Single Median Raphe Scrotal incision Orchiopexy: A safe & feasible approach for fixation of Palpable Undescended testes.

BACKGROUND: We wish to share our outcome of single median raphe scrotal incision orchiopexy (SMRSO) regarding safety & feasibility of technique by evaluating ease of access, conversion rate, duration of surgery, success rate, complications, and the need for redo-surgery. METHODS: We retrospectively analyzed data of 277 orchiopexies performed in our department on 224 patients operated on between June 2016 to June 2019. SMRSO was considered for palpable testes that can be brought to the upper limit of the scrotum under anesthesia. The conventional inguinoscrotal approach was used for high lying testes. A median raphe incision was made to access & mobilize the testis on either side, ligation of processus vaginalis performed, and the testes secured in the scrotal pouch. The approach was utilized for both unilateral and bilateral orchiopexies. Follow-up done at one week, one month & six months to evaluate the outcome. RESULTS: A total of 277 orchiopexies were performed in 224 patients. 237 (86%) orchiopexies were via the median raphe scrotal approach in 184 patients. Out of these, 53 cases had bilateral orchiopexies. 30 (11%) performed via a conventional inguinoscrotal approach and 10 (4%) by laparoscopic approach. The mean duration of surgery for SMRSO was 24 minutes for unilateral and 42 minutes for bilateral cases. Immediate postoperative complications included scrotal hematoma in three (1.6%) cases, Hematoma of the abdominal wall in one case, and scrotal edema in 4 (2 %) patients. All complications were treated conservatively & resolved. No wound infection or testicular atrophy was reported. Long-term complications included testicular ascend in three cases [1.6%]. CONCLUSION: Single-incision Median Raphe Scrotal orchiopexy is an attractive alternative to the standard inguinoscrotal orchiopexy for palpable low lying undescended testes with a better cosmetic outcome.

Factors affecting survival in patients with oesophageal atresia and tracheo-oesophageal fistula.

OBJECTIVE: To evaluate the various factors affecting survival in babies with oesophageal atresia and tracheo-oesophageal fistula. DESIGN: Descriptive study. SETTING: The study was carried out at the Department of Paediatric Surgery, The Children's Hospital, Pakistan Institute of Medical Sciences (PIMS), Islamabad from March 2004 to March 2005. PATIENTS AND METHODS: All neonates with oesophageal atresia (EA) and tracheo-oesophageal fistula (TEF) during the study period were included in the study. Patients having isolated EA were excluded. A total of 80 patients were included in the study. Patients were received from the emergency department, OPD and Neonatal ICU. Diagnosis was confirmed by passing a radio opaque orogastric tube. Investigations were done to look for other associations. After stabilisation, right thoracotomy was performed, fistula was ligated and divided. An attempt was made to do a primary oesophago-oesopahgostomy. Nasogastric feeding was started on 2nd post-operative day. A contrast oesophagogram was performed on the 7th postoperative day and having ruled out leak, oral feeding was started. RESULTS: Out of the total, 33 (41%) survived and 47 (58%) patients died. Out of 47 deaths 20 (25%) died before surgery and 27 (34%) died after surgery. Mean follow up period was 6 months. Sixteen (20%) patients had anastomotic leak, 24 (30%) had anastomotic stricture, and 64 (80%) patients had postoperative pneumonia. CONCLUSION: We conclude that proper antenatal check ups will detect the problem early, avoid home deliveries and hence improve survival. Pneumonitis and septicaemia significantly affect survival. Availability of ICU is one of the main determinants of survival. The likely cause of high mortality rate in pre-operative patients in our series is non-availability ofNICU due to limited space in our setup.

Association of Shah-Waardenburgh syndrome: a review of 6 cases.

UNLABELLED: Shah-Waardenburg syndrome (SWS) is a neurocristopathy and is characterized by Hirschsprung's disease (HD), deafness, and depigmentation of hairs, skin, and iris. OBJECTIVE: The aim of the article is to study the relative frequency of associations in 6 consecutive cases of SWS. METHODS: A review of 6 consecutive patients with SWS was performed to study the frequency of various components of the syndrome. RESULTS: Six patients had features of SWS. All patients had HD; of these, 3 had rectosigmoid HD, whereas 3 had extended HD. All patients had white forelock of hairs with skin depigmentation. One patient had sensorineural deafness, whereas other babies were less than 1 year, and thus, full evaluation of hearing deficiency was not assessed. Three patients had blue eyes, whereas other babies had normal iris pigmentation. Skin depigmentation was noted in 5 of the 6 patients. Three babies were seriously malnourished and showed higher association of enterocolitis. CONCLUSION: Shah-Waardenburg syndrome is an uncommon association of HD. Depigmentation with a white forelock and skin manifestations are common, whereas blue iris, long segment disease, and enterocolitis are present in nearly half of the patients.

Bladder polyp complicated with cystitis glandularis cystica.

A one year old boy presented with dribbling of urine since birth. Ultrasonography showed bilateral hydronephrosis and hydroureter suggestive of posterior urethral valves. Micturating cystourethrogram and cystoscopy was not possible due to obstruction at bladder neck. Vesicostomy was initially performed to relieve the obstruction. Child presented again at the age of 3 years whence MCUG suggested a filling defect in the bladder, confirmed as a mass on cystoscopy. Exploration revealed a 3 x 4 cm polypoidal mass arising from the bladder neck near verumontanum. Excision of the mass was easy due to narrow pedicle. Histology suggested it to be a large fibroepithelial polyp complicated by cystitis glandularis cystica. Child had uneventful recovery and is symptoms-free 6 months after surgery.

Meckel's diverticulum causing exsanguinating haemorrhage.

A seven month old male baby presented to emergency room in shock. Patient had 3 hours history of massive fresh bleeding per rectum. Emergency laparotomy revealed Meckel's diverticulum (MD) with large vitello-intestinal artery responsible for massive bleeding. Diverticulectomy was performed.