Background/Purpose: Anorectal malformations are one of the most common congenital defects. This study was undertaken to study the hospital incidence of anorectal malformations (ARM), frequency of various types of defects, their sex distribution and the spectrum of anomalies associated with ARM. The effect of presence of an associated defect on mortality and morbidity was also studied. Materials and Methods: One hundred consecutive children attending the pediatric surgery department were included in this study. A detailed history was taken, and examination was performed for the primary as well as the associated defects. Appropriate investigations like invertogram, cologram were done wherever indicated. Management was as per the standard protocol. The data was recorded and analyzed. Results: Out of the 100 patients, 51 were males and 49 females. One out of every 6.62 admission was for ARM. Twenty percent of the female babies had high, 76% intermediate and 4% had low anomalies, whereas 80.39% males had high, 3.92% intermediate and 15.6% showed low malformations. Ten percent of the patients had pouch colon. Associated anomalies were seen in 33 patients - 20 males and 13 females; 19 in high, 10 in intermediate, 1 in low group and 3 children with cloacal malformations. Associated defects seen were urogenital (17%), cardiovascular (7%), gastrointestinal (9%), genital (5%) and limb defects (7%). There were 8 deaths, and complications were seen in 13 patients. Ten patients had two or more defects associated with ARM. Conclusions: Anorectal malformations occurred equally in males and females. Females had intermediate defects more frequently, rectovestibular fistula being the commonest. Males were more likely to have high lesions; anorectal agenesis without fistula was the commonest defect. The most common associated defects seen were vesicoureteric reflux and esophageal atresia. Complications were seen more commonly in males with high lesions. There was a significant association between presence of an associated defect and mortality and morbidity.

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A parapagus set of male conjoined twins was brought to our institution at 12 h after birth. An extensive sharing of the abdominal viscera (single liver, hindgut), abdominal aorta, pelvis (single rectum and anus), genitalia (one set) and vertebral column was found. The surgical separation was not considered due to medical and ethical issues.

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Congenital lobar emphysema is usually restricted to one lobe of a lung. We report a rare case of bilateral congenital lobar emphysema, where the right middle lobe and its equivalent on the left side (lingula) were affected. Newer modalities of imaging (like high resolution CT scan thorax) have made the simultaneous diagnosis of bilateral involvement possible, which otherwise could be missed in a plain chest radiograph.

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Ascariasis is highly endemic in Kashmir valley as temperate climate and wet soil provide excellent conditions for its development. We studied 198 cases (11%) of biliary ascariasis out of 1,800 patients of worm infestation from December 2001 to December 2004 in the age group of 4-15 years. In our study, we found biliary ascariasis to be more common in female children, and most common clinical presentation was biliary colic in 143 patients, cholangitis in 28, cholecystitis in 15, liver abscess 7, pancreatitis 4 and postoperative worm infestation in 1 patient. Ultrasonography of abdomen was the diagnostic tool of choice with hundred percent results. Most of the patients were managed conservatively. ERCP was not done in children because of need of general anesthesia and difficulty in performing the procedure. Surgical intervention was required in 23 patients (cholecystectomy with CBD exploration in 5, choledochotomy alone in 13, drainage of liver abscess in 3, choledochoduodenostomy in 1 and peritoneal lavage in 1).

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Exomphalos major is an inherited disorder characterized by defects in the central portion of abdomen. Because of major improvements in the survival of these patients in recent years, primary repair of abdominal wall and reconstruction are considered. Therefore, umbilicus position will be an important landmark for abdominoplasty. The aim of this study was to determine normal position of umbilicus in healthy Iranian neonates to improve cosmetic outcome of exomphalos major repair. In a cross-sectional study comprising 200 healthy Iranian neonates (107 boys and 93 girls), who were born in Esfahan, the position of the umbilicus was determined in all of them by measuring the distance of xiphisternum to pubis in midline and determining umbilical position in this line. Our findings showed that umbilical position was 59.28±5.2 percent off the way from the inferior border of xiphisternum to the superior border of the pubis in the midline, and it was independent of sex and neonatal growth indices.

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Isolated congenital penile urethrocutaneous fistula is described as an unusual developmental anomaly in two infants who presented with an abnormal opening on the ventral aspect of penis with a normal foreskin and an absence of the chordee, hypospadias or other associated congenital anomalies. Tubularized incised plate urethroplasty augmented with dartos pedicle blanket wrap resulted in satisfactory recovery. Authors review their surgical experience with this new indication of tubularized incised plate urethroplasty in successful surgical repair of congenital penile urethrocutaneous fistula along with pertinent literature.

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The two main surgical procedures for high and intermediate anorectal malformations (ARM), namely, Stephens' and Peρa's procedure, are compared in terms of their outcome. Materials and Methods: Fifty-eight patients who had Stephens' procedure and 28 patients who had posterior sagittal anorectoplasty (PSARP) are clinically analyzed in terms of associated anomalies, procedural complications, anatomical reconstruction and functional results. Functional results were assessed by Kelly score, voluntary bowel movements and sensation. Statistical analysis of data was done by Chi-square test. Results: There were 34 high and 52 intermediate ARM. Associated anomalies were noted in 32%. Procedure-related complications of urethral injury, bladder injury, neurogenic bladder, anal stenosis and mucosal prolapse were seen in both procedures. Ectopic positioning of anus was seen in 25% of PSARP and 19% of sacroperineal mobilization (SPM). Good circular sphincter creation was seen in 43% of PSARP and 40% of Stephens'. Noncontractile sphincter was found more with SPM. In functional results, when assessed by Kelly score, VBM and sensation, there was no difference for high ARM, whereas results were better with SPM for intermediate anomalies. Discussion: A few reports are available in literature comparing PSARP and SPM. Procedural complications of urethral injury and neurogenic bladder are slightly more with PSARP. Ectopic positioning, poor contraction of sphincter are associated with poor results, and creation of good circular sphincter with good squeeze is associated with good results. Functional assessment by Kelly score, VBM and sensation doesn't reveal any difference between two procedures for high ARM, whereas for intermediate anomalies, Stephens' procedure seems to give better functional results.

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Rectal duplication is described in a 1-month-old male infant who presented with constipation for the last 5 days. A presence of a cystic mass in relation to posterior wall of rectum revealed on clinical and radiological examination provided a clue to diagnosis. The clinical presentations and the management protocol of this rare entity are discussed. Authors review their experience with this rare entity and the pertinent literature.

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