Analysis of Presentations and Outcomes of Care of Children with Disorders of Sexual Development in a Nigerian Hospital.

STUDY OBJECTIVE: To describe the presentation, diagnosis, management, and short-term outcome of children with disorders of sexual development (DSD) in the context of multidisciplinary team care. DESIGN: Prospective descriptive study. SETTING: University Teaching Hospital. PARTICIPANTS: All children who presented with genital ambiguity. INTERVENTIONS AND MAIN OUTCOME MEASURES: Records of all patients diagnosed and managed for DSD between January 2011 and December 2016 were reviewed. The care pathway included clinical, laboratory, internal genitalia evaluation, and panel (including parents) meeting. RESULTS: Fifteen children presented with DSD at a median age of 20 months. Only 5/15 (33.3%) presented in the neonatal period. Ten of fifteen patients (66.7%) presented with genital ambiguity. Ovotesticular DSD was the most common diagnosis (9/15; 60%). Seven of the patients were genetically female (46, XX), 1 was genetically male (46, XY) and 1 without genetic diagnosis. Six patients were assigned male gender and they underwent male genitoplasty. Five of them had excision of Müllerian structures with gonadectomy. Three of fifteen patients (20%) were diagnosed as 46, XX DSD, at a median age of 7 years. All of them were due to congenital adrenal hyperplasia and underwent female genitoplasty. Two patients were diagnosed as XY, DSD. They were both raised as female at presentation and were reassigned male sex. Both had urethroplasty done. Four patients had postoperative urethrocutaneous fistula and 1 had partial wound dehiscence. The median follow-up period was 21 months (interquartile range, 2-26 months). CONCLUSION: The frequency of ovotesticular DSD is high in our setting. The decision of sex assignment was finally made at a median age of 7.5 months in most of our patients with satisfactory short-term surgical outcome.

Short-Term Outcomes of Treatment of Boys with Posterior Urethral Valves.

BACKGROUND: Posterior urethral valve (PUV) is a significant cause of morbidity and mortality among male children resulting in renal failure in 25%-30% before adolescence irrespective of initial treatment. This study aimed at evaluating the early outcomes of children managed for PUV. MATERIALS AND METHODS: This was a prospective study of all children who were treated for PUV between 2012 and 2016 at a single referral institution. Information reviewed included demographic and clinical data, imaging findings, pre- and post-operative serum electrolytes, and postoperative renal outcomes. RESULTS: Twenty-nine male children were managed for PUV at a median age of 6 months including 7 (24.1%) neonates. Two (6.9%) patients had antenatal diagnosis. Micturating cystourethrogram confirmed PUV in all patients. Fourteen (48.3%) patients had impaired renal function (IRF) at presentation and 8 (57%) had improved renal function (RF) after initial catheter drainage. The mean creatinine at presentation was 1.86 ± 1.69 mg/dl and the mean serum creatinine following initial catheter drainage was 0. 93 ± 0.49 mg/dl (P = 0.003). For those patients with normal RF, the mean creatinine at presentation was 0.81 ± 0.22 mg/dl versus 0.74 ± 0.21 mg/dl (P = 0.012), following initial catheter drainage. Children with IRF on admission had mean creatinine at presentation of 2.61 ± 2.00 mg/dl compared to 1.17 ± 0.53 mg/dl (P = 0.002) after initial catheter drainage. Valve ablation was achieved with Mohan's valvotome in 26 (96.3%) patients. All patients had good urine stream at a median follow-up of 5 months. Four (13.8%) patients developed IRF at follow-up. Renal outcomes of patients presenting before 1 year and those presenting after 1 year were similar. Two children died preoperative of urosepsis and one out of hospital death given an overall mortality of 10.3% (n = 3). CONCLUSION: There was significant improvement in RF after initial catheter drainage. The incidence of IRF at follow-up was 13.8%. Long-term follow-up is necessary to identify patients at risk of end-stage renal disease.

Efficacy of bleomycin for non-operative treatment of cervical lymphangioma in University of Ilorin Teaching Hospital, Nigeria

Background: Lymphangiomas are the developmental defects of the lymphatic channels, and they are most commonly found in the head and neck regions. Late presentation, rejection of surgery, and traditional scarification result in fatal complications. Surgical excision often thought to give immediate relief and aesthetic results is associated with damage to contiguous structures and recurrence, hence, the need for less invasive treatment modality. Objective: To assess the effectiveness of bleomycin sclerotherapy of cervical lymphangiomas. Materials and Methods: This is a prospective study of patients with cervical lymphangioma treated with sclerosant injection between January 2008 and December 2016. Preinjection ultrasound scan and initial ultrasound-guided aspiration of the fluid in the swelling (which many times is multiloculated) using a 20G cannula into a 10 ml syringe were performed. The cannula tip is retained in the space and intralesional injection of double-diluted bleomycin 0.5 i.u./kg body weight was given as outpatient at 2­4-weekly interval. Postinjection events were documented. The clinical assessment of the pre- and postinjection of sclerosant was performed. Result: A total of 23 patients were recruited, and six were females and 17 were males. All swellings were noticed at birth but median time at presentation was 17 days. All patients but one (95.8%) had complete clinical resolution after 1­4 courses of sclerotherapy for 4­16 weeks. Only one patient had residual nodule that required surgical excision. Redundant skin and hyperpigmentation from skin wrinkle were the early effects noticed in three patients; however, these were cosmetically acceptable to the parents. No recurrence was recorded. Conclusion: The treatment of cervical lymphangiomas with intralesional bleomycin injection is shown to be effective. It is safe and associated with no complication. This treatment modality and outcome was found to be acceptable to the parents of these children

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.

Congenital prepubic sinus: a variant of epispadiac dorsal urethral duplication.

Congenital prepubic sinus is a tract originating in the skin overlying the base of the penis. Its embryologic basis is still debated. We present a 9-month-old boy with a recurrent muco-purulent discharge from a tiny opening in the midline prepubic area on the base of the penis. Examination revealed a ventrally hooded prepuce, dorsal chordee, penile torsion to the left, and a 3-mm-diameter prepubic sinus 1 cm from the base of the penis. Histology of the excised tract showed a transitional epithelium. We believe our case is an epispadiac variant of dorsal urethral duplication.

Complicated childhood inguinal hernias in UITH, Ilorin.

BACKGROUND: Complicated inguinal hernias pose a threat to the life of the child as well as increase the morbidity associated with management of an otherwise straightforward condition. The aim of this study was to determine the presentation, treatment and management outcome of complicated inguinal hernias in children. MATERIALS AND METHODS: A retrospective study of all children 15 years and less managed for complicated inguinal hernia between 2002 and 2010. Data obtained included demographic characteristics, presentation, operative findings and outcome. RESULTS: Complicated hernia rate was 13.9%.There were 41 children, 38 boys (92.7%) and 3 girls. Ages ranged between 4 days and 15 years (Median = 90days). Most were infants (48.8%, n = 20) and neonates accounted for 19.5% (n = 8). Median duration of symptoms prior to presentation was 18 h (range = 2-96 h). Seven patients had been scheduled for elective surgery. Hernia was right sided in 68.3% (n = 28). Symptoms included vomiting (68.3%), abdominal distension (34.1%) and constipation (4.9%); one patient presented with seizures. In 19 (46.3%) patients hernia was reducible while 22(53.7%) had emergency surgery. Associated anomalies included undescended testis (12.2%), umbilical hernia (14.6%). Intestinal resection rate was 7.3% and testicular gangrene occurred in 14.6%. Mean duration of surgery was 60.3 ± 26.7 min. Wound infection occurred in six patients (14.6%). Overall complication rate was 24.4%, 30% in infants. The mortality rate was 2.4% (n = 1). CONCLUSIONS: Morbidity associated with complicated inguinal hernia is high in neonates and infants. Delayed presentation is common in our setting. Educating the parents as well as primary care physicians on the need for early presentation is necessary.