Management and outcomes of vestibular anorectal malformations in a low-income country hospital.

Introduction: anorectal malformations (ARM) are among the most common congenital anomalies in pediatric surgery. Early detection and management of vestibular fistulas are crucial for optimal outcomes, capitalizing on the pliability of sphincter muscles and the preservation of somatosensory integration. This study aimed to assess the incidence, clinical presentation, and management outcomes of vestibular fistula ARM in a low-income hospital setting. Methods: a retrospective audit was conducted on female pediatric patients aged up to 12 years treated for vestibular fistula ARM from January 1, 2011, to June 30, 2016. Data were collected from medical records, and patients were categorized into one of three surgical management groups. Clinical assessments, preoperative procedures, and surgical interventions were meticulously documented. Results: among 656 neonates, the incidence of vestibular fistula ARM was 8.2%. Patients presented at various ages, with 69.4% being early presenters. Notably, 11.1% of cases presented after 30 weeks of age. Functional fistula, constipation, and bowel obstruction were common presenting symptoms. Associated anomalies were relatively low. The choice of surgical approach varied, with a predominant 3-stage at 68%. Complication development did not significantly differ between surgical groups (p-value 0.083). Immediate postoperative complications were minimal, but complications at definitive anoplasty varied among the surgical groups. Anal strictures and fistula recurrence were noted. At 12 months post-surgery, anal strictures persisted in 9 participants. Conclusion: this study highlights the challenges and outcomes associated with vestibular fistula ARM in a resource-constrained setting. The 3-stage approach, despite its historical preference, demonstrated suboptimal outcomes. A 2-stage procedure appears to offer a balanced alternative, particularly suitable for low-income healthcare systems. Further research and collaborative efforts are essential to refine the management of vestibular fistula ARM and improve patient outcomes.

Multiple Gastrointestinal Haemangiomatosis: A Report of Two Cases.

ABSTRACT: Gastrointestinal (GI) haemangiomatosis is a very rare cause of GI bleeding in children. Due to this fact, making the diagnosis sometimes is delayed. In this case report, two different patients with multiple GI haemangiomatosis are presented. Of note is an 8 year old male with a previous history of a vascular malformation of the face presented with features of bowel obstruction (intussusception) and a history of recurrent blood in stool. At laparotomy, multiple haemangiomatosis were seen on the small bowel, right colon, liver, diaphragm and on the body of the gallbladder. Surgical management with excision of the enteric lesions and stapled anastomosis was done.

Metastatic Papillary Thyroid Carcinoma in a Paediatric Patient.

Papillary thyroid carcinoma is the most common endocrine cancer in the paediatric population. Although the disease is diagnosed at a later stage, the prognosis is favourable. When these patients present with lymph nodal and/or pulmonary metastases, they may be initially confused for infectious diseases such as tuberculosis. Therefore, thorough clinical assessment including radiology and microbiological and histopathological assessment is important for early and correct diagnosis. We report an 11-year-old female patient who presented with cervical lymphadenopathy and the histopathological assessment confirmed metastatic papillary thyroid carcinoma. Subsequent radiological investigation revealed further metastasis to the lung. This manuscript highlights the difficulties that might be encountered in the initial management of paediatric PTC which present atypically.

Gastroschisis: Bellwether for neonatal surgery capacity in low resource settings?

INTRODUCTION: Economic disadvantage may adversely influence the outcomes of infants with gastroschisis (GS). Gastroschisis International (GiT) is a network of seven paediatric surgical centres, spanning two continents, evaluating GS treatment and outcomes. MATERIAL AND METHODS: A 2-year retrospective review of GS infants at GiT centres. Primary outcome was mortality. Sites were classified into high, middle and low income country (HIC, MIC, and LIC). MIC and LIC were sometimes combined for analysis (LMIC). Disability adjusted life years (DALYs) were calculated and centres with the highest mortality underwent a needs assessment. RESULTS: Mortality was higher in the LICs and LMICs: 100% in Uganda and Cote d'Ivoire, 75% in Nigeria and 60% in Malawi. 29% and 0% mortality was reported in South Africa and the UK, respectively. Septicaemia was the commonest cause of death. Averted and non-avertable DALYs were nil in Uganda and Cote d'Ivoire (no survivors). In the UK (100% survival) averted DALYs (met need) was highest, representing death and disability prevented by surgical intervention. Performance improvement measures were agreed: a prospectively maintained GS register; clarification of the key team members of a GS team and management pathway. CONCLUSIONS: We propose the use of GS as a bellwether condition for assessing institutional capacity to deliver newborn surgical care. Early access to care, efficient multidisciplinary team working, appropriate resuscitation, avoidance of abdominal compartment syndrome, stabilization prior to formal closure and proactive nutritional interventions may reduce GS-associated burden of disease in low resource settings.

Hirschsprung's disease in the neurologically challenged child.

BACKGROUND: The association between Hirschsprung's disease (HSCR) and central nervous system (CNS) anomalies and syndromes is interesting because of similar developmental pathways. In addition to associated syndromes (e.g., Trisomy 21), these include brain anomalies, mental retardation and growth, ear and hearing deformities, eye hypoplasia, and craniofacial abnormalities, suggesting an neurocristopathy. This group of patients present with neurological challenges and have special challenges in management especially in the older child and adolescent. METHODS: We retrospectively investigated 32 patients with significant HSCR-associated neurological challenges out of a local database of 555 HSCRs (6%). Data were analyzed with details of neurological problem, treatment, complications, and mortality. Long-term outcome was assessed clinically. RESULTS: A total of 32 neurologically challenged children were studied. The male/female ratio was 1.7:1 and all ethnic groups were affected. Abnormalities and syndromes included Down (n = 16) and probable Mowat-Wilson (n = 2) syndromes. Other abnormalities included ophthalmic problems (n = 8), CNS and brain abnormalities (n = 8). Mortality (22%) was mostly related to enterocolitis, particularly in Trisomy 21, ophthalmic problems (n = 8), CNS and brain abnormalities (n = 8). Follow-up age range was 1-34 years (8 years, adolescence and beyond). The outcome was variable, concomitant medical problems were common. Several patients failed to achieve satisfactory continence. CONCLUSION: The association of neurological difficulties in patients with HSCR presents many challenges. Management could involve the exploration and evaluation of alternative treatment choices.