Vijay D. Upadhyaya

Noncommunicating isolated enteric duplication cyst in childhood

Duplications of the alimentary tract are spherical or tubular structures lined by epithelium similar to intestine that are firmly attached to or share the wall of the alimentary tract and have a common blood supply with the adjacent segment of the bowel. Completely isolated duplication cysts are an extremely rare variety of gastrointestinal duplications with their own exclusive blood supply and do not communicate with the intestine. There are only 4 cases reported in the English literature-3 in childhood and 1 in an adult, and all are male. We report a case of noncommunicating isolated ileal duplication cyst in a 3-year-old female child that presented as a mass in the right iliac fossa, mimicking an intussusception. The duplication had its own blood supply arising from the terminal ileal mesentery. This report describes the first female patient with this condition and reviews the English literature.

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.

Typhoid sigmoid colon perforation in an 18-month-old boy

BackgroundTyphoid fever is a severe infective disease endemic in the areas where sanitation is poor. Its serious complication is bowel perforation with terminal ileum as the commonest perforation site. We report a case of an extremely unusual site of perforation in sigmoid colon due to typhoid fever.MethodsAn 18-month-old boy presented with no passage of flatus and feces along with guarding and rigidity. Exploratory laparotomy revealed sigmoid colon perforation and Widal test was strongly positive for typhoid fever. Sigmoid loop colostomy was performed on the patient.ResultsPostoperative period was uneventful. Colostomy started functioning on the 4th post-operative day. There was superficial wound dehiscence. The patient was given oral food intake on the 7th post-operative day. He was discharged from the hospital in satisfactory conditions on the 10th day after operation.ConclusionsWhile tackling a case of enteric perforation suspected with typhoid fever, one should keep in mind the extremely rare site of sigmoid colon perforation.

Eviscerated urinary bladder via ruptured umbilical hernia: a rare occurrence

Umbilical hernia is a common problem encountered in children. Rupture and evisceration are very rare phenomena, and the usual content that is eviscerated is the bowel. We present an infant who had a ruptured umbilical hernia with eviscerated urinary bladder dome. As this is the first case of its kind, it is being reported with a brief review of literature.

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.

A child presented with bilateral congenital constriction ring in lower extremity: a case report

IntroductionThe congenital constriction ring of lower extremity is very uncommon and rare condition. The actual incidence in general population is not known. In English literature, very few cases are reported time to time as congenital constriction band syndrome associated with musculoskeletal disorder like congenital talipes equino varus. The lesion can involve skin only or goes to deeper structure up to bone, which can lead to gangrene of foot or auto amputation.Case presentationWe are presenting a case of bilateral congenital constriction ring in lower limb who presented at age of 4 year without any other associated congenital anomaly, simply managed by Z-plasty, which improves quality of life after physiotherapy.ConclusionCongenital constriction ring of lower limb is extremely rare condition in children. Early diagnosis and management is mandatory, either in single stage or by stage procedure, to prevent auto-amputation of limb and to improve quality of life on feet.

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.