Aejaz A Baba

Recurrent malignant juxtaglomerular cell tumor: A rare cause of malignant hypertension in a child.

A juxtaglomerular cell tumor or reninoma is a very rare renin-secreting tumor of the kidney and can be an unusual cause of secondary hypertension. We report a case of recurrence of this uncommon tumor at the hilum of left kidney in an 8-year-old male child.

Undesended testis: How extensive should the work up be?

AIM The aim of this study was to highlight various anomalies associated with undescended testis and to determine how much work up is necessary for this condition. MATERIAL AND METHODS The study was conducted in the department of Pediatric Surgery SKIMS Srinagar, Kashmir. All patients between 0-14 years of age who attended out patient department (OPD) from January 2002 to December 2003 with maldescent of testes were included in the study. Detailed relevant history and physical examination findings were recorded in all the cases. Baseline investigations were performed along with ultrasonography of the abdomen. In relevant cases other investigations like intravenous urography, micturating cystourethrography, CT scan and laparoscopy were performed as and when indicated. RESULTS A total of 250 cases of undescended testis were registered during this period. Maximum number of cases were in the age group of 5-10 years. In 130 (52%) cases the right testis was undescended while 75 (30%) had left sided undescended testis and 45 (18%) had bilateral undescended testis. Maldescended testis comprised 11% of the admissions. The majority of cases were having gestational age of 37 weeks or more. The associated anomalies picked up on investigations included duplication of upper urinary tract (3.2%), hydronephrosis and polycystic kidney (0.8% each), horseshoe kidney, ectopic kidney, crossed renal ectopia (0.4% each) Posterior urethral valves, Prune belly syndrome (0.4%) and spina bifida (0.4%). On detailed clinical examination of genitalia several abnormalities were picked which included hydrocele, hypospadias, hernia, chordee, micropenis and ambiguous genitalia. CONCLUSION We recommend ultrasonography to be done in all cases of undescended testis in addition to a thorough history and physical examination. Intravenous pyelography, micturating cystourethrogram, CT scan and other investigations should be performed selectively based on history, physical examination or ultrasound findings.

Post ventriculoperitoneal shunt abdominal pseudocyst: Challenges posed in management

Background: In patients with hydrocephalus, the abdominal cavity has been used for absorption of cerebrospinal fluid (CSF) since 1905. Ventriculoperitoneal (VP) shunt operation is followed by abdominal complications in about 5-47% cases. Abdominal CSF pseudo cyst is an uncommon, but well described complication. Aim: This survey was conducted to study the clinical profile and management of this entity. We present our experience with cases of CSF pseudo cyst in children. Materials and Methods: Retrospective analysis of 4 cases diagnosed to have abdominal pseudo cyst following VP shunt between 2008 and 2013. All the four cases were suspected clinically and diagnosis was confirmed by abdominal ultrasonography. Results: In three patients, the cyst was multilocular and of varying size. Fourth one had a unilocular cyst at the lower end of VP shunt. All the four patients had features of varying degree raised intracranial pressure and a two patients had abdominal signs also. All the patients needed open exploration. Cyst fluid was drained and partial to complete excision of the cyst was done along with the repositioning of the shunt in abdominal cavity in three patients and exteriorization of shunt in one patient. Patients were followed for any further complication over a period of 1-year. Conclusion: Abdominal pseudo cyst is a rare complication after VP Shunt and could result in shunt malfunction or abdominal symptoms and signs. Whenever suspected it should be confirmed by imaging, followed by open exploration and repositioning of the shunt.

Solitary crossed ectopia with vesicoureteric junction obstruction: A rare case report

Solitary crossed renal ectopia (SCRE) is a very rare anomaly of urinary tract. Most cases are diagnosed incidentally. We report a case of SCRE associated with vesicoureteric junction obstruction in a 7-year-old child which has not been reported in the literature till date.

Late-presenting congenital diaphragmatic hernia

Background: This study was undertaken to highlight the clinical profile, misdiagnosis, surgical treatment,and prognosis of late-presenting congenital diaphragmatic hernia (CDH) cases in a tertiary level hospital. Patients and Methods: This retrospective study included all the babies and children >1 month of age with CDH who were admitted in our Hospital (Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Kashmir, India) during the period between January 2008 and December 2013. Babies with age <1 month were excluded from the study. Data regarding clinical profile, operative records, and follow-up was reviewed and analysed statistically. Results: A total of 20 patients were included in this study. The clinical picture ranged from respiratory distress (13 patients) to non-specific gastrointestinal complaints (5 patients). In two patients, CDH was misdiagnosed as pneumothorax and had got chest tube inserted in other hospitals before referral to this tertiary care centre. In 14 patients chest, X-ray revealed the diagnosis of CDH and in remaining five patients (including the two patients with misdiagnosis) further investigations were undertaken to establish the diagnosis. Age ranged from 45 days to 17 years with an average age of 58.9 months. There were 12 male and 8 female patients. In all the 20 patients, surgical procedures were undertaken with the retrieval of herniated contents from the thoracic cavity and repair of the diaphragmatic defect. There was no mortality in our series. All the 20 patients were followed-up for a period ranging from 6 months to 5 years (median 3.1 years). Conclusions: Late-presenting CDH can have diverse clinical presentation. Late diagnosis and misdiagnosis can result in significant morbidity and potential mortality if these cases are not managed properly at an appropriate stage. Outcome is favourable if these patients are expeditiously identified and surgically repaired.

Prune belly syndrome with pouch colon and absent dermatome

Prune belly syndrome (PBS) is a rare congenital constellation of defects in pediatric surgical practice. Although anorectal anomalies have been reported in association with PBS, only few case of pouch colon with PBS has been reported. [1] In addition, our patient had deficient abdominal wall with absent dermatome in left upper quadrant, which has never been reported in the English literature. This association with abdominal wall deficiency and absent dermatome not only strengthens the theory of mesodermal arrest in the etiology of PBS but also points towards a defect in the ectodermal development.

Tunica vaginalis flap following 'Tubularised Incised Plate' urethroplasty to prevent urethrocutaneous fistulaa.

Introduction: Surgery for hypospadias has been continuously evolving, implying thereby that no single technique is perfect and suitable for all types of hypospadias. Snodgrass technique is presently the most common surgical procedure performed for hypospadias. Materials and Methods: We analysed the results of tunica vaginalis flap (TVF) as an additional cover to the tubularised incised plate (TIP) repair. Results: A total of 35 patients of hypospadias were repaired using TIP urethroplasty and TVF as a second layer. Mean age at the time of presentation was 6.63 ± 3.4 years. Post-operative complications namely wound infection, flap necrosis, scrotal haematoma, scrotal abscess, urethral fistula, meatal stenosis were recorded and analysed during follow-up period. Need for re-do surgery was considered as failure of the operative procedure. Out of 35 patients, 8 (22.85%) patients had proximal penile hypospadias and 27 (77.14%) patients had distal penile hypospadias. Mean post-operative follow-up was 24.53 months. During the follow-up complications noticed included wound infection (n = 2), urethrocutaneous fistula (n = 1) and meatal stenosis (n = 1). Wound infection was managed with appropriate antibiotics as per hospital policy/culture and sensitivity reports. Meatal stenosis responded to bougie dilatation/calibration during follow-up. Conclusion: To conclude, TVF as an additional cover is associated with an acceptable complication rate and good cosmetic results if performed with meticulous tissue handling

Comparative Study of Snodgrass and Mathieu’s Procedure for Primary Hypospadias Repair

Objective. Present study was undertaken to compare the results of two single stage hypospadias repairs, namely, Tubularized Incised Plate (TIP) repair and Mathieus repair. Methods. The study included 100 patients of distal penile hypospadias, from January, 2008 to January, 2013. After a detailed history, local examination was performed with reference to the site of meatus, shape of glans, and presence of chordee. TIP repair was performed in 52 patients and Mathieus repair in 48 patients. On follow-up, the patients were examined for the position of meatus, shape of meatus, urinary stream, urethrocutaneous fistula, and stricture formation. Results. The mean age of presentation was 6.2 ± 3.2 years (range 1.5–15years). The mean operative time was 63.7 ± 14.3 (45–90) minutes and 95.0 ± 19.1 (70–125) minutes in TIP and Mathieus repair, respectively.Complications after surgery were urethero cutaneous fistula in 3(5.76%) and 7 (14.5%), meatal stenosis in 3(5.33%) and 4(8.33%), wound infection in 19.2% and 8.3% cases in TIP repair and Mathieu repair, respectively. The shape of meatus was slit-like and vertically oriented in 48(92.3%) patients who had undergone TIP repair. Conclusion. The Snodgrass repair is significantly faster, with more natural cosmetic appearance of the meatus than the Mathieus repair.

Neonatal intestinal obstruction due to double jejunal web causing Windsock deformity

Windsock deformity (WD) is a rare anomaly. A case of double jejunal web with WD causing neonatal intestinal obstruction is being reported.

Buck's fascia repair with glanuloplasty in hypospadias surgery: A simple approach with excellent outcome

BACKGROUND Hypospadias is a common problem encountered in surgical practice and its repair is challenging because of various complications. Urethro-cutaneous fistula is the most common postoperative complication (incidence of 0-33%). Different types of intermediate protective layers have been used in attempts to decrease UCF formation; however, no single surgical technique is ideal. OBJECTIVE The aim of this study was to compare use of dartos fascia and Bucks fascia as intermediate layers in prevention of the formation of UCF. STUDY DESIGN This was a prospective, comparative study conducted over a period of 2 years from January 2014 to December 2015. Patients with primary hypospadias without or with mild ventral penile curvature were included in the study. Patients were categorised into two groups, A and B, with alternate patients assigned to each group. Patients in group A underwent Snodgrass repair with urethroplasty by two-layer subepithelial closure and dartos tissue as an intermediate layer. Patients in group B underwent a urethral repair followed by Bucks fascia repair as intermediate layer and glanuloplasty after excision of a triangular skin strip on either side of the urethral plate. Patients were followed at regular intervals for a minimum of 6 months and complications were noted. RESULTS Over a period of 2 years, 160 patients were included in the study: 80 patients in group A and 80 patients in group B. The age of patients ranged from 1 year to 4.6 years, with a mean age of 1.8 years. Postoperative complications are listed in the summary table. DISCUSSION A protective intermediate layer between the neourethra and the skin can be used to reduce fistula formation. We describe a technique of urethroplasty using Bucks fascia as intermediate layer and glanuloplasty, with excellent results. Bucks fascia over the corpora spongiosum which is deficient ventrally in hypospadias is not completely absent, and can be easily used to cover the neourethra, needs minimal dissection and hence vascularity of tissues is preserved (summary Fig.). We used this Bucks fascia as a second protective layer over the neourethra in an attempt to decrease UCF formation, and compared it with use of dartos fascia. In our study, UCF occurred in 2.5% of patients in the Bucks fascia group and 12.5% of patients in the dartos group, a statistically significant difference. We believe that the native Bucks fascia lateral to spongiosum is a more appropriate, natural, and strong layer to cover the neourethra. CONCLUSION Use of Bucks fascia as an intermediate layer along with glanuloplasty is simple and very effective in preventing UCF formation and glanular dehiscence. We recommend the use of Bucks fascia as an intermediate layer to cover the neourethra to reduce incidence of postoperative complications and improve results.