Characteristics and Clinical Outcomes of Children With Wilms' Tumour: A 15-year Experience in a Single Centre in Nigeria.

BACKGROUND: Wilms' tumor (WT) is the most common paediatric renal tumor and is one of the most treatment-responsive solid tumours. Survival from Wilms tumour (WT) in sub-Saharan Africa remains dismal as a result of late presentation, treatment abandonment and infrastructure deficit. The purpose of this study was to analyze the clinical outcome of children with Wilms tumour managed in a Nigerian referral centre over a 15-year period. METHODS: This is a retrospective study of children with WT (nephroblastoma) who were treated at our institution between January 2006 and December 2020. Clinical characteristics, treatments, and outcomes were analyzed. RESULTS: Thirty-five patients were identified. The median age at diagnosis was 36 months including 22 (62.9 %) females. Twenty-six (74.3 %) had advanced (stage III & IV) disease. Confirmatory histology was available for 16 patients ((45.7 %) among which 10 (62.5 %) were mixed type. The right kidney was affected in 18 patients (51.4 %), left in 15 (42.9 %) and 2 were not documented. Preoperative chemotherapy was given in 22 (62.9 %) patients and 13 (37.1) patients had primary nephrectomy. Eight (22.9 %) patients died during treatment (from disease or treatment related causes), and one abandoned treatment. A total of 26 patients completed treatment. Out of these, 8 (30.8 %) were lost to follow up, four patients died and 14 (53.8 %) patients survived at a median follow-up period of 18 months. The survival decreased with advancing stages of the disease, p = 0.002. CONCLUSIONS: Majority of children with Wilms tumour in our practice presented with advanced disease. Death during treatment, treatment abandonment and lost to follow up were common. LEVEL OF EVIDENCE: Level II. TYPE OF STUDY: Retrospective Study.

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.

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.

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

Medical students' perception of objective structured clinical examination: a feedback for process improvement.

BACKGROUND: Medical educators have always been desirous of the best methods for formative and summative evaluation of trainees. The Objective Structured Clinical Examination (OSCE) is an approach for student assessment in which aspects of clinical competence are evaluated in a comprehensive, consistent, and structured manner with close attention to the objectivity of the process. Though popular in most medical schools globally, its use in Nigeria medical schools appears limited. OBJECTIVES: This study was conceived to explore students' perception about the acceptability of OSCE process and to provide feedback to be used to improve the assessment technique. DESIGN: A cross-sectional survey was conducted on final-year medical students, who participated in the final MBBS surgery examination in June 2011. A 19-item self-administered structured questionnaire was employed to obtain relevant data on demographics of respondents and questions evaluating the OSCE stations in terms of the quality of instructions and organization, learning opportunities, authenticity and transparency of the process, and usefulness of the OSCE as an assessment instrument compared with other formats. Students' responses were based on a 5-point Likert scales ranging from strongly disagree to strongly agree. The data were analyzed using SPSS, version 15 (SPSS, Inc, Chicago, IL). SETTING: The study took place at the University of Ilorin, College of Health Science. PARTICIPANTS: A total of 187 final-year medical students were enrolled in to the survey. RESULTS: Of 187 eligible students, 151 completed the self-administered questionnaire representing 80.7% response rate. A total of 61 (40.4%) students felt that it was easy to understand written instructions at the OSCE stations. In total, 106 (70.2%) students felt that the time allocated to each station was adequate. A total of 89 (58.9%) students agreed that the OSCE accurately measured their knowledge and skill, and 85 (56.3%) reported that OSCE enhanced their communication skill. Of the respondents, 80 (53%) felt that OSCE caused them to be nervous, and 73 (48.3%) expressed their concern about the interevaluator variability at manned stations. OSCE was perceived to be the second most fair test format by 53 (35.1%) respondents, and 56 (37.1%) also suggested that OSCE needs to be used much more than the other assessment formats. CONCLUSION: The findings in this survey appear reassuring regarding students' perception about the validity, objectivity, comprehensiveness, and overall organization of OSCE in the department of surgery. The need to introduce OSCE early in the surgical curriculum is also underscored. The overall feedback was very useful and will facilitate a critical review of the process.

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.

Genitourinary plexiform neurofibroma mimicking sacrococcygeal teratoma.

Neurofibromatosis is a common inherited autosomal dominant disease, but genitourinary neurofibroma is rare. The unpredictable nature of neurofibromas has a serious impact on the quality of life of patients, and their management is challenging for clinicians. We present a 9-year-old girl with plexiform neurofibroma of genitourinary system associated with pulmonary hypertension, masquerading as sacrococcygeal teratoma. Intraoperative finding and histological examination of the resected tumor confirmed the diagnosis.

Is intraabdominal drainage necessary after laparotomy for typhoid intestinal perforation?

PURPOSE: This study was designed to compare the safety and effectiveness of routine drainage and nondrainage after emergency laparotomy for typhoid intestinal perforation (TIP). METHODS: A retrospective review of children 15 years or younger who underwent surgery for TIP from 2002 to 2009 was performed. All children underwent resuscitation and laparotomy and were given antibiotics but were then divided into 2 groups: group I (n = 81), postoperative peritoneal drainage, and group II, (n = 66) no drainage. RESULTS: There was no demographic difference between the groups (e.g., mean age 9.6 vs 9.0 years; P = .21). There was no significant difference in mean time for return of bowel function (3.8 vs 4.0 days; P = .6), rate of surgical site infection (63% vs 70%; P = .39), wound dehiscence (36% vs 27%; P = .27), anastomotic leak (2.5% vs 1.5%; P = .27), enterocutaneous fistula formation (10% vs 6.1%; P = .40), intraabdominal abscess formation (4% vs 9%; P = .18), or mean length of hospital stay (22 vs 19 days; P = .26). CONCLUSION: The results of this study clearly show that routine peritoneal drain placement after laparotomy for TIP is unnecessary, and such drains are not effective in reducing the rate of postoperative complications.

Challenges and outcomes of management of anterior abdominal wall defects in a Nigerian tertiary hospital.

BACKGROUND: Abdominal wall defect presents a great challenge when it is large, ruptured, or associated with other anomalies. OBJECTIVE: To review the challenges and outcome of management of anterior abdominal wall defects (AAWD). MATERIALS AND METHODS: A retrospective review of omphalocele and gastroschisis managed over 8 years at our institution. RESULTS: Omphalocele (n=49) and gastroschisis (n=7) constituted 2.4% of total admission. The median age was 23.5 hours, with male-female ratio of 1:1.1. Term infants were 91.7% and more than 75% weighed above 2.5 kg. The mean maternal age was 28.5±5.87 years and mean parity was 3.1±2.0, with P values of 0.318 and 0.768, respectively. More than 92.9% of infants were out-born, 46 pregnancies (82.1%) were booked, and 51 (91.1%) had at least one ultrasound scan, but only 1 (1.8%) was diagnosed with gastroschisis. Ruptured omphalocele were 11 (6 major, 5 minor) in number, 3 of which presented with enterocutaneous fistula, and 3 (6.1%) were syndromic omphalocele. Positive blood culture confirmed septicaemia in 21 cases (37.5%). Surgical repair was done in 35 cases (62.5%), 44.6% as emergency, and 17.9% as elective. Non-operative management was done in 21 patients (37.5%) and 5 (8.9%) were discharged against medical advice. Median length of hospital stay was 10 days (mean, 15.98±14.38). Postoperative complication rate was 32.1% and overall mortality was 30.4%, with the highest case fatality among gastroschisis (57.1%) and omphalocele major (32.1%). CONCLUSIONS: There were large numbers of out-born infants due to poor prenatal diagnosis in spite of high instances of antenatal ultrasound scan. Many patients presented with complications that resulted in poor outcome.

Posterior urethral valve.

BACKGROUND: Posterior urethral valve (PUV) is a significant cause of morbidity, mortality and ongoing renal damage in children. It accounts for end-stage renal disease in a proportion of children. This article aims at highlighting the current trend in the management of boys with posterior urethral valve. DATA SOURCES: PubMed/Medline and bibliographic search for posterior urethral valve was done. Relevant literatures on presentation, pathology, evaluation, management and outcomes of PUV were reviewed. RESULTS: PUV which is increasingly diagnosed prenatally presents a spectrum of severity. The varied severity and degree of obstruction caused by this abnormality depend on the configuration of the obstructive membrane within the urethra. The decision to intervene prenatally is dependent on gestational age, amniotic volume, and renal function of fetal urine aspiration. Identification of the patients who may benefit from early intervention remains inconclusive. Endoscopic ablation of the valve is the gold standard of treatment but use of Mohan's valvotome and other modalities are invaluable in developing countries where endoscopic facilities are limited. Proximal urinary diversion may result in poor bladder compliance and should be reserved for patients with persisting or increasing upper urinary tract dilatation, increasing serum creatinine or inappropriate instruments. The behavior of the bladder and its subsequent management after valve ablation may influence the long-term renal outcome in PUV patients. CONCLUSIONS: The care of children with PUV continues to improve as a result of earlier diagnosis by ultrasound, developments in surgical technique and meticulous attention to neonatal care. The ultimate goal of management should be to maximize renal function, maintain normal bladder function, minimize morbidity and prevent iatrogenic problems.

Predictor of mortality in children with typhoid intestinal perforation in a Tertiary Hospital in Nigeria.

PURPOSE: Typhoid intestinal perforation (TIP) exerts a great toll both on the patients and the surgeons in resource poor countries. Factors that predict mortality in patients with TIP remain controversial. The study aims to determine factors that predict mortality in a Nigerian tertiary facility and to offer strategies for improvement. MATERIALS AND METHODS: We conducted a retrospective analysis of data of 153 children who underwent surgery for TIP at a Tertiary Hospital in Nigeria over a period of 8 years (January 2002 to December 2009). Data collected included patient demographics, presentation, haemoglobin level (Hgb), presentation-operation interval, type of surgical procedure, nature of peritoneal collection, the number of perforations and duration of surgery. Postoperative complications, duration of hospital stay and outcome were also examined. RESULTS: There were 99 (64.7%) boys and 54 (35.3%) girls aged 3-15 years, mean (SD) = 9.4 (±3.6) years. There were 105 single perforations and 46 multiple perforations (range 1-32). The surgical procedure was simple 2-layer closure in 128 (83.7%) and segmental resection in 9 (5.9%) children. The mortality was 16 (10.4%). The mean (SD) age of children who survived and those who died was 9.3 (±3.6) years and 10.1 (±4.0) years, respectively; p = 0.446, the mean duration of symptom in children who survived and those who died was 10.3 (±4.9) days and 12.3 (±5.6) days; p = 0.164, and the mean interval between presentation and operation in those who died and those who survived was 29.3 (±25) h and 28.4 (±13.4) h; p = 0.896. Temperature ≥ 38.5°C (p = 0.040), anastomotic leak (p = 0.029) and faecal fistula (p = 0.000) were significantly associated with mortality. Age <5 years (p = 0.675), male gender (p = 0.845), presentation-operation interval ≥24 h (p = 0.940), Hgb less than 8 g/dL (p = 0.058), faeculent peritoneal collection (p = 0.757), number of perforations (p = 0.518) and the surgical technique (p = 0.375) were not related to mortality. Logistic regression analysis showed that only postoperative faecal fistula (p = 0.001; OR = 13.7) independently predicted mortality. CONCLUSION: Development of postoperative fecal fistula significantly predicted mortality. Prioritizing the prevention of typhoid fever than its treatment and attention to surgical details may significantly reduce mortality of TIP in children in this setting.

Penile shaft sinus: A sequalae of circumcision in urethral duplication.

Urethral duplication (UD) is rare congenital anomalies with varied presentation. Careful clinical evaluation of children by specialist would enhance diagnosis, adequate management and reduce occurrence of complication. We present a 12-year-old boy with chronic post circumcision ventral penile sinus that was successfully managed for urethral duplication.