Experience with full-thickness rectal biopsy in the evaluation of patients with suspected Hirschsprung’s disease

Background and purpose Rectal biopsy is the main modality for the diagnosis of Hirschsprung’s disease (HD). In Africa, transanal full-thickness rectal biopsy is commonly performed. We aimed to audit our practice of rectal biopsy in the evaluation of HD.Materials and methods A retrospective review was carried out of the records of children (r15 years) who were evaluated for HD between 2007 and 2011. Clinical presentation, details of the operation, and histologic result were analyzed using SPSS version 15.0.Results Fifty-seven children were evaluated for suspected HD during the period. Thirty-six children underwent a rectal biopsy. There were 29 (80.6%) males and nine (19.4%) females, of which two were preterm. Neonates and infants accounted for 72.2% (n= 26). The median age at biopsy was 90 days (range, 5 days to 9 years). Delayed passage of meconium was present in 64.7%, constipation in 85.7%, abdominal distension in 88.6%, and bilious vomiting in 55.9%. Thirty biopsies (83.3%) yielded a histologic diagnosis. Twenty-six (72.2%) confirmed HD, whereas four (11.1%) yielded normal rectal histology. In six (16.7%), the sample taken was deemed inadequate for opinion. None of the symptoms assessed was associated significantly with a diagnosis of HD, stalling further analysis. Where a single biopsy was taken, 20% (n =5) were inadequate for analysis; where more than one sample was taken, a histologic diagnosis was possible in 100% (n =11). Consultant surgeons and trainees returned inadequate samples in 15.8% (n= 3) and 12.5% (n= 2), respectively. An inadequate sample was obtained in four infants (15.4%) and one child older than 1 year of age (10%). Distance of biopsy from the dentate was not indicated in 63.9% (n= 23).Conclusion No clinical parameter can accurately predict a diagnosis of HD. More than one sample at a sitting may improve the diagnostic yield. Larger prospective studies are needed to confirm these findings.Keywords: full-thickness biopsy, Hirschsprung’s disease, rectal biops

Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary 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. Methods We 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. Findings We 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, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Is non-operative management still justified in the treatment of adhesive small bowel obstruction in children?

Background: Adhesive small bowel obstruction (ASBO) is a feared complication after abdominal operations in both children and adults. The optimal management of ASBO in the pediatric population is debated. The aim of the present study was to examine the safety and effectiveness of non-operative management in ASBO. Patients and Methods: A retrospective review of 33 patients who were admitted for ASBO over a 5-year period was carried out. Follow-up data were available for 29 patients. Demographic, clinical, and operative details and outcomes were collected for these patients. Data analysis was done with SPSS version 15.0. P ≤ 0.05 was regarded as significant. Results: Out of 618 abdominal surgeries within the 5-year period, 34 admissions were recorded from 29 patients at the follow-up period of 1-28 months. There were 19 boys (65.5%). The median age of patients was 4.5 years. Typhoid intestinal perforation (n = 7), intussusception (n = 6), intestinal malrotation (n = 5), and appendicitis (n = 4) were the major indications for a prior abdominal surgery leading to ASBO. Twenty-five patients (73.5%) developed SBO due to adhesions within the first year of the primary procedure. Of the 34 patients admitted with ASBO, 18 (53%) underwent operative intervention and 16 (47%) were successfully managed non-operatively. There were no differences in sex (P = 0.24), initial procedure (P = 0.12), age, duration of symptoms, and time to re-admission between the patients who responded to non-operative management and those who underwent operative intervention. However, the length of hospital stay was significantly shorter in the non-operative group (P < 0.0001). Five (14.7%) patients had small bowel resection. A 43-day-old child who initially underwent Ladd′s procedure died within 15 h of re-admission while being prepared for surgery, accounting for the only mortality (3.4%). Conclusion: Non-operative management is still a safe and preferred approach in selected patients with ASBO. However, 53% eventually required surgery

Pediatric laparoscopic surgery in North-Central Nigeria: Achievements and challenges

Background and Objective: Advances in laparoscopy are making the service accessible even in resource-poor countries where adaptations are made to meet local challenges. We report our experience in the provision of laparoscopy service to children at a tertiary health center in North-central Nigeria. Methods: A team of pediatric surgeons, anesthetists, and nurses collaborated to provide service and train other personnel. A prospective collection of data on biodata, diagnoses, procedure, and outcome over an effective period of 36 months of laparoscopy intervention of the 54 months between September 2009 and February 2014 was done. Consent, which also included the possibility of conversion to open was obtained from the parents of the patients. Results: A total of 73 patients aged 2 weeks to 16 years with a male: female ratio of 3 to 1 had laparoscopy done during the period. Fifty-two (71.2%) procedures were therapeutic, and 21 (28.8%) cases were done as emergency. Laparoscopic appendectomy was the most commonly performed procedure 25 (34.3%), followed by laparoscopic orchidopexy 17 (23. 3%), and diagnostic laparoscopy for disorders of sexual differentiation in 13 (17.8%). The length of stay in hospital postoperative was 1-3 days with a mean of 1.34 ΁ 0.45 days. The complications recorded included hemorrhage, in a case of infantile hypertrophic pyloric stenosis due to failed electrocautery, one port site burns injury from diathermy dissection, and two periport pain postoperation. There was no mortality recorded. Conclusion: Pediatric laparoscopic service is gaining recognition in our practice in spite of poor resources, incessant industrial actions, and apathy from support staff. The outcomes are encouraging as the patients had minimal morbidities. Skills are improved through practice and retraining and manpower, and instruments are being expanded through our collaboration and training

Oral fetus-in-fetu: A case report

Fetus-in-fetu is a rare condition, less than 200 cases have been reported. Its embryopathogenesis is linked to a monozygotic, diamniotic parasitic twin. The presence of a calcified vertebral column and other body parts are key to the diagnosis, and differentiate it from a teratoma. We report a case of a neonate who was admitted immediately after delivery by Caesarian section following a prolonged obstructed labor caused by a huge mass projecting from the hard palate. The mass had identifiable malformed body parts but was anencephalic. Intraoperative findings were a short stalk and cleft of the soft palate. He had excision of the mass and did well post operatively. Persistent mouth breathing and difficult nasal cannulation lead to request for post operative magnetic resonance imaging which showed patent nostrils and absent residual mass. Prompt and skillful anesthesia and surgical intervention assisted in the survival of this patient