"Wide Skeletonization" Tubularised Incised Plate (TIP) Repair of Distal Penile Hypospadias with Narrow Urethral Plate.

UNLABELLED: The importance of an adequate caliber neo-meatus for success of any hypospadias repair cannot be overstated. TIP repair incorporates a midline relaxing incision thus enabling tubularization but ultimately may not result in adequate neourethral caliber to avoid fistulas or meatal stenosis when the plate was narrow or flat, respectively. Objectives of this study is to evaluate results of a modification of TIP - "wide skeletonization" tubularized incised plate (TIP) - for repair of distal penile hypospadias with narrow urethral plate (UP), with regard to meatal stenosis and urethrocutaneous fistula. MATERIALS & METHODS: Total of 108 hypospadias patients were operated upon. 26 were found to have distal penile ypospadias with narrow UP (Narrow UP defined as any plate less than 6 mm width). 22 were selected for the study; All underwent "wide skeletonization" of the UP distally ("Wide Skeletonization" implies meatal based ventral "V" shaped incisions on outer margins of UP so as to incorporate generous portions of adjoining skin thereby allowing recruitment of penile and glandular skin lateral to the urethral plate to facilitate tubularization). Urethroplasty was fashioned over 10mm circumference of native UP with adjoining skin over a urethral stent. Repair was started with meatus first (calibrated over size 10-12F bougie). Meatal stitch was followed proximally with subepithelial tubularization of the neourethra; second layer comprising of spongiosal tissue. Barrier flaps were interposed between the neourethra and overlying glans and shaft skin closure, using inner prepucial mucosal flap. RESULTS: Two patients had urethrocutaneous fistulae; one child had superficial dehiscence of glans. All three children were managed conservatively with no child requiring further surgery. All children had a good sized meatus and good stream of urine without any splaying. CONCLUSIONS: "Wide Skeletonization" of the urethral plate distally (in a "V" fashion rather than "U") gives an adequate caliber aesthetic neo-meatus especially in hypospadias children with narrow UP. It further helps in decreasing incidence of neourethral and/or meatal stenosis without increasing urethroplasty complication leading to an excellent functional outcome. Whether this leads to better long term functionality of neourethra remains to be seen.

Cystic lymphangioma of the mesentery causing intestinal obstruction.

Mesenteric cystic lymphangioma is a rare lesion that is not often described in the literature. A four-year-old boy, who presented with abdominal distension and pain, is reported. At surgery, a huge mesenteric cyst was found to be the cause of the intestinal obstruction and was completely excised. Histology was consistent with a cystic lymphangioma. Abdominal lymphangioma is a rare cause of bowel obstruction. Clinical presentation varies and may be misleading due to a lack of awareness of the clinical condition. Occasionally, the diagnosis is made during surgery. General awareness of this entity with a high index of suspicion is needed to avoid complications.

Surgical complications and outcome of paediatric liver transplantation: the Singapore experience.

The purpose of this study was to analyse the early and late results of paediatric liver transplantation (LT), with particular reference to complications that required surgical intervention. The charts of all children who underwent LT between 1990 and 2002 were reviewed retrospectively. Results were analysed with a minimum follow up of 9 months. Thirty-five children have undergone 38 LTs; 22 received grafts from their parents, 16 received cadaveric organs and three children had retransplantation. The ages of the children ranged from 12 to 168 months. Biliary atresia was the most frequent indication for transplant (n=27). Twenty-seven children had complications that required surgical or radiological interventional procedures. Vascular complications included hepatic artery thrombosis (n=2), hepatic vein (HV) thrombosis (n=1), and the majority being portal vein thrombosis (n=6). Bile leaks were observed in eight children. Other complications included intestinal perforation (n=2), intra-abdominal abscesses (n=1), wound dehiscence (n=2), post-operative bleed (n=2), intestinal obstruction (n=2), ventral hernia (n=1), and multiple abdominal wound sinuses (n=1). Three children underwent retransplantation, two for hepatic artery thrombosis with multiple episodes of cholangitis and intrahepatic biliomas and the third was done for hepatic vein thrombosis. Patient and graft survival at 1 year is 81.5 and 74.2%, respectively. Paediatric LT is associated with significant morbidity, the main complications being vascular and biliary.

Management of ventral hernia after giant exomphalos with external pressure compression using helmet device.

PURPOSE: The aim of this study was to evaluate an alternative technique of reducing a ventral hernia that follows the primary conservative treatment of a giant omphalocoele. METHODS: The patient is a full-term male neonate with a giant exomphalos. Initially triple dye was applied as an eschar-inducing agent. This resulted in a ventral hernia after 1 month. It was decided to achieve expansion of the abdominal cavity based on the principle of external pressure compression using a sphygmomanometer cuff over the hernia. The cuff was worn continuously, and manual pressure was applied daily. Care was taken to avoid intraabdominal hypertension using the reading of the manometer that was attached. The external pressure was corroborated with observations of respiration and circulation. RESULTS: The child did not show any ill effects of raised intraabdominal pressure. Throughout the treatment, the child was on full oral feedings and did not require any ventilator support. Reduction of the ventral hernia was achieved in 9 months. Surgical repair of the residual hernia defect was carried out by double breasting of the fascia. CONCLUSIONS: The application of controlled external pressure using a specially constructed device is a safe, noninvasive, and effective method of achieving reduction of a ventral hernia after primary conservative treatment of a giant omphalocoele.

Urogenital sinus, rectovaginal fistula, and an anterior stenosed anus--another cloacal variant.

The persistent cloaca is one of the most complex and challenging developmental malformations. It is a rare anomaly occurring only in the female newborn and is represented by the association of urogenital sinus with an anorectal malformation (ARM). Each case is probably unique. We report here one such case of cloaca with the VATER association-tracheoesophageal fistula (TOF) with a urogenital sinus, rectovaginal fistula, and an anteposed stenosed anus, along with preaxial syndactyly of the right hand. The spine, renal, and cardiac systems were normal. Interim management was directed towards repair of the TOF and a right transverse defunctioning colostomy. Despite thorough radioendoscopic preoperative investigations, the complexity of the cloacal anomaly was not delineated until surgery. This case is a rather rare combination of an intermediate form of the cloacal-ARM spectrum. Such patients present with many diagnostic and therapeutic problems. Interval surgery should be directed towards decompression of the affected organ systems, and definitive surgery must be carefully planned and, whenever possible, done in a single stage with simultaneous multisystem repair to correct all significant malformations related to the cloacal complex.

Anorectal malformation with congenital absence of vagina: a case report and review of the literature.

Congenital anorectal malformations are relatively common, and associated genitourinary malformations have been reported in up to 40% of patients. Uterovaginal malformations are also not rare in children. They are known to occur with increased frequency in children with anorectal malformations, but the diagnosis may still be difficult. We report herein one such case of anorectal malformation associated with congenital total absence of the vagina in which the uterovaginal malformation was not diagnosed until the operative repair of the anorectal malformation. The operative procedure was thereafter directed, along with the anorectoplasty, towards restoring a functional uterovaginal tract. Review of the literature revealed that such a diagnosis is unsuspected or delayed in more than half of affected patients. Furthermore, these patients present with many diagnostic and therapeutic problems. Our report highlights the need to be aware of this condition to allow for an earlier diagnosis and appropriate operative treatment.

Bronchoplasty for bronchial stenosis in a neonate: a case report.

Bronchial stenosis is an uncommon problem in children. Management can be difficult because of the small luminal diameter and proximity of the lung parenchyma to the bronchial lesion. Bronchoplasty procedures have obvious advantages in children because of their long life expectancy. Long-term functional results are superior compared with pneumonectomy because of preserved lung tissue. A premature neonate weighing only 779 g at birth was born with severe respiratory distress syndrome complicated with hyperbillirubinemia and bronchopulmonary dysplasia. The child underwent bronchoplasty using a costal cartilage graft for a right main bronchus stenosis after 2 failed attempts at bronchoscopic dilatations. In spite of all complications, the child improved sufficiently to be discharged without any oxygen dependency. Our case highlights the successful application of bronchoplasty in a very small baby. Preoperative and intraoperative bronchoscopy was helpful in localization of the endobronchial lesion. Understanding the principle, precise attention to technical detail and meticulous postoperative care contribute toward long-term benefit for pulmonary conservation.

Meckel's diverticulum: an alternative conduit for the Mitrofanoff procedure.

The Mitrofanoff procedure is a versatile technique that is successfully used in achieving continent urinary diversion for a wide variety of urological conditions. Appendix and usable segment of ureter are commonly employed for this purpose and provide desirable results. This communication describes a teenage girl with lumbosacral agenesis and neurogenic bladder in whom Meckel's diverticulum was successfully used for the Mitrofanoff procedure.

Extrarenal Wilm's tumour - a rare entity.

A rare case of extrarenal Wilms' tumour (ERWT) that presented as an asymptomatic subcutaneous lumbar mass is reported. The diagnosis could only be established postoperatively.