Satyendra Narayan Singh

Application of a plain abdominal radiograph transition zone (PARTZ) in Hirschsprung's disease

BackgroundA standard contrast enema for Hirschsprungs disease can sometimes be inconclusive in delineating a transition zone especially in neonates and infants. The aim of this study was to determine the utility and diagnostic accuracy of a plain abdominal radiograph transition zone (PARTZ) in predicting the level of aganglionosis.MethodsA prospective observational study of neonates and infants with biopsy proven Hirschsprungs disease was carried out from March 2004 through March 2006. All patients underwent a plain abdominal radiograph and a contrast enema followed by a rectal biopsy. The transition zone on a plain radiograph (PARTZ) and contrast enema (CETZ) were compared with operative and pathology reports. Results were analyzed by chi square test and expressed as their p values and 95% confidence intervals.ResultsPARTZ and CETZ suggestive of Hirschsprungs disease was seen in 24(89%) and 18(67%) patients respectively. The PARTZ and CETZ matched with the pathologic level of transition zone in 22(92%) and 13(72%) patients, p = 0.001, 95% CI (-1.87 to -0.79). In the 9 (33%) patients in whom contrast enema failed to reveal a transition zone, PARTZ was seen in 6/9(66%) patients and correlated with the pathological level of aganglionosis in 4/6(67%) patients, p = 0.001 95% CI (-1.87 to -0.79). The overall accuracy of PARTZ and CETZ was 96% and 84% respectively, p = 0.008, 95% CI (-6.09 to -3.6).ConclusionA plain abdominal radiographic transition zone is reliable in predicting the level of transition zone in cases of inconclusive contrast enema. It may be particularly helpful developing countries where laparoscopic techniques are not available to accurately identify the transition zone.

Sphincter saving anorectoplasty (SSARP) for the reconstruction of Anorectal malformations

BackgroundThis report describes a new technique of sphincter saving anorectoplasty (SSARP) for the repair of anorectal malformations (ARM).MethodsTwenty six males with high ARM were treated with SSARP. Preoperative localization of the center of the muscle complex is facilitated using real time sonography and computed tomography. A soft guide wire is inserted under image control which serves as the route for final pull through of bowel. The operative technique consists of a subcoccygeal approach to dissect the blind rectal pouch. The separation of the rectum from the fistulous communication followed by pull through of the bowel is performed through the same incision. The skin or the levators in the midline posteriorly are not divided. Postoperative anorectal function as assessed by clinical Wingspread scoring was judged as excellent, good, fair and poor. Older patients were examined for sensations of touch, pain, heat and cold in the circumanal skin and the perineum. Electromyography (EMG) was done to assess preoperative and postoperative integrity of external anal sphincter (EAS).ResultsThe patients were separated in 2 groups. The first group, Group I (n = 10), were newborns in whom SSARP was performed as a primary procedure. The second group, Group II (n = 16), were children who underwent an initial colostomy followed by delayed SSARP. There were no operative complications. The follow up ranged from 4 months to 18 months. Group I patients have symmetric anal contraction to stimulation and strong squeeze on digital rectal examination with an average number of bowel movements per day was 3–5. In group II the rate of excellent and good scores was 81% (13/16). All patients have an appropriate size anus and regular bowel actions. There has been no rectal prolapse, or anal stricture. EAS activity and perineal proprioception were preserved postoperatively. Follow up computed tomogram showed central placement the pull through bowel in between the muscle complex.ConclusionThe technique of SSARP allows safe and anatomical reconstruction in a significant proportion of patients with ARMs without the need to divide the levator plate and muscle complex. It preserves all the components contributing to superior faecal continence, and avoids the potential complications associated with the open posterior sagittal approach.

Delayed presentation of congenital pulmonary airway malformation type 4 mimicking lobar emphysema.

© 2008 Japan Pediatric Society Congenital pulmonary airway malformation (CPAM), previously termed ‘congenital cystadenomatoid malformation’, is a rare but potentially life-threatening pulmonary anomaly. 1 Approximately half to two-thirds of children with CPAM present with some form of respiratory distress in the neonatal period. 2 The remainder are discovered incidentally or present with chest infections generally before the age of 6 years. The diagnosis of CPAM in the older group can be confusing because the radiological and histologic features of the mass are often altered by hyperinfl ation, pneumothorax or superimposed infection. 3,4 Although computed tomography (CT) is helpful in these cases for diagnosis and delineating the extent of the lesion, 5 we report a child with CPAM that masqueraded as lobar emphysema on chest X-ray (CXR) and CT scan.

One-stage Correction of Rectovestibular Fistula by Transfistula Anorectoplasty (TFARP)

BackgroundThe present article details a new technique for the repair of rectovestibular fistula.Materials and MethodsTwenty-five patients with rectovestibular fistula, between 13 days and 4 years of age underwent surgical correction by transfistula anorectoplasty (TFARP). The technique, described in detail, involves mobilization of the fistula and the rectum through the fistula and creation of a new anus in the anatomically normal site by preserving both the perineal skin bridge (skin between the neo-anus and the posterior fourchette) and the levator muscle.ResultsThe mean operating time was 85 min, and the mean hospital stay was 5 days. Moderate anal stenosis developed in 1 patient and was treated successfully by anal dilatations using Hegar dilators. A diverting colostomy was not required in any patient, and none of the patients developed rectal prolapse. Eleven patients who are now 3 years of age or older have voluntary bowel movements with good fecal continence scores. The 14 neonates and infants, who are still too young to be evaluated for continence, have symmetric anal contraction to stimulation and strong squeeze on digital rectal examination. The average number of bowel movements per day was three to five, without the need for any laxative or enema.ConclusionsTransfistula anorectoplasty is a simple surgical procedure that does not divide the levator muscle or the perineal body. Preservation of these structures contributes significantly toward improvement of the aesthetic appearance of the perineum and of fecal continence.

Giant retroperitoneal fetus-in-fetu: An unusual cause of respiratory distress

Fetus-in-fetu (FIF) is a rare congenital condition in which a vertebrate fetus is incorporated within its host, a newborn or an infant. We report an interesting case of FIF presenting as an abdominal mass with respiratory distress in a 4-month-old girl, and review the existing literature. An ultrasound abdomen showed a right sided cystic mass containing multiple calcifications. Laparotomy revealed a well-encapsulated right retroperitoneal mass above the right kidney with two rudimentary limbs attached to an amniotic bag by an umbilical cord. Excision of the capsule revealed a yellowish fluid and an incompletely developed fetus covered by vernix caseosa. Pathologic examination showed an irregular fetiforme mass, weighing 1200 gm, with two rudimentary limbs, encephalus, stomach, duodenum, bowel, bone, cartilage, bone marrow, upper and lower respiratory tissue, and spleen. Complete excision of the FIF was performed. The postoperative period was uneventful and the patient was discharged on the seventh postoperative day.

Complex urethral disruptions: In pursuit of a successful reconstruction

Objectives:  We analyzed the methods and outcomes of urethroplasty in men with complex urethral disruptions.