Classical bladder exstrophy in an adolescent: A case report on management, challenges and outcome.

Classical bladder exstrophy is a congenital anomaly whose management and outcome has advanced over years. Management and outcome are better when management starts at the newborn period. This was the management of a neglected bladder exstrophy in a male presenting at 16 years of age. We report our challenges, management and outcome to highlight the rarity of this presentation, and the adaptation to the usual protocol of care. The patient presented at 16 years of age with classic bladder exstrophy. The bladder plate was contracted and had cystitis. The patient had a modification of complete primary repair of exstrophy (CPRE) with bilateral pelvic osteotomy stabilised with a 7-hole plate and 4 screws, then bladder neck reconstruction + bladder augmentation + cross-trigonal neocystoureterostomy in a 12-h procedure. He had surgical site infection, superficial wound breakdown and vesicocutaneous fistula that all healed with dressing and prolonged suprapubic cystostomy drainage. He achieved some degree of urinary continence and ability to void, though he still has stress incontinence and frequency at 6 months of follow-up. He has a micturition interval of 60-120 min, and is expected to improve. Presentation and repair of classic bladder exstrophy in the adolescent is very rare in the literature and therefore no known standard of care. This report adds to the body of knowledge. Again, this experience lends credence to the proponents of CPRE in reducing the number of procedures required to treat exstrophy.

Post-circumcision urethrocutaneous fistulae: presentations, repairs and outcomes in a tertiary centre.

Background: Circumcision is regarded as the most common surgical procedure world over. It is also perceived to be a simple and safe procedure; however, it could be fraught with major urological complications such as urethrocutaneous fistula (UCF). Repair of these fistulae poses a great reconstructive challenge to the paediatric surgeon/urologist with varied outcomes. Aim: We seek to review the presentations, repair and outcome of post-circumcision UCFs managed in a tertiary centre. Patients and Methods: This is a retrospective review of all the consecutive cases of repaired post-circumcision UCFs in Nnamdi Azikiwe University Teaching Hospital, Nnewi, South-East Nigeria, over a period of 9 years (January 2012-December 2020). Data on demography, size of fistula, type of repair and outcome were retrieved from the Records Department and patients' case notes and were analysed using SPSS (version 22, Chicago, Illinois). Results: A total of 22 boys had post-circumcision UCF repair within the period, and they were aged between 2 weeks and 108 months with a median age of 4 months at presentation and aged between 8 months and 144 months with a median age of 24 months at the time of surgery. Circumcisions were by freehand technique in 21 (95.5%). Nurses performed most of the circumcisions in 19 (86.4%), and most of the circumcisions were performed in private hospitals 7 (31.8%), maternity homes 3 (13.6%) and general hospitals in 10 (45.5%) each, respectively. Most 17 (77.3%) fistulae were coronal, and the size of defect ranged from 1 mm to 10 mm. The most commonly employed technique of repair was simple closure in 16 (72.2%). Meatal stenosis and re-fistulation occurred in 2 (9.1%) and 4 (18.2%), respectively. Only two (12.5%) required reoperation. There was no relationship between the size of defect and re-fistulation, P = 0.377. Conclusion: Majority of the Post-circumcision urethrocutaneous fistulae were from free hand technique of circumcisions . These were performed mostly by nurses in general, private hospitals and maternity homes. Hence, there is a need to ramp up training of providers of neonatal circumcision in our environment. Most UCF in children could be repaired with simple closure technique reinforced with dartos flap.

Anthropometric Predictors of Appropriate-Sized Plastibell for Neonatal Circumcision.

Background: Using anthropometric parameters to determine the appropriate Plastibell size before circumcision ensures that cumbersome carrying of all the sizes before each procedure is eliminated and also complications reduced. Methods: Male neonates who presented for routine circumcision by Plastibell method were recruited. Collected on a proforma were their age in days, weight in Kg, stretched penile length (SPL) in cm, penile diameter (PD) in cm and the Plastibell size used by the "circumciser". The routine circumcision was carried out for each neonate according to protocol. P value was set at <0.05. Results: There were 231 neonates who had Plastibell circumcision. Their mean age, weight, SPL and PD were 15.6(±5.73) days, 3.7(±0.58) Kg, 3.66 (±0.58) cm and 3.79 (±0.64) cm, respectively. Plastibell size 1.3 is the most used (53.6%). There was a positive correlation between weight, SPL, PD, on one hand and Plastibell size , on the other hand with P-values of <0.001, <0.001 and <0.001 respectively. The weight was a weak determinant for Plastibell sizes 1.1 and 1.3: (OR 7.104; 95% CI 1.108 - 45.559; P = .039) and (OR 2.044; 95% CI 1.054 - 3.963; P = .034) respectively. The SPL is also a weak predictor for Plastibell sizes 1.2 and 1.5: (OR 2.176; 95% CI 1.136 -4.136; P = .019) and (OR .043; 95% CI .072 - .984; P = .047), respectively. Conclusion: The anthropometric parameters correlate well with Plastibell sizes. However, they are not effective in predicting the appropriate sized Plastibell for neonatal circumcision.

Pentalogy of Cantrell: Reconstructive Challenges of Two Complete Cases in a Resource-Constrained Setting.

Pentalogy of Cantrell (POC) is a very rare congenital anomaly and treatment has remained a challenge worldwide owing to extensive reconstructive surgeries, more so in resource-constrained settings. We, therefore, report two cases of complete POC. Both successfully underwent planned reconstructions by a multidisciplinary team. The techniques and challenges are hereby highlighted.

Early Experience with Laparoscopic Management of Nonpalpable Undescended Testes.

BACKGROUND: Nonpalpable undescended testes (NPT) constitute 20%-30% of undescended testes, and its management has been a challenge both in diagnosis and treatment. Worldwide, laparoscopy is the current gold standard of management. In Nigeria, the management of NPT has largely been by open surgery with consequent high morbidity. In Nigeria, the trend is changing from a largely open management with its attendant high morbidity, to laparoscopic management which is the current worldwide gold standard of care. AIM: This study aims to classify the laparoscopic features of NPT and determine the outcome of managed cases in our center. METHODOLOGY: Prospective data were collected from consecutive patients who had laparoscopy for NPT at the Paediatric Surgical Unit of Nnamdi Azikiwe University Teaching Hospital, Nnewi, Nigeria from June 2014 to July 2016. RESULTS: A total of 15 patients with 23 testes were treated. There were eight patients with bilateral NPT; four had left and the remaining three right NPT. The age ranged from 1.2 to 29 years with a median of 5 years. Eleven out of the 22 internal inguinal rings were open. The position of the testes was canalicular (2), peeping (2), low abdominal (6), high abdominal (6), blind-ended vas (1), absent vas and vessels (5). No further intervention was needed for the six agenetic/atrophic testes. Standard open orchiopexy was done for the two canalicular testes. Eight testes were brought down by one stage laparoscopic orchiopexy while four were brought down by staged laparoscopic Fowler-Stephens procedure. Laparoscopic orchiectomy was done in two patients (a grossly dysmorphic testes [nubbin] and a high abdominal testis in a 29-year-old). Orchiopexy was successful in 11 out of 15 fixed testes. Of the unsuccessful ones, three testes were atrophic (volume less than what it was initially) while two were high scrotal (one testes has both complications). There was no conversion to open abdominal surgery. All patients were discharged within 24 h of surgery. CONCLUSION: Laparoscopy provides for a better management of NPT by combining diagnosis and intervention in the same sitting with a good success rate and minimal postoperative morbidity.

A Case of Retroperitoneal Malignant Triton Tumor in a Nigerian Boy.

Malignant peripheral nerve sheath tumor is a rare tumor occurring in 5%-10% of all malignant soft tissues sarcomas and triton tumor arising from neurofibromatosis type 1 (NF-1) is even rarer with associated high rate of mortality. No case of triton tumor has been reported in Nigeria to the best of our knowledge. We seek to report a case of lately detected retroperitoneal triton tumor presenting in a 12-year-old Nigerian child who was brought with bilateral lower limb weaknesses, weight loss, and a right lumbar mass. There were multiple café au lait spots on the body. Abdominal computerized tomographic scan revealed a huge right retroperitoneal mass crossing the midline, compressing adjacent structures with multilevel intraspinal extensions. Core needle biopsy performed and both histology and immunohistochemical studies confirmed the diagnosis, but patient demised in the course of care. The aim is to heighten suspicion of this extremely very rare malignant tumor in children with NF-1.

Comparison of trans-perineal ultrasound-guided pressure augmented saline colostomy distension study and conventional contrast radiographic colostography in children with anorectal malformation.

BACKGROUND: In children with high and intermediate anorectal malformation, distal colostography is an important investigation done to determine the relationship between the position of the rectal pouch and the probable site of the neo-anus as well as the presence or absence of a fistula. Conventionally, this is done using contrast with fluoroscopy or still X-ray imaging. This, however, has the challenges of irradiation, availability and affordability, especially in developing countries. This study compared the accuracy of trans-perineal ultrasound-guided pressure augmented saline colostomy distension study (SCDS) with conventional contrast distal colostography (CCDC) in the determination of the precise location of the distal rectal pouch and in detecting the presence and site of fistulous communication between the rectum and the urogenital tract was studied. MATERIALS AND METHODS: Trans-perineal ultrasound-guided pressure augmented SCDS, CCDC and intra-operative measurements were done sequentially for qualified infants with anorectal malformation and colostomy. Pouch skin distance and presence or absence of recto urinary or genital fistula was measured prospectively in each case. Statistical significance was inferred at P-value of <0.01. RESULTS: There were thirteen infants, 9 males and 4 females. The age at onset of investigation ranged from 2 to 12 months with a median value of 9 months. Using paired t-test at a confidence interval of 95%, the P value when SCDS values are compared with CCDC is 0.19; and 0.06 when SCDS was compared with intra-operative measurements. Hence, there is no statistical difference as P > 0.01. On its ability to detect presence or absence of a fistula: SCDS had a sensitivity of 50.0%, specificity of 100.0%, accuracy of 69.2%, negative predictive value of fistulas of 55.6% and a positive predictive value of fistulas of 100.0%. CONCLUSION: Ultrasound-guided pressure augmented SCDS can safely and reliably be used to assess the distal colonic anatomy and the presence of fistula in infants with Anorectal malformation who are on colostomy.

Parental circumcision preferences and early outcome of plastibell circumcision in a Nigerian tertiary hospital.

BACKGROUND: Parents are central in decisions and choices concerning circumcision of their male children and plastibell circumcision is a widely practiced technique. This study determined parental preferences for male neonatal and infant circumcisions and evaluate the early outcomes of plastibell circumcisions in a tertiary centre. PATIENTS AND METHODS: This is a prospective study on consecutive male neonates and infants who were brought for circumcisions at Nnamdi Azikiwe University Teaching Hospital Nnewi, South-East Nigeria and their respective parents between January 2012 and December 2012. Data on demography, parental choices and early outcome of plastibell circumcision were obtained and analysed. RESULTS: A total of 337 requests for circumcisions were made for boys with age range of 2-140 days. Culture and religion were the most common reasons for circumcision requests in 200 (59.3%) and 122 (36.2%), respectively, other reasons were medical, cosmesis, to reduce promiscuity and just to follow the norm. Most parents, 249 (73.9%) preferred the procedure to be performed on the 8 th day and 88.7% would like the doctors to perform the procedure while 84.6% preferred the plastibell method. Among those who had circumcision, 114 complied with follow-up schedules and there were complications in 22 (19.3%) patients. Parents assessed the early outcome as excellent, very good, good and poor in 30.7%, 45.6%, 18.4% and 5.3% of the patients, respectively. CONCLUSION: Parents request for male circumcision in our environment is largely for cultural and religious reasons; and prefer the procedure to be performed by a physician. Plastibell method is well known and preferred and its outcome is acceptable by most parents.