Spinal and Vertebral Anomalies Associated with Anorectal Malformations

ObjectiveThe associations between imperforate anus and spinal and vertebral abnormalities and neurologic deficits are well recognized; these neurologic deficits have been considered static rather than progressive. However, recent experience indicates that some patients may develop progressive neurologic problems due to spinal cord lesions that are amenable to neurosurgical correction.Materials & MethodsThe medical records of 105 patients with imperforate anus, operated on by us, were retrospectively reviewed from 1996 to 2005. Patient's sex, anorectal type lesion and vertebral or spinal anomalies were determined by ultrasound, excretory urography, voiding cystouretherography (VCUG) and  lumbosacral x-ray.ResultsA hundred and five cases, consisting 48 (45.7%) boys and 57 (54.3%) girls, with anorectal malformations were studied; 70 patients were in high and intermediate type level, and totally 25 patients (35.7%) with spinal and vertebral anomalies were found in this group.ConclusionAll patients with anorectal malformations (ARM) should be investigated for spinal and vertebral anomalies to improve treatment outcomes in ARM.

LONG TERM FOLLOW-UP OF KASAI OPERATION FOR BILIARY ATRESIA: A SINGLE CENTER EXPERIENCE

Children who are operated for biliary atresia by the Kasai procedure have approximately 30% chance of survival for 5 years. In an attempt to define the role of this operation for biliary atresia, the surgery records of the past 15 years were reviewed. The aim of this study was to assess the benefits achieved from this operation in infants with biliary atresia. This study was conducted in the department of pediatric surgery Taleghanei Medical Center from 1986 to 2000. A total of 36 cases, 15 boys and 21 girls were reviewed retrospectively. All the operations were performed uniformly by Kasai procedure by three investigators. Data regarding patient history, clinical presentation, laboratory and radiological features, operative finding, complication and mortality were collected and retrospectively studied. In these series 36 cases were classified as three groups. Group A, represented the successful category after the Kasai operation (11 patients, 30.5%) characterized by survival of more than 3 years and no jaundice. Group B (2 patients, 5.5%) was defined as survival of more than 3 years, but with jaundice, and group C (23 patients 63.8%) was defined by survival of less than 3 years (this group was further divided to subgroups). It seems that jaundice is the main prognostic factor after operation

URETERO PELVIC JUNCTION OBSTRUCTION IN THE NEWBORN

Uretero pelvic junction (UPJ) obstruction is a common cause of hydronephrosis in infants. The frequent use of fetal ultrasound is allowing early (prenatal) diagnosis of numerous uropathies previously delayed until the child either became symptomatic or had a palpable mass. Newborn with severe obstruction often has marked improvement following correction, therefore, early diagnosis and operation are important. From 1993 to 2002, 21 patients were operated on for severe UPJ obstruction who were diagnosed before 6 weeks of age. Only 10 patients (63%) had antenatal ultrasonographic diagnosis, the remaining were diagnosed by postnatal ultrasound and IVP or radionuclide scan for palpable renal enlargement or for associated anomalies. Eighteen of them had unilateral and three had bilateral obstruction. Twenty-four pyeloplasties were done; all pyeloplasties were dismembered with tailoring of the dilatated renal pelvis. Postoperative renal function was followed with laboratory blood test, urine test or radionuclide scan or IVP. Postoperative complications included urinary tract infection in three patients and postoperative stenosis in one patient were seen. No mortality occurred on infants in unilateral but one occurred in bilateral obstruction. Also, there was one unrelated late death. We report documented functional improvement with minimal complications in unilateral or bilateral pyeloplasty in newborns with UPJ obstruction. We recommend that if the initial scan shows substantially reduced function in the obstructed kidney, a pyeloplasty (rather than nephrectomy) generally should be performed, because the newborn kidney has tremendous capacity for improvement in renal function following decompression