Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. MethodsWe did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung's disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. FindingsWe included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung's disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58•0%) were male. Median gestational age at birth was 38 weeks (IQR 36-39) and median bodyweight at presentation was 2•8 kg (2•3-3•3). Mortality among all patients was 37 (39•8%) of 93 in low-income countries, 583 (20•4%) of 2860 in middle-income countries, and 50 (5•6%) of 896 in high-income countries (p<0•0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90•0%] of ten in lowincome countries, 97 [31•9%] of 304 in middle-income countries, and two [1•4%] of 139 in high-income countries; p≤0•0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2•78 [95% CI 1•88-4•11], p<0•0001; middle-income vs high-income countries, 2•11 [1•59-2•79], p<0•0001), sepsis at presentation (1•20 [1•04-1•40], p=0•016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4-5 vs ASA 1-2, 1•82 [1•40-2•35], p<0•0001; ASA 3 vs ASA 1-2, 1•58, [1•30-1•92], p<0•0001]), surgical safety checklist not used (1•39 [1•02-1•90], p=0•035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1•96, [1•4...
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.
Background Pediatric solid tumors require coordinated multidisciplinary specialist care. However, expertise and resources to conduct multidisciplinary tumor board (MDTB) meetings are lacking in low-and middle-income countries (LMICs). We aimed to profile practices and perceptions on MDTBs among pediatric solid tumor units (PSTUs) in Southeast Asian LMIC countries. Methods Using online survey forms, availability of specialty manpower and MDTBs among PSTUs was first determined. From the subset of PSTUs with MDTBs, 1 pediatric surgeon and 1 pediatric oncologist from each center were queried using 5-point Likert scale questions adapted from published questionnaires. Results In 37/46 (80.4%) identified PSTUs, pediatric-trained oncologists, surgeons, radiologists, pathologists, radiation oncologists, nuclear medicine physicians and nurses were available in 94.6%, 91.9%, 54.1%, 40.5%, 29.7%, 13.5% and 81.1% of PSTUs, respectively. Availability of pediatric-trained surgeons, radiologists and pathologists were significantly associated with existence of MDTBs (p=0.037, 0.005, 0.022 respectively). Among 43/48 (89.6%) 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 both groups. Majority agreed MDTBs helped improve accuracy of treatment recommendations and team competence. Complex cases, insufficient radiology and pathology preparation, and need for supplementary investigations, were the top barriers. Conclusions Availability of pediatric-trained subspecialists was a significant prerequisite for pediatric MDTBs. Most PSTUs lacked pediatric-trained pathologists and radiologists. Correspondingly, gaps in radiographic and pathological diagnoses were the commonest limitations. Greater emphasis on holistic multidisciplinary subspecialty development is needed to advance pediatric solid tumor care in Southeast Asia.
ObjectiveTo review biliary complications following liver resection for liver tumors in children and their associated risk factors.MethodsRetrospectively, 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.ResultsEighty-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).ConclusionsBiliary 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.
Tuberous Sclerosis Complex (TSC) is a rare neuro-cutaneous disorder that is associated with the development of benign hamartomas including renal angiomyolipoma (RAML). TSC associated RAML are usually asymptomatic, but it carries a life-threatening bleeding risk. We are sharing a case of a 5-year-old girl who was diagnosed to have TSC with associated subependymal giant cell astrocytoma, cardiac rhabdomyoma and autism. She presented with a history of worsening abdominal distension over 3 weeks duration and clinically noted to be pale with a ballotable left flank mass. Ultrasound and CT scan found to have multiple RAML in both kidneys with a huge mass on the left side. The mass represented a huge RAML (8cm) with aneurysmal formation with suspicion of intratumoral bleeding. The option of conservative management with mammalian target of rapamycin inhibitor followed with partial nephrectomy has been questioned with its life-threatening risk of bleeding and inability to do biopsy to rule out the possibility of renal cell carcinoma. Decision for nephrectomy was then made clearer following a MAG-3 scan which revealed only 11% differential function of the left kidney. She underwent a total left nephrectomy uneventfully and intraoperatively noted to have an enlarging lesion as compared to the previous imaging; 15cm in largest diameter. Histopathological finding was consistent with multifocal angiomyolipoma with intratumoral haematoma. Decision for nephrectomy in TSC-associated RAML need to be justified carefully in view of its risk of losing the contralateral kidney following the disease progression which may end up with life-long renal replacement therapy.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S26
Background: Duodenal atresia is a common congenital anomaly causing neonatal intestinal obstruction. The definitive treatment is to restore the intestinal continuity. Laparotomy via upper transverse abdominal incision has been described by many authors as a standard approach for duodenal atresia repair. Here we report a case of newborn underwent duodenal atresia repair via modified circumumbilical approach with satisfactory postoperative outcome. Case presentation: This is a case of premature baby boy, born at 32-week gestation with a birth weight of 1.5kg. Antenatally he was detected to have double bubble sign which was confirmed upon postnatal abdominal radiograph, suggestive of duodenal atresia. He was noted to have a fleshy lump at the umbilicus presumed to be a patent vitello-intestinal duct from the referring hospital. Subsequently he developed umbilical swelling suspicion of umbilical hernia with incarceration and scheduled for emergency umbilical exploration. A circumumbilical incision was made and found to have an umbilical defect with omental herniation, instead of a patent vitello-intestinal duct. A proximal midline incision was made as an extension to the circumumbilical wound enabling a duodenal kocherization and subsequently had duodeno-jejujonostomy anastomosis for the duodenal atresia repair. Postoperative recovery was uneventful with appealing cosmesis. Conclusion: A modified circumumbilical incision is an attractive alternative approach for a duodenal atresia repair. Apart from its cosmetic advantage, the approach provides adequate exposure for similar outcome with transverse abdominal incision.
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