Penile Agenesis Associated with Anorectal Malformation and Bilateral Renal Agenesis: A Case Report and a Review of the Literature.

ABSTRACT: Penile agenesis is complete absence of the penis in an otherwise normal phenotypic and genotypic male at birth that results from failure of development of the genital tubercle. It is an extremely rare anomaly that may be associated with anomalies in other organ systems, the extent and severity of which may affect the prognosis. The management is challenging and may have far reaching implications for the individual and family. While gender reassignment with bilateral orchidectomy and feminising genitoplasty has been carried out for most patients, significant psychosexual and social issues related to the male identity may occur due to foetal or postnatal sex steroid imprinting. We report a neonate with penile agenesis with bilateral renal agenesis and anorectal malformation.

Comparison of dorsal penile nerve block alone and in combination with lidocaine-prilocaine cream in neonates undergoing circumcision: a randomized controlled study.

Background: Suboptimal neonatal circumcision analgesia causes a significant stress response. There is continued search for more effective analgesia for neonatal circumcision. We aimed to determine whether lidocaine-prilocaine cream (LPC) prior to dorsal penile nerve block (DPNB) offers better analgesia than DPNB alone. Methods: A prospective randomized study of 72 neonates undergoing plastibell circumcision was randomized into two groups of 36 each. Group I received DPNB alone with 4 mg/kg plain lidocaine. Group II had 1 g LPC applied to the phallus 1 hour before DPNB. Serum cortisol levels were measured 30 min preoperatively and 30 min postoperatively. Heart rate (HR) and peripheral arterial oxygen saturation (SpO2) were noted at 30 min preoperatively, after DPNB injection, after excising the prepuce, and at 30 min thereafter. The primary outcome measure was change in serum cortisol levels, while secondary outcomes were changes in HR and SpO2. Results: Both groups had increased mean serum cortisol levels, decreased mean SpO2 and increased mean HR from baseline. The rise in mean serum cortisol level was significantly lower in group II (8.09±4.53 µg/dL) than that in group I (11.42±7.83 µg/dL) (p=0.034). Changes in SpO2 and HR were significantly less in group II than that in group I patients (p<0.05), except for HR changes at 30 min after excision of the prepuce (p=0.086). Conclusion: These data suggest that neonates who received LPC prior to DPNB experienced significantly less pain from plastibell circumcision than those who had DPNB alone. Trial registration number: PACTR201906554747665.

Comparison of the Inguinal and High Scrotal Approaches for the Treatment of Inguinal Hernias in Boys.

BACKGROUND: The conventional groin incision herniotomy is still being adhered to despite high success rate of high scrotal approach. Hence, the aim of this study was to compare the outcomes of high scrotal and conventional approaches for the treatment of inguinal hernia in boys. METHODS: A prospective study of 100 boys with 108 inguinal hernias whose ages were less than 15 years. They were randomized into 2 groups; high scrotal and conventional approaches. Ninety-four patients with 101 hernias were analyzed. The operative time, conversion rate (high scrotal approach), and postoperative complications were reported. RESULTS: A total of 100 boys with 108 hernias were enrolled but 94 patients with 101 hernias were analyzed. They comprised of 48 patients with 51 hernias in the high scrotal group and 46 patients with 50 hernias in the conventional group. Their age range was between 2 months and 168 months with a mean of 47.9 ± 46.7 months. The conversion rate of high scrotal approach was 1.9%. The mean duration of operation in the high scrotal group was 37.1 ± 13.3 minutes compared with 37.2 ± 15.1 minutes in the conventional group, p = 0.982. Early postoperative scrotal edema was more in the high scrotal group compared to the conventional group, p = 0.018. The Hollander wound evaluation score was better in the high scrotal incision compared to the conventional approach, p = 0.003. CONCLUSION: The high scrotal approach may be an alternative to conventional herniotomy in boys.

Perioperative mortality in children in a tertiary teaching hospital in Nigeria: a prospective study.

Background: Perioperative mortality is one of the tools that can help to assess the adequacy of healthcare delivery in a nation. This audit was conducted to determine the 24-hour, 7-day and 30-day perioperative mortality rates and the predictors of mortality in a pediatric surgical cohort. Methods: This was a prospective study of children whose ages ranged from a few hours to 15 years and who were operated on between May 2019 and April 2020. The primary outcome was to determine the incidence of in-hospital perioperative mortality. Results: A total of 530 procedures were done in 502 children. Their ages ranged from a few hours to 15 years with a median of 36 months. The 24-hour, 7-day and 30-day mortality rates were 113.2 per 10 000 procedures [95% confidence interval (CI) =40 to 210], 207.6 per 10 000 procedures (95% CI=110 to 320) and 320.8 per 10 000 procedures (95% CI=190 to 470), respectively. Congenital anomalies complicated by postoperative sepsis contributed to death in the majority of cases. The predictors of mortality were neonatal age group [adjusted odds ratio (AOR)=19.92, 95% CI=2.32 to 170.37, p=0.006], higher American Society of Anesthesiologists Physical Status III and above (AOR=21.6, 95% CI=3.05 to 152.91, p=0.002), emergency surgery (AOR=17.1, 95% CI=5.21 to 60.27, p=0.001), congenital anomalies (AOR=12.7, 95% CI=3.37 to 47.52, p=0.001), and multiple surgical procedures (AOR=9.7, 95% CI=2.79 to 33.54, p=0.001). Conclusion: Perioperative mortality remains high in our institution.

Mitigating the challenges of laparoscopic paediatric surgery in Ile Ife: The trend so far and lessons learnt.

BACKGROUND: Paediatric laparoscopic surgery is fast growing worldwide, with many pathologies now being treated even in the youngest of patients. We hereby report our experience with the first 114 cases. OBJECTIVES: Our aim was to highlight our progress and lessons learnt practicing laparoscopic paediatric surgery in our institution. MATERIALS AND METHODS: This is a retrospective study of the first 114 children who underwent laparoscopic surgery in our hospital. We focussed on demographics, indications, procedures performed, rate of conversion to open and complications. Records were retrieved from January 2011 to December 2019. Data were analysed using the SPSS software version 23 (SPSS Inc., Chicago, Illinois, USA). RESULTS: There were 83 males and 31 females (ratio of 3:1). Age groups included infants (13.2%), 1-5 years (21.9%), 5-10 years (33.3%) and > 10 years (31.6%). There was a remarkable increase in the frequency and complexity of cases performed from an average of 5 per year between 2011 and 2015 to an average of 23.5 per year between 2016 and 2019. The conversion rate was 6%, 5 appendectomies, 1 Swenson pull-through, 1 diagnostic laparoscopy and 1 Ladd's procedure. Four complications were noted; one recurrent adhesive intestinal obstruction, one residual intra-abdominal abscess, one port site abscess and one excessive bleeding from liver biopsy requiring conversion to open surgery. CONCLUSION: We have demonstrated that the routine use of laparoscopy in children is feasible and safe in our environment. However, the need for training, endurance through a steep learning curve and the willingness to battle the technical challenges are necessary for success.

Sonographic guided hydrostatic saline enema reduction of childhood intussusception: a prospective study.

BACKGROUND: The management of childhood intussusception in our sub-region is still via surgical intervention. Currently, the gold standard of treatment is non-operative reduction. We sought to assess the suitability of hydrostatic (saline) reduction of intussusception in children in our institution. MATERIALS AND METHODS: A prospective study was conducted between January 2016 and June 2017 in all children with ultrasound confirmed intussusception at a tertiary teaching hospital in Nigeria. All children excluding those with signs of peritonitis, bowel gangrene and intestinal prolapse were selected for ultrasound-guided hydrostatic reduction (USGHR). We allowed a maximum of three attempts at reduction. RESULTS: The age range was 3 months to 48 months with a mean of 10.8 ± 9.1 months. Forty percent (N = 18) presented after 24 h of onset of symptoms. The success rate of hydrostatic reduction with saline enema was 84.4% (N = 38). Two (4.4%) perforations occurred during the procedure. Three (7.5%) patients had recurrent intussusception within six months. The duration of symptoms greater than 24 h, age and sex of patients did not influence successful reduction p > 0.05. The duration of admission between those who had successful non-operative reduction and those who subsequently had operative reduction and or resection attained statistical significant difference, p = 0.001. There was no mortality. We achieved a 68% decrease in the operative reduction of intussusception using USGHR as the primary modality of treatment. CONCLUSION: Our study found out that USGHR is a suitable alternative for the treatment of childhood intussusception.

A 10-year retrospective review of perioperative mortality in pediatric general surgery at Ile-Ife Hospital, Nigeria.

BACKGROUND/PURPOSE: The analysis of perioperative mortality as well as surgery- and anesthesia-related death in pediatric patients may serve as a potential tool to improve outcome. The aim of this study is to report the 24-h and 30-day overall, and surgery and anesthesia-related, mortality in a tertiary hospital. METHODS: This is a retrospective review of perioperative mortality in children ≤15years at a general pediatric surgery unit. All pediatric general surgery cases operated under general anesthesia between January 2007 and December 2016 were included in the study and data analyzed. RESULTS: A total of 4108 surgical procedures were performed in 4040 patients. The age was 1day to 15years with a median age of 2years. The all cause 24-h mortality was 34 per 10,000 procedures and the all cause 30-day mortality was 156 per 10,000 procedures. Septicemia was the most common cause of death. The determinants of mortality were neonatal age group (Adjusted Odd Ratio (AOR)=0.033, 95% CI=0.015-0.070, p=0.001), emergency surgery (AOR=90.91, 95% CI=27.78-333.33, p=0.001), higher ASA status (AOR=0.014, 95% CI=0.005-0.041, p=0.001) and multiple operative procedures (AOR=38.46, 95% CI=10.64-142.85, p=0.001). CONCLUSIONS: Neonatal age group, children with poorer ASA status, emergency and multiple surgeries were predictors of perioperative mortality. LEVEL OF EVIDENCE: Retrospective study.

Posterior Urethral Valves in Children: Pattern of Presentation and Outcome of Initial Treatment in Ile-Ife, Nigeria.

BACKGROUND: The management of posterior urethral valves (PUV) and its sequelae is still a challenge to most pediatric surgeons in our environment due to late presentation and inadequate facilities for long-term evaluation and treatment. Despite initial successful treatment about 40% would develop chronic renal failure. The aim is to describe the presentation, management and outcome of the initial treatment in boys with PUV. MATERIALS AND METHODS: It is a retrospective analysis of PUV in boys 8 years and below over a 17 years period. Demographic characteristics, clinical features, investigations, and treatment outcome were reviewed. RESULTS: Thirty-seven cases were analyzed. The median age was 5 months (range from birth to 8 years). Three (8.1%) patients had prenatal ultrasound diagnosis. The most common presentation was voiding dysfunction 37 (100%). Part of the preoperative investigation included micturating cystourethrogram (n = 31: 83.8%) and abdomino-pelvic ultrasonography (n = 37:(100%). The mean serum creatinine value of those who presented within the first 30 days of life and those who presented afterwards were 325 (±251) µmol/L and 141 (±100) µmol/L respectively, P = 0.003. Surgical interventions included trans-vesical excision of valves (n = 9: 28.1%), valvotomy (n = 10: 31.3%), balloon avulsion (n = 8: 25.0%), vesicostomy (n = 4: 12.5%) and endoscopic valve avulsion (n = 1: 3.1%). Seventeen (56.7%) patients had serum creatinine >70.4 µmol/L after 1-month of valve excision. Five (13.5%) patients had postrelief complications and 5 (13.5%) died on admission. Ninety percentage (27/30) of patients had poor prognostic indices. CONCLUSIONS: The initial treatment outcome was good but most had poor prognostic factors.

Early experience with laparoscopic surgery in children in Ile-Ife, Nigeria.

BACKGROUND: Laparoscopy is not yet routinely employed in many Paediatric Surgical Units in Nigeria despite the advantages it offers. This study describes the preliminary experience with laparoscopic procedures in a single centre. PATIENTS AND METHODS: A retrospective analysis of all children who had laparoscopic surgery between January 2009 and December 2013 at the Paediatric Surgical Unit of Obafemi Awolowo University Teaching Hospitals Complex Ile-Ife was carried out. Their sociodemographic, preoperative and intraoperative data along with postoperative records were subjected to descriptive analysis. RESULTS: Eleven (44%) diagnostic and 14 (56%) therapeutic procedures were performed on 25 children whose age ranged from 5 months to 15 years (Median: 84 months, Mean: 103 ± 64.1 months), including eight (32%) females and 17 (68%) males. Indications included acute appendicitis in 12 (48%), intra-abdominal masses in six (24%), three (12%) disorders of sexual differentiation, two (8%) ventriculoperitoneal shunt malfunctions and impalpable undescended testes in two (8%) children. The procedures lasted 15-90 minutes (Mean = 54 (±21.6) minutes). Conversion rate was 17% for two patients who had ruptured retrocaecal appendices. No intra operative complications were recorded while three (12%) patients had superficial port site infections post-operatively. All diagnostic (11) and two therapeutic procedures were done as day case surgery. The mean duration of hospital stay was 3.1 (±3.3) days for those who had appendectomies. CONCLUSION: Laparoscopic surgery in children is safe and feasible in our hospital. We advocate increased use of laparoscopy in paediatric surgical practice in Nigeria and similar developing settings.

Early experience with laparoscopic surgery in children in Ile-Ife, Nigeria

Background: Laparoscopy is not yet routinely employed in many Paediatric Surgical Units in Nigeria despite the advantages it offers. This study describes the preliminary experience with laparoscopic procedures in a single centre. Patients and Methods: A retrospective analysis of all children who had laparoscopic surgery between January 2009 and December 2013 at the Paediatric Surgical Unit of Obafemi Awolowo University Teaching Hospitals Complex Ile-Ife was carried out. Their sociodemographic, preoperative and intraoperative data along with postoperative records were subjected to descriptive analysis. Results: Eleven (44%) diagnostic and 14 (56%) therapeutic procedures were performed on 25 children whose age ranged from 5 months to 15 years (Median: 84 months, Mean: 103 ± 64.1 months), including eight (32%) females and 17 (68%) males. Indications included acute appendicitis in 12 (48%), intra-abdominal masses in six (24%), three (12%) disorders of sexual differentiation, two (8%) ventriculoperitoneal shunt malfunctions and impalpable undescended testes in two (8%) children. The procedures lasted 15-90 minutes (Mean = 54 (±21.6) minutes). Conversion rate was 17% for two patients who had ruptured retrocaecal appendices. No intra operative complications were recorded while three (12%) patients had superficial port site infections post-operatively. All diagnostic (11) and two therapeutic procedures were done as day case surgery. The mean duration of hospital stay was 3.1 (±3.3) days for those who had appendectomies. Conclusion: Laparoscopic surgery in children is safe and feasible in our hospital. We advocate increased use of laparoscopy in paediatric surgical practice in Nigeria and similar developing settings

Predictors of mortality in children with typhoid ileal perforation in a Nigerian tertiary hospital.

PURPOSE: Childhood typhoid ileal perforation is associated with high morbidity and mortality. Our aim was to ascertain the predictors of survival in children. MATERIALS AND METHODS: This is a tertiary hospital-based retrospective review of patients aged ≤15 years managed for typhoid ileal perforations between January 2005 and December 2013. The details of their biodata, potential risk factors and outcome were evaluated. RESULTS: Forty-five children out of a total of 97 with typhoid fever had typhoid ileal perforation. The age range was 2-15 years, mean (±SD) = 9.3 (±3.31) years, median = 10 years. There were more males than females (26:19). Thirty-nine (86.7%) patients were >5 years old. There were nine deaths (20% mortality). The mean (±SD) age of survivors was 9.8 (±2.9) years and 7.1 (±4.2) for non-survivors (p = 0.026). The duration of illness at presentation, gender, admission temperature, nutritional status and packed cell volume, perforation-operation interval, number of perforations, surgical procedure, and the duration of surgery did not statistically influence survival (p > 0.05). The age of the patients and burst abdomen attained statistical significance (p < 0.05). CONCLUSION: The patients' age and postoperative burst abdomen were significant determinants of survival in children with typhoid ileal perforation.

Childhood intussusception in Ile-ife: what has changed?

BACKGROUND: Intussusception is one of the most common causes of intestinal obstruction in children. While the outcome has improved in the developed nations, the same cannot be said of the developing countries, more especially in the sub-Saharan region. This study aims to review our current experience in the management of childhood intussusception and factors affecting surgical outcome at the Obafemi Awolowo University Teaching Hospitals Complex Ile-Ife. PATIENTS AND METHODS: This was a retrospective study of 78 patients treated for intussusception at paediatric surgical unit of Obafemi Awolowo University Teaching Hospitals Complex Ile-Ife between January 1993 and December 2011. The case notes of the patients were retrieved and the following information was recorded: Demographic characteristics, month of occurrence, clinical presentation, investigations, and management as well as the post-operative outcome. The patients were divided into two groups in terms of outcome. RESULTS: There were 58 males and 20 females (M:F = 2.9-1). The age of most of the patients was between 3 months and 9 months with peak incidence at 6 months. Most patients 46 (58.9%) were seen during the dry season of December to April. Only six patients (7.7%) presented within 24 hours of onset of illness. More than half of the patients presented after 24 hours. Passage of red currant stool, vomiting, abdominal pain, fever, and abdominal distension, passage of watery stool, anal protrusion and palpable abdominal mass in various combinations were the clinical features. All the patients had surgical operations. The most common type of intussusception was ileo-colic type in 64 patients (82.1%). Intestinal resection rate was 41%. The overall mortality rate was 15.4%. CONCLUSION: There was a delay in presentation of children with intussusception with high post-operative mortality.

Situs inversus in association with duodenal atresia.

Situs inversus in association with duodenal atresia is very rare. A high index of suspicion coupled with appropriate evaluation is necessary for diagnosis and operative planning. We report a case of a 5-day-old who presented with duodenal atresia associated with polysplenia and situs inversus with a review of the medical literature.

Ten-year experience with the Swenson procedure in Nigerian children with Hirschsprung's disease.

BACKGROUND: Hirschsprung's disease (HD) is a common cause of intestinal obstruction in children. Definitive treatments consist of excision of the aganglionic segment and anastomosing the normal colon to the anal remnant. The outcome of this approach in Nigerian children is not known. The aim of this study was to analyze the short to mid-term outcome of children who have undergone the Swenson Procedure (SPT) as a treatment of HD over a period of 10 years. PATIENTS AND METHODS: The clinical data of biopsy-proven cases of HD managed at the Obafemi Awolowo University Teaching Hospital, Ile-Ife, Nigeria, between 1998 and 2007 were reviewed. RESULTS: There were 33 children 28 were males and five were females (M 5.6: F = 1). The median age at presentation was eight months (range three days to 11 years). Eighteen (54.5%) patients had primary pull-through without colostomy while 15 (45.5%) patients had a two-staged operation. The operation was carried out at a median age of 14 months with a range of seven weeks to 11 years, four months. Twenty-one (64%) of the patients were older than one year at the time of surgery. The level of aganglionosis was in the recto-sigmoid area in 30 (91%) patients. The commonest post-SPT complication was intestinal obstruction from adhesions. Bowel opening varied from once daily to eight times daily, median thrice daily. CONCLUSION: The Swenson's Procedure is an effective procedure in the treatment of HD in children in Ile Ife, Nigeria.

Focal segmental glomerulosclerosis and nephrotic syndrome in a child with embryonal rhabdomyosarcoma.

A case of nephrotic syndrome (NS) and acute renal failure (ARF) associated with embryonal rhabdomyosarcoma (RMS) in a 10-year-old boy is reported. Ultrasound revealed irregular, echogenic, circumferential urinary bladder base mass, bilateral hydroureter and hydronephrosis. Histopathology of percutaneous renal and urethrocystoscopic biopsy specimens, respectively, revealed focal segmental glomerulosclerosis (FSGS) and embryonal RMS. Tumour remission was induced with pulse doses of intravenous vincristine, cyclophosphamide, methotrexate and actinomycin D over a 15-month period. He has been followed-up for 28 months and has maintained a drug-free tumour and proteinuria remission for 1 year. While some malignancies have been reported in association with NS, its occurrence in association with RMS is quite exceptional. We conclude that RMS may be associated with FSGS and NS. Effective treatment of the RMS was associated with sustained remission of the nephrotic proteinuria.