and Multidisciplinary Tumor Boards in Low- and Middle-Income Countries in Southeast Asia.

PURPOSE: Pediatric solid tumors require coordinated multidisciplinary specialist care. However, expertise and resources to conduct multidisciplinary tumor boards (MDTBs) are lacking in low- and middle-income countries (LMICs). We aimed to profile the landscape of pediatric solid tumor care and practices and perceptions on MDTBs among pediatric solid tumor units (PSTUs) in Southeast Asian LMICs. METHODS: Using online surveys, availability of specialty manpower and MDTBs among PSTUs was first determined. From the subset of PSTUs with MDTBs, one pediatric surgeon and one pediatric oncologist from each center were queried using 5-point Likert scale questions adapted from published questionnaires. RESULTS: In 37 (80.4%) of 46 identified PSTUs, availability of pediatric-trained specialists was as follows: oncologists, 94.6%; surgeons, 91.9%; radiologists, 54.1%; pathologists, 40.5%; radiation oncologists, 29.7%; nuclear medicine physicians, 13.5%; and nurses, 81.1%. Availability of pediatric-trained surgeons, radiologists, and pathologists was significantly associated with the existence of MDTBs (P = .037, .005, and .022, respectively). Among 43 (89.6%) of 48 respondents from 24 PSTUs with MDTBs, 90.5% of oncologists reported > 50% oncology-dedicated workload versus 22.7% of surgeons. Views on benefits and barriers did not significantly differ between oncologists and surgeons. The majority agreed that MDTBs helped to improve accuracy of treatment recommendations and team competence. Complex cases, insufficient radiology and pathology preparation, and need for supplementary investigations were the top barriers. CONCLUSION: This first known profile of pediatric solid tumor care in Southeast Asia found that availability of pediatric-trained subspecialists was a significant prerequisite for pediatric MDTBs in this region. Most PSTUs lacked pediatric-trained pathologists and radiologists. Correspondingly, gaps in radiographic and pathologic diagnoses were the most common limitations for MDTBs. Greater emphasis on holistic multidisciplinary subspecialty development is needed to advance pediatric solid tumor care in Southeast Asia.