45 years' experience with early childhood anatomical technique of feminising genitoplasty for 46 XX Congenital Adrenal Hyperplasia -observations of vaginal introital anatomy and its relationship to the perineal body.

In Manchester, feminising genitoplasty is offered to children with 46XX Congenital Adrenal Hyperplasia (CAH) when there is a single perineal opening and/or enlarged clitoris. Our aims are to describe the anatomical reconstructive technique and present long-term outcomes. Our hypothesis is that 'the common channel (CC) length and distance to the vagina from perineal skin is mostly due to virilisation and hypertrophy of perineal tissue over the almost normally positioned vaginal introitus (V-I) in relation to the perineal body (PB)'. METHOD AND RESULTS: This is a retrospective notes review of all consecutive 46XX CAH operations from 1976 to December 2021. 99 patients, who had feminising genitoplasty and being followed-up, were included. 15 patients who were lost to follow up were excluded. Median age at surgery was 15 months. In 91, midline division of the labia majora, spongiosum, bulbo-spongiosus muscle (BSM) and CC down to PB was performed. This was sufficient to expose the V-I at the same level or within 5 mm depth of PB in 88. In 78 V-I was adequate taking 10/12fr dilator (Type 1). In 10, CC resembled a male urethra and V-I was narrow (Type 2), requiring widening by 5-10 mm incision at 6 o'clock position. Dartos of labia majora was attached to BSM to reduce the distance to V-I from perineal skin and the gap was lined with inner foreskin to create a vestibule. Out of 70 who were post-pubertal, 75% (53/70) had adequate calibre vaginal openings. 5 had introitoplasty and 2 had dilatation under anaesthesia. 10 needed self dilators only. 29 patients, of one of the three surgeons, had measurements of clitoris, CC, urethra and vagina. A hymen was found in 86% (25/29). There was significant strong, inverse correlation between the CC length and the urethral length (r = -0.708, p < 0.001, n = 27) but not between CC and vaginal lengths. After adjusting for age, the urethral length of Type 2 patients was 3.825 mm shorter than those of Type 1 (p = 0.017). CONCLUSION: Our data show that 'high' confluence is mostly due to virilisation of genitalia; and the anatomical technique of reversing the fusion of the urethral folds, spongiosum and bulbo-spongiosus muscle could be performed with all degrees of virilisation with success in early childhood with no need of local flaps or mobilisation of the urethro-vaginal complex. About 10% require surgery to treat narrowing of vaginal opening post puberty.

Spectrum of paediatric surgical cases in a private mission teaching hospital in Nigeria.

INTRODUCTION: Establishing the nature of conditions requiring surgery among children in a particular location may be crucial for policy formulation and implementation as regards paediatric surgery. OBJECTIVE: This study aimed to describe the pattern and outcome of paediatric surgical cases operated upon in a newly established paediatric surgical unit in Nigeria. SUBJECTS AND METHODS: This was a cross-sectional study of all subjects that were operated upon by the paediatric surgery unit over a 28-month period. Data obtained included age, sex, diagnosis, timing of surgery, post-treatment complications and outcome. Diagnoses were categorised based on the International Classification of Diseases 11th revision for morbidity and mortality statistics. Data analysis was done using Stata version 12. RESULTS: A total of 377 procedures were performed on 336 patients with a male-to-female ratio of 2.1:1. The median age at surgery was 36 months. Disorders of the digestive system (184, 48.8%) and developmental anomalies (119, 31.6%) accounted for majority of the cases, with inguinal hernias and hydrocoeles accounting for 17.0% of all cases. Thirty-six per cent of the procedures were emergent ones, and the overall complication rate was 23.6% (89/377). The unplanned re-operation rate was 7.4% (25/336) and mortality rate was 5.1% (17/336). Typhoid ileal perforation was responsible for 4 (23.5%) of the deaths. CONCLUSION: Congenital anomalies and surgical infections represent a major surgical burden among children in our sub-region of Nigeria. There is, therefore, the need for focused research on these conditions and the integration of children surgery into public health programmes for children in sub-Saharan Africa.

Changing trend in the management of omphalocoele in a tertiary hospital of a middle-income country.

BACKGROUND: The peri-operative management of omphalocoele in low- and middle-income countries is challenging owing to non-availability of neonatal intensive care units and equipment needed for the care of this anomaly. AIM: This study examined our experience in the management of omphalocoele and compared the pattern and outcome with a similar study from the same centre conducted four decades ago. METHODS: A retrospective study of neonates managed for omphalocoele from 2003 to 2017 (Group A) was performed. Their demographic characteristics, clinical presentation, management modality and outcome were obtained and statistical analysis was performed to determine the predictors of mortality. The findings were also compared with the findings of a similar study (Group B) published from this centre four decades ago from 1973 to 1978. RESULTS: A total of 95 patients were managed in Group A and 33 in Group B. Their ages ranged from 1 to 15 days with a median age of 1 day and a median gestational age at birth of 37 weeks (range - 36-43 weeks) in Group A and 5-72 h in Group B. There were 54 (56.8%) boys and 41 (43.2%) girls in Group A and 17 (51.5%) boys and 16 (48.5%) girls in Group B. Rupture of the sac was observed in 18 (18.9%) patients in Group A and 13 (39.4%) in Group B. Operative management was adopted for 55 (57.9%) patients in Group A compared to 14 (42.4%) in Group B. Mortality was recorded in 16 (16.8%) patients in Group A and 16 (48.5%) in Group B. Following further analysis in Group A, management outcome was noted to be significantly associated with the state of the sac (P = 0.011), presence of associated sepsis (P = 0.002) at presentation and management modality (P = 0.048) with only associated sepsis independently predicting mortality. CONCLUSION: Although epidemiological trend and clinical presentation are still similar, management outcome has improved over the years.

Trends in the publication of infantile hypertrophic pyloric stenosis in Africa: a systematic review

There are still global variations in the epidemiology of infantile hypertrophic pyloric stenosis, although the clinical presentations may be similar. Outcome of management, however, may depend on the degree of evolution of management of the anomaly. This review aimed at evaluating the trends of reporting of infantile hypertrophic pyloric stenosis from Africa. An evaluation of all publications from Africa on infantile hypertrophic pyloric stenosis focusing on epidemiology, evolution of management of the anomaly was carried out. Literature search of all publications from Africa on Infantile hypertrophic pyloric stenosis was conducted from January 1, 1951, to December 31, 2018. The articles were sourced from the databases of African Index Medicus, OvidSP, PubMed, African Journal Online, and Google Scholar. Extracted from these publications were information on the type of article, trend of reporting, the country of publication, demographic details of the patients, number of cases, clinical presentation, pre-operative management, type of surgical approach, and the outcome of management. Overall, 40 articles were published from 11 countries. Of these, 16 (40.0%) were published in the first 35 years (Group A, 1951­1985) and 24 (60.0%) published in the later 33 years (Group B, 1986­ 2018). Case reports 8 (20.0%) and case series 5 (12.5%) were predominant in Group A, whereas retrospective studies 12 (30.0%) predominated in Group B. The countries of publication included Nigeria (27.5%), South Africa (15.0%), Egypt (12.5%), Tanzania (10.0%), and Zimbabwe (10.0%). A total of 811 patients diagnosed and managed for infantile hypertrophic pyloric stenosis (IHPS) were reported. Their ages ranged from 1 day to 1 year with an incidence that ranged from 1 in 550 to 12.9 in 1000. There were 621 boys and 114 girls (M:F ­ 5.5:1). All the patients were breastfed with an average birth rank incidence of 42.4% among firstborns, 19.5% in second borns, 15.2% in third borns, 13.2% among fourth borns, and 10.0% among fifth borns and beyond. Associated congenital anomalies were reported in 5 (12.5%) studies with an incidence of 6.9­20% occurring in a total of 28 patients. All but 3 (7.5%) studies reported that open surgery was adopted to perform Ramstedt's pyloromyotomy on the patients. Reported post-operative complications include mucosal perforation in 8 (20.0%) studies, surgical site infection in 7 (17.5%), gastroduodenal tear 2 (5.0%), and hemorrhage and incisional hernia in 1 (2.5%) study each. Mortality was reported in 26 (65.0%) studies with a range of 1.8­50% and a mean mortality rate of 5.2%. There has been a change in the trend of reporting IHPS in Africa over the years, with increasing comparative studies on the modalities of management compared to case reports and series. Still very limited work has been done in the aspect of genetics and etiology of IHPS among Africans. There is a need to increase funding in this regard and to encourage multi-center collaborations in the study of this relatively rare condition

Congenital infantile digital fibromatosis: a case report and review of the literature.

Infantile digital fibromatosis (IDF), also called inclusion body fibromatosis is an uncommon benign tumour occurring in the digits of young children. In about a third of cases, it is congenital and the diagnosis is based on the presence of peculiar intracytoplasmic inclusions on histology. Recurrence rate post-surgery is high. However, spontaneous regression has been reported. We present a case of a 5-month-old infant who had excision of a right second toe mass, which has been present from birth. Histological examination revealed this to be infantile digital fibromatosis. To the best of our knowledge, no report of this has been made in Nigeria. It is important that this diagnosis be entertained in young children with masses on the digits as this will influence the management instituted.

Clinical predictors and outcome of bowel resection in paediatric intussusception.

INTRODUCTION: Surgery remains the mainstay in treating intussusception in developing countries, with a correspondingly high bowel resection rate despite a shift to non-operative reduction in high-income countries. OBJECTIVE: To assess factors associated with bowel resection and the outcomes of resection in childhood intussusception. METHODS: A review of children with intussusception between January 2006 and December 2015 at the University College Hospital, Ibadan, Nigeria. The patients were categorized based on the need for bowel resection and analysis done using the SPSS version 23. RESULTS: 121 children were managed for intussusception during this period. 53 (43.8%) had bowel resection, 61 (50.4%) did not require resection and 7 (5.8%) were unknown. 40 (75.5%) of the resections were right hemi-colectomy. The presence of fever, abdominal pain, distension, rectal mass, age < 12 months, heart rate > 145/min and duration of symptoms > 2 days were associated with the need for bowel resection (p < 0.05). However, only age and abdominal pain independently predicted need for resection. Bowel resection was more associated with development of post-operative complications and prolonged hospital stay (p < 0.05). CONCLUSION: Infants presenting with abdominal pain and abdominal distension after two days of onset of symptoms were more likely to require bowel resection. Resection in intussusception significantly increased post-operative complications and length of hospital stay.

Outcome of Management of Neonatal Intestinal Obstruction at a Tertiary Center in Nigeria.

BACKGROUND: Intestinal obstruction in a newborn remains a significant emergency in pediatric surgery. Clinical presentation is often subtle with sudden deterioration of their clinical states. Clinical outcome in the developing countries is poor owing to a variety of factors. OBJECTIVE: The objective of this study is to identify the factors affecting the management outcome in our environment. PATIENTS AND METHODS: Data on clinical presentation, management, and outcome of all neonates managed for intestinal obstruction over a 13-year period at a tertiary center in Nigeria were retrospectively reviewed. Analysis of factors affecting the management outcome was also performed. RESULTS: One hundred and seventeen neonates comprising 85 (72.7%) boys and 32 (27.3%) girls were managed for intestinal obstruction. The age at presentation ranged from 0 to 29 days, with a mean of 6.86 ± 8.4 days. Seventy-five (64.1%) patients presented within a week of onset of symptoms and 42 (35.9%) patients later. Eighty-five patients (72.6%) presented with symptoms from birth. The most common causes of intestinal obstruction included anorectal malformation in 62 (53%) neonates and Hirschsprung's disease in 16 (13.7%) neonates. Other causes included obstructed inguinoscrotal hernias, duodenal atresia, jejunoileal atresia, malrotation, and annular pancreas. Eleven patients died with a mortality rate of 9.4%. The age at presentation (P = 0.001) and the presence of postoperative complications (P = 0.009) were significantly related to the duration of hospital stay. Furthermore, the presence of postoperative complications (P = 0.012) was significantly associated with postoperative mortality. CONCLUSION: Early presentation and postoperative complications significantly affected the morbidity and mortality associated with the management of neonates with intestinal obstruction.

Prevalence, risk factors and outcome of congenital anomalies among neonatal admissions in OGBOMOSO, Nigeria.

BACKGROUND: Congenital anomalies (CA) are a major cause of neonatal morbidity and mortality, especially in developing countries. Data on these anomalies are still poorly collated in developing countries. We aimed to assess the prevalence, pattern, risk factors and outcome of congenital anomalies among neonatal admissions in Ogbomoso Town, Nigeria. METHODS: A cross-sectional retrospective study in which a review of the records of all neonates admitted in the neonatal unit of the Bowen University Teaching Hospital, Ogbomoso over a five-year period (January 2012-December 2016) was undertaken. The occurrence rate and pattern of anomalies were determined, while factors associated with the occurrence and outcome of anomalies were calculated with the odds ratio and 95% confidence interval. Data entry and analysis were performed using SPSS version 21. RESULTS: CA were found in 67 of the 1057 neonatal admissions, giving a prevalence rate of 6.3%. Anomalies of the cardiovascular and digestive systems were the most common. A higher proportion of babies referred from other facilities had CA, and this was found statistically significant. There was no significant association between CA and low birth weight, sex, maternal age or parity. The mortality rate among neonates with CA was 10.4%. Although, CA was associated with reduced risk of neonatal mortality compared to those with other acute conditions, this was not statistically significantly. CONCLUSION: CA is a major indication for neonatal admissions in Ogbomoso. There is the need to establish a surveillance system for CA and efforts should be made to raise awareness of the occurrence and risk factors of CA in developing countries.