Primary obstructive megaureter in children; 10 years' experience from a tertiary care center.

Introduction: Primary obstructive megaureter (POM) is a congenital dilatation of the ureter due to an adynamic segment of vesicoureteric junction obstruction. Surgical intervention is needed if nuclear scan shows obstructive curve. We analyzed our data and outcome of conservative and surgical treatment in such cases at our tertiary care hospital. Materials and Methods: We evaluated all cases of POMs during the study period. Investigations included ultrasonography (USG), voiding cystourethrogram, diethylene pentacetic acid (DTPA) scan, and dimercaptosuccinyle acid scan. In antenatal cases, any pelvic dilatation ≥12 mm after 6 weeks were subjected to reonography. Patients with anterior-posterior pelvic diameter (APPD) ≥12 mm had to undergo DTPA scan to look for DRF and drainage. Follow-up USG was done in all cases of mild-to-moderate hydroureteronephrosis, with APPD <12 at 3 months interval. Results: A total of 270 megaureters were registered and treated during the study period (2008-2019). The total number of patients included was 50 (64 ureters). The mean age of presentation in these 30 children was 21.78 ± 18.1 months (range 1-72 months) and the mean weeks of gestation in antenatal cases at presentation as megaureter was 24 ± 7 weeks (range 13-37 weeks). The mean weight of babies was 2.72 ± 0.7 g. The duration of follow-up ranged from 16 to 1W12 months. The mean APPD on the affected side was 19.99 ± 10.3 mm (range 11-43 mm). The mean ureteric diameter was 1.67 ± 0.33 mm (range 0.78-2.66 cm). The mean split function of patients with POM was 34.88% ± 11.5% on the affected side. Twenty patients (40%) had spontaneous resolution over a mean time period of 24.1 ± 11.1 months. Thirty patients underwent surgical procedures. In three children, HTN was observed over a mean follow-up period of 3 years. Conclusion: The babies with POM need a close follow-up. Surgery is indicated in prolonged t½/Tmax on renal scan, function <40% at the initial scan, or >5% split function deterioration in the subsequent renal scan.

Comparison of laparoscopic and open surgery in hepatic hydatid disease in children: Feasibility, efficacy and safety.

Background: : Surgery continues to be the mainstay of treatment of hydatid cysts of the liver. Laparoscopy provides a lesser invasive tool for achieving results same as with the established open surgical techniques. The purpose of the study was to evaluate the feasibility and safety of laparoscopic management of hepatic hydatid disease in children. Patients and Methods: It was a prospective randomised study conducted over a period of 7 years. Children with Gharbi Type I, II, III cysts, ≤3 cysts and superficial accessible cysts were treated laparoscopically and their outcomes were compared with matched controls treated by open approach. Results: Sixty patients were included in the study with thirty patients in each matched group. Thirty paediatric patients (male 12:female 18) with 35 liver hydatid cysts underwent laparoscopic surgery. The mean cyst size was 8.8 ± 2.39 cm. Two patients needed conversion to open. No significant spillage of cyst contents was observed in any of the patients. Duration of hospital stay, time to removal of drains, duration of parenteral analgesia, severity of pain in postoperative period, time to ambulation and time to return to full orals were significantly lower in laparoscopic group compared to open group. Complication rates in both the groups were similar. Conclusion: With proper patient selection, laparoscopic management of hydatid cysts of the liver in children is feasible and safe option with low morbidity, low rates of conversion and minimal complications.

Bracka verses Byar's two-stage repair in proximal hypospadias associated with severe chordee: a randomized comparative study.

INTRODUCTION: Proximal hypospadias associated with severe chordee represents a major surgical challenge and the debate over its optimal treatment is ongoing. The objective of this study is to compare the outcome of two-stage Bracka and Byar's repair in proximal hypospadias. MATERIALS AND METHODS: This study was conducted from January 2013 to February 2018 in a tertiary care centre. Patients of hypospadias with severe chordee who required urethral plate transection were included in the study. Patients were randomly divided into two groups by simple randomization method. Bracka staged repair was done in Group A and Byar's staged repair in Group B. Postoperatively complications including graft loss, flap necrosis, fistula formation, meatal stenosis, stricture, diverticula formation, residual chordee were noted in both the groups and compared. p value of < 0.05 was considered statistically significant. RESULTS: Over a period of 5 years, 74 patients in group A and 68 patients in group B were operated. Fistula occurred in 6.8% and 10.2% in group A and group B, respectively (p value 0.629). Meatal stenosis was seen in 4% in group A and 3% in group B (p value 0.731). Stricture was seen in 1% in each group (p value 0.339). Diverticula formation was seen in 2% in group B and none of the patient in group A (p value 0.960). None of the patient had recurrence of chordee in either group. CONCLUSION: Bracka and Byar's two-stage repair have similar postoperative outcome and the choice between the two depends up on the surgeon's choice and experience rather than scientific evidence.

Disorders of Sex Development: A 10 Years Experience with 73 Cases from the Kashmir Valley.

PURPOSE: To present the clinical data, investigative profile, and management of patients with disorders of sex development (DSD) from the endocrine unit of a tertiary care university hospital. MATERIALS AND METHODS: This retrospective study included 73 cases of DSD, evaluated and managed at Department of Endocrinology, Sher-i-Kashmir Institute of Medical Sciences, Srinagar, Kashmir, over a period of 10 years from September 2008 to August 2018. RESULTS: Twenty-nine patients (39.7%) had 46 XY DSD and twenty-nine patients (39.7%) had 46 XX. Sex chromosome DSD was diagnosed in 15 (20.5%) patients. Of 29 patients with 46 XY DSD, 17 (58.6%) had 5α-reductase type-2 deficiency (5α-RD) and 6 (20.7%) had complete androgen insensitivity syndrome. In our patients with 5α-RD, the history of consanguinity was documented in nine (52.9%) patients. Two patients had testosterone biosynthetic defect and one patient had partial androgen insensitivity syndrome. Of 29 patients with 46 XX DSD, 16 (55.1%) had congenital adrenal hyperplasia (CAH). Of 15 patients with sex chromosome DSD, 7 patients had Turner's syndrome, 7 had Klinefelter's syndrome, and 1 patient had mixed gonadal dysgenesis. CONCLUSION: In our study, equal number of patients had 46 XY DSD and 46 XX DSD. We are for the first time reporting from India that the most common cause of 46 XY DSD is 5α-RD, whereas CAH is the most common cause of 46 XX DSD as reported previously.

Posterior Reversible Encephalopathy Syndrome after Augmentation Cystoplasty in a Child with Neurogenic Bladder.

Posterior reversible encephalopathy syndrome (PRES) or leukoencephalopathy syndrome was introduced into clinical practice in 1996 by Hinchey et al., to describe unique syndrome, clinically expressed during hypertensive and uremic encephalopathy, eclampsia, and immunosuppressive therapy. Hyperperfusion with resultant disruption of the blood-brain barrier results in vasogenic edema, but not infarction, most commonly in the parieto-occipital regions. The severity of this clinical symptom varies. For example, the visual disturbance can manifest as blurred vision, homonymous hemianopsia, or even cortical blindness. Patients may be mildly confused or agitated but can become comatose. Other symptoms less commonly seen include nausea, vomiting, seizures, and brainstem deficits. Chronic kidney disease (CKD) and acute kidney injury are both commonly present in patients with PRES. We are presenting a rare case of neurogenic bladder who developed PRES after augmentation cystoplasty due to underlying CKD.

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 Buck's 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 Buck's 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 Buck's fascia as intermediate layer and glanuloplasty, with excellent results. Buck's 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 Buck's 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 Buck's fascia group and 12.5% of patients in the dartos group, a statistically significant difference. We believe that the native Buck's fascia lateral to spongiosum is a more appropriate, natural, and strong layer to cover the neourethra. CONCLUSION: Use of Buck's 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 Buck's fascia as an intermediate layer to cover the neourethra to reduce incidence of postoperative complications and improve results.

Neonatal mastitis: a clinico-microbiological study.

OBJECTIVE: Neonatal breast hypertrophy is a common phenomenon in term infants, superadded infection can lead to mastitis and that can progress to breast abscess with short and long term detrimental effects. Our effort is to study the prevalence, risk factors, the current microbial profile and sensitivity pattern in these infections in order to suggest an optimal treatment plan for these patients. DESIGN: Case series. SETTING: Hospital based study conducted in Kashmir on the native population. DURATION: 2011 to 2013. MATERIALS AND METHODS: 32 neonates with features of mastitis or abscess were included in the study. Demographic and clinical data, laboratory work-up were recorded for all these patients in a patient form. Gram stain of the purulent nipple discharge or pus obtained on drainage was done and the specimens were culture plated. Antibiotic sensitivity was determined by disk diffusion and categorized by current Clinical and Laboratory Standards Institute (CLSI) guidelines. RESULTS: Most babies were full term, the age range was 6-48 days. Peak incidence for mastitis was in the 2nd week and for abscess in the 4th week. The ratio of male: female was 1:2 in the entire group, there was greater preponderance of female involvement with increasing age. Massage for expression of secretions a common practice in the study population had been done in 15 patients, especially in male babies. The babies were generally well and associated skin pustulosis was common. Laboratory workup showed polymorphonuclear leucocytosis and CRP positivity. Gram staining showed gram positive cocci in 13 patients and gram negative rods in 1 patient. Culture revealed Staphylococcus aureus in 18, E.col in 2, klebsiella in 1 patient and was sterile in 2 patients. Most strains of Staphylococcus aureus were resistant to macrolides and penicillins. Fifteen were methicillin sensitive and 3 were resistant but were sensitive to amikacin, ofloxacin and vancomycin. Gram negative rods were sensitive to, aminoglycosides, chloramphenicol, quinolones, piperacillin-tazobactum and cefoperazone-sulbactum, but were resistant to cephalosporins including third generation cephalosporins. Treatment with oral antibiotic was not successful. Patients responded well to open drainage via a stab incision away from the breast mound; 4 patients were managed by repeated needle aspirations. IV antibiotics were prescribed in all patients for 2-5 days, followed by oral continuation therapy of 7-14 days. CONCLUSION: From our study, we can conclude that parental counseling to avoid massage, and early treatment for pustulosis is important to prevent mastitis. Intravenous antibiotics should be used for this condition guided by gram stain or culture sensitivity once available. Empirically a drug with good anti-staph cover may be instituted till appropriate reports are available. Incision drainage gives uniformly good results, though; multiple sittings of needle drainage may obviate the need for incision drainage. Therapy can be shifted to oral drugs once clinical improvement is seen.

Role of preoperative chemotherapy in squamous cell carcinoma of esophagus in kashmir, a cancer belt - a pilot study.

BACKGROUND: Esophageal carcinoma is the fifth leading gastrointestinal malignancy and is one of the leading causes of cancer related death. Despite improvements in surgical technique over the last few decades, the outcome has been dismal, with overall 5 year survival not exceeding 15%-25%. AIMS AND OBJECTIVES: To evaluate the effect of preoperative chemotherapy on resectability, complication rate and overall survival in patients with squamous cell carcinoma esophagus. MATERIALS AND METHODS: 50 patients with histologically confirmed squamous cell carcinoma (SCC), with localised or loco-regional disease (stage 4 excluded) were divided into 2 groups. Group A patients were subjected to 2-3 cycles of pre-operative chemotherapy (5FU-CDDP), whereas Group B patients were directly operated on. OBSERVATIONS: 3 (12%) patients in group A showed complete pathological response to chemotherapy and 18 (72%) showed a partial response, with four patients (16%) showing resistance to chemotherapy. There was no statistically significant difference in terms of response to chemotherapy with respect to degree of differentiation of tumor. There was no significant difference in the overall resectability rates between the two groups (p > 0.05), but R0 resection was achieved in 20 (80%) of group A and only 10 (40%) of group B, the difference being statistically significant (p < 0.05). The rate of overall complications was also much higher in the control group. Initially there was no significant difference in the survival between the two groups, but later (20 months) the study group showed a slight non-significant advantage. CONCLUSION: Preoperative chemotherapy significantly increases the rate of R0 resection without significantly increasing postoperative morbidity and mortality in patients with squamous cell carcinoma of esophagus. However, to assess the impact on survival the study period needs to be extended.

Ascaris liver abscess in children.

BACKGROUND: Liver abscess in childhood is a very challenging subject both for patients and doctors. In India, pyogenic liver abscesses have been found mostly in children, especially in rural areas. Liver abscess due to wandering ascarids is a common complication in children in Kashmir, although it is rare outside. METHODS: The study was carried over a period of 10 years at Sheri-Kashmir Institute of Medical Sciences Srinagar India. Ten cases of liver abscesses due to wandering ascarids in children were encountered. The children were aged 2 years to 13 years. RESULTS: 70% of the children were from rural areas. All ten patients presented with fever, while three patients presented with peritonitis. Ultrasonography was the main tool of diagnosis. All ten patients were managed by open surgical drainage. CONCLUSION: We conclude that Ascaris infestation should always be kept in mind while dealing with liver abscess in children.