D. K. Gupta

Esophageal achalasia of unknown etiology in infants

BackgroundAchalasia cardia is an uncommon disease in children particularly in infants. We present 8 cases of achalasia who were encountered over a 12-year period. In infantile achalasia, respiratory symptoms predominate and vomiting may easily be mistaken for gastroesophageal reflux (GER). Vomiting of uncurdled milk is characteristic of achalasia.MethodsIn this retrospective study, the data were obtained from records of the Department of Pediatric Surgery IMS, BHU Varanasi, India. The patients were diagnosed by clinical examination and barium study. Any other associated anomalies were noted in these patients. The patients underwent esophagocardiomyotomy with antireflux procedure via the abdominal route.ResultsIn the present series, 7 patients survived. The follow-up study after operation showed remarkable relief of symptoms with satisfactory weight gain. No post-operative death occurred in the patients. Achalasia associated with alacrimia was noted in one infant who was lost to follow up. One infant was initially diagnosed as having gastric volvulus, but exploration revealed achalasia cardia.ConclusionsEsophageal achalasia is a rare disease in children and its origin is generally indeterminable. Achalasia cardia should be emphasized in the differential diagnosis of an infant presenting with signs and symptoms of esophageal obstruction.

Single system ectopic ureter in females: A single center study

The purpose of this study was to inquire into the clinical features and methods for the diagnosis and management of single-system ectopic ureters associated with renal dysplasia. Materials and Methods: A total of 13 female patients were studied. Main stay of diagnosis was ultrasonography of KUB region and intravenous urography and renal scan was used to confirm the diagnosis. Histopathological evaluation was done in all cases for documentation of renal dysplasia. Result: In eight cases ectopic ureter with dysplastic kidney was seen on left side and in five it was on right side. All the patients were treated with nephroureterectomy of the affected side because of poor functioning of ipsilateral dysplastic kidney. Conclusion: Continuous urinary incontinence in females with a normal voiding pattern should prompt an evaluation for ureteric ectopia and when initial evaluation yields diagnosis of solitary kidney the clinician should be aware of the possibility of a hypoplastic and/or dysplastic on one side and normally functioning kidney on opposite side. Nephroureterectomy is the treatment of choice for unilateral single system ectopic ureter with renal dysplasia of affected side.

An unusual cause of hilar lymphadenopathy

Allergic bronchopulmonary aspergillosis is an uncommon condition characterized by hypersensitivity to aspergillus antigen. It commonly presents as refractory asthma, fleeting pulmonary opacities and bronchiectasis. The authors describe an unusual presentation of allergic bronchopulmonary aspergillosis, when it was mistaken as bronchogenic carcinoma and review the current literature on the diagnosis and management of this rare condition.

Split notochord syndrome associated with dorsal neuroenteric fistula: A rare entity.

Split notochord syndrome (SNS) is an extremely rare congenital malformation associated with anomalies of the vertebral column, gastrointestinal tract and central nervous system. Twenty cases of SNS associated with dorsal enteric fistula have been reported in literature till date. The present report describes a unique case of SNS associated with lumbosacral meningomyelocele, dorsal neuroenteric fistula and dorsal herniation of right kidney along with vessels. The neonate was well managed by excision of enteric fistula, closure of duramater of meningomyelocele and repair of posterior wall hernia after placement of kidney in renal fossa. This kind of entity is uncommon and not been reported earlier.

Single-Stage Repair versus Traditional Repair of High Anorectal Malformations, Functional Results’ Correlation with Kelly’s Score and Postoperative Magnetic Resonance Imaging Findings

IntroductionPosterior sagittal anorectoplasty (PSARP) is the standard treatment for anorectal malformations. In the present study, the clinical evaluation of anal continence was carried out using Kelly’s scoring system and the results of primary PSARP or abdomino-PSARP were compared with the traditional three-stage procedure and the functional outcome was correlated with the findings of MRI, which was used as an objective method of evaluation. Patients and methodsA total of 40 patients with intermediate and high anorectal malformations were studied and were divided into two groups on the basis of a random number table. The patients in group A were treated with a single-stage operation, whereas the patients in group B were treated with a standard staged operation (either PSARP or abdominoperineal pull-through). After clinical evaluation using the Kelly score, patients were divided into three clinical groups irrespective of whether they were operated in one stage or in three stages. All patients were subjected to MRI at the age of 3 years and the findings were correlated with the clinical scoring system. ResultPatients were categorized according to their Kelly’s scores as follows: group 1: clinically good (score 5–6); group 2: clinically fair (score 3–4); and group 3: clinically poor (score 0–2). The proportions of good development of the muscles (puborectalis, external sphincter muscle, and levator muscle hammock) were 78.9% in group 1, 40% in group 2, and none in group 3. Development of muscles was found to be a significant factor for anal continence. Other significant factors for anal continence are rectal diameter and anorectal angle. ConclusionClinical assessment using the Kelly score was similar for the single-stage operation and the staged procedure, and this was supported by MRI findings. Therefore, we recommend the single-stage procedure to achieve a better outcome in intermediate and high anorectal malformation.

Unusual content of omphalocele: a congenital mature cystic teratoma of falciform ligament of the liver

Omphalocele is a congenital midline abdominal wall defect that results in herniation of intraabdominal contents covered by a lining of peritoneum and amnion. The severity of the defect often ranges from a minor herniation of the umbilical cord to a significant protrusion that includes large proportions of intestine and liver. We report unusual content of omphalocele that is congenital mature cystic teratoma of falciform ligament of liver which was confirmed by histopathology. This is a very uncommon entity and not described in world literature.

Chronic bilious vomiting in children in developing countries due to high bowel obstruction: not always malrotation or tuberculosis

BackgroundBilious vomiting, in conjunction with abdominal pain is considered to be a surgical problem, unless proved otherwise. In children, besides tuberculosis (TB), we have found jejunal stricture (JS) due to non-specific jejunoileitis (NSJI) to be an important cause of chronic high small bowel obstruction and bilious vomiting.Materials and methodsIn this retrospective study, the records of all children with complaint of intermittent bilious vomiting and failure to thrive were evaluated. Investigations included oral contrast study, ultrasound abdomen, chest X-ray and Mantoux test. Final confirmation was made at laparotomy. Treatment included jejuno-jejunal resection and anastomosis. Histopathology of the specimen was done to look for caseation, granuloma formation and other details.ResultsOut of total 100 patients with the complaint of bilious vomiting, 25 were having JS. Radiologic confirmation was possible in 19 (76%) patients of JS. No patient had evidence of TB as per our protocol. Histopathology revealed non-specific ischemic changes in all specimens.ConclusionJejunal stricture due to NSJI is a common entity in our setup leading to bilious vomiting. Contrast study can provide high index of suspicion in most of the patients. The diagnosis must be confirmed after proper histopathological examination. The results of the surgery are excellent.

Atresia of ileocecal junction, ileocecal valve: Rare variants of bowel atresia

Atresia of ileocecal junction and isolated atresia of ileocecal valve are rare types of intestinal atresia with very few reports in literature. We report two such cases. Radiology showed dilated ileal segment and distal micro colon in both the cases. At laparotomy there was atresia of ileocecal junction in the first case and isolated ileocaecal valve atresia with normal ileocecal junction in the other case. Both the babies were managed by ileocolic resection with an end to end anastomosis. The prognosis of ileocecal atresias is satisfactory.

Juvenile polyposis syndrome.

Aim: Report of a series of 12 cases of juvenile polyposis coli. Methods: The study period was from 1995 to 2005. All the patients were treated by total colectomy with rectal mucosectomy and endorectal ileoanal pullthrough with or without ileal pouch formation. Covering ileostomy was avoided in all the cases. Time taken for the surgery, postoperative complications and continence were documented. Results: The mean operating time was 4.2 h (range: 4–5 h). The mean duration of hospital stay was 16.3 days (range: 15–18 days). The most common postoperative complication was pouchitis and perianal excoriation. Initially, all the patients were passing stools at an interval of 2 h, and after 3 weeks, the frequency has reduced to 6–8 stools per day. In the follow-up after 3 months, the frequency was 3–5 per day with minimal soiling. Conclusions: Single-stage total colectomy with rectal mucosectomy and endorectal ileoanal pull-through without covering ileostomy and pouch formation is a safe and definitive treatment for juvenile polyposis coli if the patient selection is appropriate.

Mid-ureteric obstruction due to diaphragmatic valve: Case report with review of literature

Congenital ureteric obstruction caused by a ureteric valve is an extremely rare entity. Our patient, a 6-year-old male, had undergone evaluation for recurrent pain in the left lower part of abdomen and was diagnosed as a case of left hydrouretronehrosis on ultrasound abdomen. Intravenous urography revealed left hydronephrosis and hydroureter with a cut-off in mid –ureteric region. Micturating cysto-urethrogram (MCUG) and cystoscopy were normal. Laparotomy with excision of valve with end to end uretro-uretrostomy was performed. Histopathology revealed Type II ureteric valve. A high index of suspicion is required to make a correct preoperative diagnosis.