Scrotoschisis: Management Challenges and Review of Literature.

ABSTRACT: Scrotoschisis (extracorporeal testicular ectopia) is a rare congenital defect of the scrotal sac associated with the extrusion of one or both testicles. The exact mechanism causing the anomaly is largely unknown. This is a report of two infants aged 3 and 4 days, respectively, presented with infected unilateral extracorporeal testicular ectopia. Both infants had orchidopexy and repair of the scrotal defect following debridement of the infected defects and administration of broad-spectrum parenteral antibiotics. The infants have remained well at 3 years of follow-up. The isolated unilateral disease was described by several authors. Delayed presentation in scrotoschisis results in superimposed infection which complicates surgical management resulting in a prolonged hospital stay.

Outcome of Transanal Endorectal Pull-Through in Patients with Hirschsprung's Disease.

BACKGROUND: Transanal endorectal pull-through (TERPT) has become one of the preferred treatments for Hirschsprung's disease (HD) in our setting. This report aims to evaluate the current outcome of TERPT in the setting. MATERIALS AND METHODS: A retrospective review of 71 children who had TERPT for histologically-confirmed HD in 11 years (2006-2017) in Nigeria. RESULTS: There were 48 boys and 23 girls; aged 3 days-12 years at initial presentation (median = 10 months). Three (4.2%) patients had associated anomalies (duodenal atresia; anorectal malformation and sensorineural deafness with hypopigmented skin patches each). Age at TERPT was 2 months to 15 years (median = 3 years), with surgery waiting time of 1 month-14.9 years (median = 18 months). Sixty-six (93.0%) patients had rectosigmoid, four (5.6%) patients had long segment and one (1.4%) had total colonic disease. Five (7.0%) patients with large megacolon and one (1.4%) with the total colonic disease had assisted abdominal resection of the colon at TERPT. Seventeen (23.9%) patients had post-operative complications, including post-operative enterocolitis 8 (11.3%); anastomotic dehiscence 3 (4.2%); retained aganglionic segment 2 (2.8%); anastomotic stenosis 2 (2.8%), resulting in prolonged hospital stay (P = 0.0001; range = 1-30 days; median = 5 days). The mortality rate was 4.2% (3) from malignant hyperthermia in one patient and in 2 patients, the cause of mortality was unclear. Patients were followed up for 3-6 years (median = 3.5 years). Bowel movement stabilised to 2-4 times daily by 6 weeks after surgery. CONCLUSION: TERPT is a safe treatment for HD in this setting with good short-term outcomes. Longer follow-up is necessary to further evaluate the long-term bowel movement outcomes.!

A Modified Rectal Mucosal Biopsy for the Diagnosis of Hirschsprung's Disease: Zaria Experience.

Background: Full-thickness rectal biopsy is often used for the diagnosis of Hirschsprung's disease (where a suction biopsy kit is not available). This is associated with some challenges such as limited theatre space and the need for general anaesthesia. We aim to highlight the usefulness and sensitivity of a modification of the rectal mucosal biopsy without general anaesthesia in the diagnosis of Hirschsprung's disease. Materials and Methods: This is a retrospective analysis of children with Hirschsprung's disease who had rectal mucosal biopsy over a 16-year period (January 2004-December 2019). Research was approved with institutional number ABUTHZ/HREC/H22/2022. The patients had the biopsy with small, curved artery forceps, surgical blade, and dissecting scissors. Histological analyses of tissue were done. Clinical data and results were recorded on a structured pro forma, and the data were analysed. Results: There were 263 boys and 97 girls with a median age of 10.5 months. Only 37 (10.3%) of the rectal biopsies were done by consultants. Hirschsprung's disease was confirmed in 279 (75.5%) of the partial-thickness biopsies, whereas 52 (14.4%) biopsies were inadequate specimens. Resident doctors were responsible for 92.2% (47) of inadequate biopsies (P = 0.63), although they did 89.7% of all biopsies. In one (0.3%) patient, the procedure ended as a full-thickness biopsy leading to a significant haemorrhage that required blood transfusion. Conclusions: The modified rectal mucosal biopsy is a simple, safe, and effective method for making the diagnosis of Hirschsprung's disease. This is performed without general anaesthesia and is useful where a suction biopsy kit is unavailable.

Duodenal obstruction due to missed pre-duodenal portal vein in a patient with intestinal malrotation.

Pre-duodenal portal vein (PDPV) is a rare anomaly and a rare cause of duodenal obstruction (DO), with only a few cases reported in the literature. We present an infant whose bilious vomiting persisted despite having Ladd's procedure for intestinal malrotation due to a missed diagnosis of DO from PDPV that was found at re-exploration. The patient was diagnosed with malrotation and had Ladd's procedure at 12 weeks of age, but bilious vomiting persisted post-operatively. The patient presented to us after 4 weeks, was clinically malnourished and dehydrated, resuscitation was done and re-exploratory laparotomy performed, where an obstructing PDPV was found and a duodeno-duodenostomy was performed anterior to PDPV. However, the patient died on post-operative day 7 probably from severe malnutrition due to delayed diagnosis and absence of parenteral nutrition. We conclude that PDPV may be a cause of DO in infants with malrotation and should be properly sought for during Ladd's procedure for possible bypass surgery if found.

Accuracy of ultrasonography in the evaluation of abdominal masses in children in Nigeria.

INTRODUCTION: Abdominal masses in children constitute a spectrum of lesions of diverse origin, nature and significance. In a low-income setting with limited investigative facilities, accurate diagnosis of abdominal masses can be very challenging. However, ultrasound has been used preferentially as a diagnostic tool in evaluating abdominal masses in children. This study aimed to determine the accuracy of ultrasound evaluation of abdominal masses in children. MATERIALS AND METHODS: This was a prospective descriptive study of 135 consecutive children presenting with palpable and/or suspected abdominal masses over a 1-year period at a tertiary hospital. Data analyses were done using SPSS version 17 software and level of significance was set at P < 0.05. RESULTS: Abdominal mass was located in the kidney in 40 (32.3%) patients. Definitive diagnoses of abdominal masses were confirmed intraoperatively in 33 (24.4%) and by histopathological examination in 102 (75.6%) cases. Abdominal masses were detected in 124/135 (91.9%) of the participants. Ultrasound (US) correctly identified 109/124 (87.9%) of the pathologies. The sensitivity and specificity of USS were 87.9% and 81.8%, respectively. Positive predictive value (PPV) and negative predictive value (NPV) were 97.2%-98.2% and 37.5%-45.7%, respectively. The overall US diagnostic accuracy was 87.4%. The predictive value of US for intussusceptions, Wilms' tumour and hydronephrosis was high (sensitivity was 92.0%, 95.8% and 81.1% and PPV was 90.0%, 83.0% and 93.8%, respectively). On the other hand, the sensitivity indices for appendiceal mass/abscess, lymphoma and neuroblastoma were low (28.6%, 66.7% and 57.1%, respectively), while their PPV was low too (66.7%, 42.9% and 77.7%, respectively). The PPV was especially poor for lymphoma. The specificity and NPV indices for these six disease conditions were good. CONCLUSION: Ultrasound is a reliable tool in the evaluation of abdominal masses in children in this setting.

Presentation and outcome of treatment of jejunoileal atresia in Nigeria.

BACKGROUND: Intestinal atresia is a common cause of neonatal intestinal obstruction. Previous reports from Nigeria have indicated a high mortality rate. This is a report of current outcome review from one tertiary center. PATIENTS AND METHODS: A retrospective analysis of infants managed for jejunoileal atresia in 10 years (2005-2014). The information retrieved from patients' records was analyzed using SPSS 17. RESULTS: There were 38 patients (19 boys and 19 girls) aged 1-28 days (median 4 days). Twenty-four patients (63.2%) presented after 48 h of life. Twenty-five (65.8%) had jejunal atresia and 13 (34.2%) had ileal atresia. Six patients had associated anomalies. The most common atresia was type III (39.5%, 15 patients). Twenty-eight (73.7%) patients had a resection of the atresia and anastomosis and others had enterostomies. Total parenteral nutrition and neonatal intensive care support were not available during the period of the study. Bowel function was established within 1 week and 27 (71.1%) patients commenced oral feeding. Twenty-six (68.4%) patients had postoperative complications resulting in prolonged hospital stay of 2-44 days (median = 13). Mortality was 34.2% (13 patients). Factors that significantly affected mortality were intestinal necrosis at presentation, postoperative complications, and severe malnutrition. CONCLUSION: Intestinal atresia is still associated with unacceptably high morbidity and mortality, due to late presentation, and lack neonatal intensive care services and parenteral nutritional support. Efforts need to be intensified to address these factors to improve outcome.