Jujju Jacob Kurian

Clinical profile and management options of children with congenital esophageal stenosis: A single center experience

Aim: The aim of the study is to review 7 patients with congenital esophageal stenosis treated in our institution from a diagnostic and therapeutic point of view. Materials and Methods: This is a retrospective cohort study of 7 patients treated in Christian Medical College, Vellore from 2008 to 2014. The data were analyzed with regards to age at onset of symptoms, investigative findings, age at definitive treatment, pathology, modalities of treatment, and outcomes. Results: Symptoms started within the 1 st year of life in all children with a median age of 4 months. The time of delay in diagnosis ranged from 8 months to 81 months with a mean period of 37 months. About 6 patients had a lower esophageal stenosis and 1 patient had a mid-esophageal stenosis. About 4 of the 7 children underwent endoscopic balloon dilatation from elsewhere, with 2 of the above 4 undergoing a myotomy for a wrongly diagnosed achalasia. The number of dilatations ranged from 2 to 7 with a mean of 4 dilatations. Resection of the stenotic segment with end to end anastomosis was employed in 6 of the 7 patients, and a transverse colon interpositioning was done in 1 patient. An antireflux procedure was performed in one patient. Histopathological examination of the resected specimen revealed tracheobronchial remnant in 3 patients, fibromuscular thickening in 3 patients, and membranous web in 1 patient. Postoperatively, 2 of the 7 patients had asymptomatic gastroesophageal reflux and 1 patient had postoperative stricture requiring one session of endoscopic balloon dilatation. The mean follow-up period was 42 months (range 18-72 months). At the time of the last follow-up, all 7 patients were able to eat solid food, and none of the children were found to have symptoms suggestive of obstruction or gastroesophageal reflux. There was a statistically significant increase in the weight for age after the operation. Conclusion: Congenital esophageal stenosis is rare and often confused with other causes of esophageal obstruction. Although endoscopic balloon dilatation offers an effective temporary relief, we feel that definitive surgery is curative. Long-term results following definitive surgery have been good, especially with respect to symptoms and weight gain.

A rare case of bilateral cystic nephroma associated with embryonal rhabdomyosarcoma of the penile urethra

Bilateral cystic nephroma is an extremely rare benign renal neoplasm. Here we present a case of bilateral cystic nephroma in a four month old boy who subsequently developed embryonal rhabdomyosarcoma of the urethra. Both tumors were successfully treated. To our knowledge this is the first reported case of this association which could be related to Dicer-1 mutation.

A rare case of bilateral cystic partially differentiated nephroblastoma recurring as bilateral cystic Wilms tumour

Childhood cystic partially differentiated nephroblastoma (CPDN) is an uncommon renal neoplasm. Bilateral CPDN or CPDN co-existing with a cystic nephroma/Wilms tumour is extremely rare. Treatment of CPDN is by complete surgical excision. Although local recurrences are uncommon, distant metastases have not been described. We present a case of bilateral CPDN that, after complete excision, recurred as bilateral cystic Wilms tumour. To the best of our knowledge, this is the first reported case in the literature where a bilateral CPDN has recurred as bilateral Wilms tumour.

A rare case of kaposiform hemangioendothelioma presenting as intussusception in a 4-month-old child without Kasabach-Merrit syndrome: A case report

We report the identification of Kaposiform hemangioendothelioma of the cecum presenting as colocolic intussusception in a 4-month-old boy without Kasabach–Merrit syndrome. To our knowledge this is the first reported case in the literature of such a presentation.

Outcome and renal function following salvage surgery for bilateral Wilms tumor: a single-institution experience

Objective The aim of this study was to determine the surgical outcomes and renal function following salvage surgery for bilateral Wilms tumor (BWT). Summary background data The challenge for the surgeon treating BWT lies in striking a fine balance between renal preservation and oncological clearance. Methods This is a retrospective review of medical records in a tertiary care hospital in India. Nine children with BWT who presented between 2005 and 2015 were reviewed and followed up through telephone. Survival rates were calculated using the Kaplan–Meier method. A P value of less than 0.05 was considered statistically significant. Results Seven (78%) of nine children were boys and two (22%) were syndromic. Six (67%) children presented at less than 1 year of age. Eight (89%) children presented with an abdominal mass. There were no metastases at presentation. All children underwent trucut biopsy and neoadjuvant chemotherapy. Six children underwent surgery: four underwent bilateral nephron sparing surgery (NSS) and two underwent unilateral nephrectomy with contralateral NSS. Tumor recurred in two children. The mean follow-up was 38 months (range: 5–108 months). Creatinine clearance (CrCl) improved postoperatively in all children. Postoperative hypertension was transient and resolved with improvement in CrCl. Conclusion Children with BWT in the Indian subcontinent may be younger than those in the rest of the world. NSS yields good outcomes even for recurrences. Postoperative hypertension is transient in the majority of patients and correlated with improvement in CrCl. Prognosis is related to operability and syndromic association.

The use of pedicled prepucial skin flap urethroplasty for proximal bulbomembraneous urethral stricture in children: an easy alternative to transpubic urethroplasty

Objective Pediatric urethral strictures are not uncommon, and a myriad of treatment options is available. The use of pedicled prepucial skin to augment a narrowed urethra is one of the useful methods. In this study, we describe the successful use of this technique in three children with stricture of the proximal bulbomembranous urethra. Materials and methods In this study, we reviewed three children aged 4, 7, and 10 years, respectively, with proximal bulbomembranous urethral stricture, who were treated at Christian Medical College, Vellore, India, between 2012 and 2014, using a pedicled prepucial skin flap. Results The mean follow-up time was 28 months (range: 20–41 months). There were no intraoperative or postoperative complications. All three children were symptom-free with a good urinary stream at last follow-up. Conclusion The pedicled prepucial flap technique for proximal bulbomembranous urethral stricture in children is simple and easy to perform with good overall outcome.

Idiopathic female pseudohermaphroditism with urethral duplication and female hypospadias

Female hypospadias is a rare anomaly of the female urethra where it opens on the anterior vaginal wall anywhere between the introitus and the fornix. It is often associated with other genitourinary anomalies such as Cloacal malformation, female pseudohermaphroditism, nonneurogenic neurogenic bladder and urethral duplication. Idiopathic female pseudohermaphroditism is extremely rare, and most cases occur secondary to adrenogenital syndrome or maternal androgen exposure. We report a unique case of a 1-year and 4-month-old girl who presented with ambiguous genitalia and renal failure secondary to a non-neurogenic neurogenic bladder. On further evaluation, she was found to have urethral duplication with a hypospadiac female urethra. She initially underwent a vesicostomy and was further planned to undergo an appendicular Mitrofanoff at an older age. The mainstay of treatment in these cases includes relief of bladder outlet obstruction and recovery of renal function by adequate urinary drainage. Clitoral reduction, if cosmetically warranted, may be planned at puberty.

A rare case of urethral triplication in association with tethered cord and vertebral anomalies

In this manuscript, we describe the case of a 5-year-old boy with Y triplication of the urethra in association with fatty filum terminale, tethered cord and vertebral anomalies. Unlike a Y duplication in which the penile meatus is hypoplastic, a Y triplication can have a penile meatus of adequate calibre, which can be used as a functional conduit. Most patients with urethral triplication have been found to have associated malformations. To our knowledge, this is the first reported case of urethral triplication seen in association with fatty filum terminale, tethered cord and vertebral anomalies.

Use of congenital pouch colon for augmenting the neurogenic bladder in a child: a 13-year follow-up.

Congenital pouch colon is an anomaly always associated with anorectal malformation, where the colon is replaced by or terminates into a large aperistaltic intestinal pouch. Vertebral anomalies leading to neurogenic bladder are rare associated malformations. The pouch is aperistaltic and thus a poor rectal substitute but this very property makes it ideal for bladder augmentation. We report the first case where the pouch has been used to augment a high-pressure neurogenic bladder.

Giant juvenile xanthogranuloma of the tongue presenting from birth in a 1-month-old infant: a case report

Juvenile xanthogranuloma is a rare benign proliferative lesion of the non-Langerhans cell histiocytic group. Extracutaneous involvement is extremely rare. We present a case of a giant juvenile xanthogranuloma involving the tongue from birth.