Anorectal Malformation with Rectovestibular Fistula and Vaginal Agenesis: Usage of Rectovestibular Fistula as a Neovagina Followed by PSARP with Preservation of the Anal Sphincter.

Anorectal malformation with rectovestibular fistula associated with vaginal agenesis is rare. We report on a child in whom this combination was diagnosed at the age of 1 year. After creation of a divided descending colostomy, we chose to leave the rectum-rectovestibular fistula to function as a neovagina, while the sigmoid colon was relocated via modified posterior sagittal anorectoplasty. The colostomy was closed 6 months after the main surgery. After a follow-up of 3 years, the patient is continent for stool and urine. The introitus of the neovagina appears wide and easy to explore. We conclude that our surgical approach may be a good option for these children.

Intrahepatic bile duct primary cilia in biliary atresia.

AIM: The etiopathogenesis of non-syndromic biliary atresia (BA) is obscure. The primary aim was to investigate intrahepatic bile duct cilia (IHBC) in BA at diagnosis and its correlation with clinical outcome. The secondary aim was to analyze IHBC in routine paraffin-embedded liver biopsies using conventional scanning electron microscopy (SEM). METHODS: Surgical liver biopsies taken at diagnosis from 22 BA infants (age range, 39-116 days) and from eight children with non-BA chronic cholestasis (age range, 162 days -16.8 years) were evaluated for IHBC by immunofluorescence (IF) and SEM. A minimum 18-month follow-up after surgery was available for all patients. RESULTS: By IF, cilia were present in 6/8 (75%) non-BA but only in 3/22 (14%) BA cases, and cilia were reduced or absent in 19/22 (86%) BA and 2/8 (25%) non-BA livers (P < 0.01). In BA, cilia presence was found to be associated with clearance of jaundice at 6-month follow-up (P < 0.05). However, high overall survival rates with native liver, >90% at 12 months, and >70% at 24 months post-surgery, were recorded regardless of cilia presence/absence at diagnosis. Electron microscopy was able to detect bile ducts and cilia in routine liver biopsies, revealing significant abnormalities in 100% BA livers. CONCLUSIONS: The presence of IHBC in BA livers at the diagnosis was associated with resolution of cholestasis, although was not predictive of short-term survival with native liver. Scanning electron microscopy represents a powerful new tool to study routine liver biopsies in biliary disorders. Cilia dysfunction in BA pathogenesis and/or disease progression warrants further investigation.

Experimental use of labial mucosa free graft urinary conduit in the Mitrofanoff principle.

PURPOSE: One method of treating urinary incontinence is to create a catheterizable and continent vesicostomy by the Mitrofanoff principle, based on the use of a conduit, which in the original method is the appendix. The authors present an experimental technique in which a continent vesicostomy is created by the Mitrofanoff principle, using a conduit made of a labial mucosa free graft. METHODS: Six 30-day-old pigs underwent surgery to create a continent vesicostomy by the Mitrofanoff principle. A tube, made of labial mucosa free graft from the lower lip, was used. The labial mucosal conduit was anastomosed to the bladder mucosa. The tunnel was then prepared, part of which passed through the fibers of the right rectus muscle. The conduit was taken through the tunnel and anastomosed to the skin in the right iliac fossa. RESULTS: Fifty days after the operation, the vesicostomy was continent, pervious, and easily catheterizable in 5 animals. In the sixth pig, the cystostomic tube did not graft successfully because it was infected. CONCLUSIONS: A number of investigators have extrapolated Mitrofanoff method using the ileum, the ureter, or others. The use of a tube made by labial mucosa free graft is an easily performed technique, and as with the Mitrofanoff method, continence and easy catheterization are successfully achieved.

Substitution of thoracic oesophagus by interposition of a pedicled gastric tube, preserving LES function: clinical and histological follow-up.

Assessment of clinical evolution and histological findings in a group of animals experimentally operated on to substitute the thoracic oesophagus with a gastric tube. Six piglets underwent oesophageal replacement with a gastric tube, constructed from the greater curvature of stomach and pedicled on the gastroepiploic vessels, which was interposed between the oesophageal stumps. At follow-up, all animals were found to be growing and eating normally, apart from case no 1 (stenosis of the lower oesophageal anastomosis). Ph-metry showed a neutral pH on the gastric tube. Postmortem histological analysis of the gastric tube and native oesophagus samples did not show any significant lesions, except in case no 1 (inflammation of the gastric tube and upper oesophagus due to food stasis). The technique of substitution of the oesophagus with an interposed pedicled gastric tube can be a breakthrough in existing surgical methods of oesophageal replacement.

Phimosis. Preputial plasty using transversal widening on the dorsal side with EMLA local anesthetic cream.

BACKGROUND/PURPOSE: In the United States, the treatment of choice for the correction of phimosis is circumcision, whereas in European countries, the condition is usually treated by preputial plasty using Duhamel's method or modified versions. We report our experience in correcting phimosis by preputial plasty using transversal widening on the dorsal side with EMLA local anesthetic cream. METHODS: Twenty-six patients with phimosis were operated on by preputial plasty, under local anesthesia with EMLA cream. A transversal incision is made on the dorsal side of the ring of prepuce, like 3 contiguous Ts, the middle one inverted with the long arm on the preputial mucosa side. The 2 small mucocutaneous flaps of the prepuce are separated and then sutured with interrupted stitches, thus transforming the incisions from T to V. RESULTS: No postoperative complications were observed. At 1-year follow-up, the cosmetic and functional results were satisfactory. CONCLUSIONS: The technique of preputial plasty that the authors present enlarges the stenotic ring of prepuce by a transversal widening on the dorsal side. The ring of prepuce obtained is wide and symmetrical on its dorsal and ventral sides and therefore cosmetically and functionally satisfactory. It is a good alternative to the more radical circumcision technique.

Postoperative radiologic evaluation of the esophagus.

A wide range of surgical procedures are performed for either benign and malignant esophageal lesions, that may be classified as demolitive or conservative interventions. The former is characterized by resection and replacement of the esophagus with another organ, whereas conservative surgery is obtained preserving esophageal function without resection. Knowledge of surgical techniques and major complications is extremely important for a correctly performed radiographic study and a proper interpretation of the imaging findings. Functional evaluation of the operated alimentary tract and early detection of postoperative complications are essential for a successful recovery of esophageal surgery patients. We provide a brief overview of the main esophageal surgical procedures, reporting our experience on postoperative imaging of 104 patients submitted to surgery for either benign or malignant esophageal disease during a 6-year period.

Pyloric atresia: a new operation to reconstruct the pyloric sphincter.

BACKGROUND/PURPOSE: The standard method of surgical correction of pyloric atresia "solid segment" type is a gastroduodenostomy that can in the long term cause bilious duodenogastric reflux. The authors report 2 cases of pyloric atresia in which the pyloric sphincter was reconstructed by a new technique of gastroduodenal mucosal advancement anastomosis. METHODS: Two premature babies with "solid segment"-type pyloric atresia, one with an associated junctional epidermolysis bullosa, underwent surgery for reconstruction of the pyloric sphincter. By a longitudinal incision of the atretic pylorus, the cul-de-sacs of gastric and duodenal mucosa were isolated in the respective gastric and duodenal sides, advanced into the opened pyloric canal, and sutured together using end-to-end anastomosis. The longitudinal pyloromyotomy then was closed above the reconstructed mucosal pyloric neocanal. RESULTS: The postoperative course was normal. At 7 years (patient 1) and 2 years (patient 2) after the operation, both are well, and no gastrointestinal disorders are present. Good competence of the pyloric sphincter has been confirmed by x-ray barium meal in both cases, and by HIDA technetium 99m hepatic scintiscan and esophagogastroduodenoscopy (EGD) with biopsy in patient 1. CONCLUSIONS: Our technique of surgical correction of pyloric atresia allows preservation of the pyloric sphincter, whose muscular layer, although hypoplastic, is present in these cases.

Reconstruction of hypospadias and epispadias with buccal mucosa free graft as primary surgery: more than 10 years of experience.

PURPOSE: The most common techniques to reconstruct the urethra for severe hypospadias or epispadias involve use of the genital skin. Buccal mucosa free graft is usually used in a second operation due to a paucity of usable genital tissue. We report our experience using buccal mucosa free graft as primary surgery for hypospadias and epispadias. MATERIALS AND METHODS: Between 1989 and 2001 the urethra was reconstructed using buccal mucosa free graft as a primary operation in 22 patients 3 to 13 years old with middle or proximal hypospadias (20) and exstrophy/epispadias (2). In the first 5 cases of hypospadias the buccal mucosa was combined with bladder mucosa. In the 2 exstrophy/epispadias cases treated with the penile disassembly technique the distal part of the urethra was reconstructed with buccal mucosa. RESULTS: At mean 5-year followup 2 cases of urethral fistulas and 1 case of severe stenosis of the proximal anastomosis were observed, which required surgical correction. In 4 other cases mild stenosis of the anastomosis required urethral dilations in the first month after surgery. CONCLUSIONS: Buccal mucosa is an excellent source of graft material for urethral replacement in complex urethroplasties as primary surgery. It is readily available, elastic, resistant and technically easy to harvest.

Correction of congenital chordee penis by 'ventral separation and outward rotation of corpora'.

BACKGROUND/PURPOSE: The formation of congenital chordee penis in patients with or without hypospadias is multifactorial and not completely clear. In most cases, after release of all known causes of chordee, "residual" penile curvature can persist. The authors discuss the etiopathogenetic mechanisms of congenital chordee penis and describe their experience in surgical correction of "residual" penile curvature by "ventral separation and outward rotation of corpora." METHODS: In 22 patients with congenital chordee penis with or without hypospadias, "residual" penile curvature was corrected by "ventral separation and outward rotation of corpora." RESULTS: Ventral separation and outward rotation of corpora resulted in satisfactory correction of penile curvature. In only 2 cases of chordee penis without hypospadias was further intervention necessary: a Nesbit's plication was carried out in one and a neourethra placed in the other. At follow-up no residual penile curvature was observed. CONCLUSIONS: An arrest of development of the penis caused by failure of outward ventral rotation of the corpora, may contribute to the formation of chordee penis. When "residual" penile curvature persists, "ventral separation and outward rotation of corpora" can be used to correct this complex malformation.