Transanal endorectal pull-through for Hirschsprung's disease: experience with 50 patients.

INTRODUCTION: Transanal endorectal pull-through (TEPT) is the latest advancement in the treatment of Hirschsprung's disease (HD). The aim of this study was to evaluate the safety and efficacy of TEPT as a definitive treatment for patients with HD. PATIENTS AND METHODS: A retrospective study of 50 patients who underwent TEPT at Uludag University, Bursa, Turkey, between June 2001 and April 2012 was conducted. These patients were evaluated with regard to their age, sex, preoperative findings, and length of the aganglionic segment, intraoperative and postoperative complications, and results of the follow-up. RESULTS: Forty-three patients were boys and seven were girls. The median age was 3 months (range 0-96 months). The most common symptoms were abdominal distention and vomiting. HD was diagnosed in 38 patients using barium enema and anorectal manometry, in 11 patients using rectal biopsy, and in 1 patient using laparotomy with biopsy. The mean time from the beginning of enteral feeding was 2.2 ± 1.1 days. The mean follow-up period was 26.7 ± 20.8 months. The postoperative complications included transient perianal excoriation in 12 patients, enterocolitis in 10, anastomotic stricture in 3, soiling in 3, recurrent constipation in 2, prolapse of the pulled through colon in 1, anastomotic leak in 1, and rectovestibular fistula in 1 patient. CONCLUSIONS: TEPT is a feasible and safe procedure in children with rectosigmoid HD.

A very rare cause of intestinal atresia: intrauterine intussusception due to Meckel's diverticulum.

UNLABELLED: Intrauterine intussusception is one of the rare causes of intestinal atresia. CONCLUSION: A case of ileal atresia due to intrauterine intussusception caused by Meckel's diverticulum is reported.

Oromandibular limb hypogenesis and gastroschisis.

The oromandibular limb hypogenesis syndromes (OLHS), which are congenital malformations involving the tongue, mandible, and the limbs are extremely rare with a few sporadic cases reported in the literature. In this report, the authors report a case of OLHS with gastroschisis, that has not been described previously according to the medline search.

Urodynamic findings in the tethered spinal cord: the effect of tethered cord division on lower urinary tract functions.

PURPOSE: The aim of this study is to evaluate the effect of division of the tethered spinal cord urodynamically in spinal dysraphic cases. METHODS: Between 1995-1997, 20 cases (11 males, 9 females) aged from 5 months to 13 years with TSC were investigated. 13 cases (65%) were classed as belonging to the myelomeningocele group and 5 cases (35%) to the spina bifida occulta group. We used a computerized urodynamic system to evaluate the functions of the lower urinary tracts pre- and postoperatively. The definitive diagnosis of cord tethering was made using magnetic resonance imaging (MRI) in 19 cases (95%) and spinal ultrasound in 1 case (5%). Division of filum terminale and laminectomy were carried out in all cases by the Neurosurgery Department, and 2 cases with retethering were operated on twice. RESULTS: All of these cases were assessed urodynamically in the preoperative and postoperative period. Significant improvements were noted in detrusor functions (35%); electromyography recordings (45%); high leak point pressures (55%) and anal and urinary continence (70%). CONCLUSION: Lower urinary tract dysfunctions secondary to tethered cord syndrome are very common in spinal dysraphic cases and significant improvements can be achieved with a judiciously timed division of the spinal tethered cord.

Solitary rectal ulcer syndrome in children.

The solitary rectal ulcer syndrome (SRUS) is an unusual disorder in childhood. Although well recognized in adult literature, the pediatric experience with this condition is limited, so SRUS often goes unrecognized or misdiagnosed. There are very few pediatric case reports in the English literature. This report describes four patients who presented with rectal bleeding, constipation, mucous discharge, and lower abdominal pain, with a diagnosis of SRUS. The diagnosis was made by rectoscopy, defecogram, anorectal manometry and histopathological evaluation. In two patients, defecogram showed a rectocele with both, the sphincter failed to relax to voluntary squeeze pressure on anorectal manometric examination. The histopathological finding in all patients was fibrous obliteration of the lamina propria with disorientation of muscle fibers. All of the patients responded well to conservative therapy, which included defecation training, laxatives, sulfasalazine, and application of rectal sucralfate enema, and remained asymptomatic on the follow-up. Although rare in the pediatric population, SRUS should be relatively easy to recognize in the child with rectal bleeding, after elimination of other causes. If suspected, the diagnosis of SRUS may be made at endoscopy and confirmed by rectal biopsy.

Quantitation of defecation function using radionuclide artificial stool in children with chronic constipation.

We aimed to investigate the value of scintigraphically determined evacuation fraction (EVF) as an objective follow-up criterion in medically treated children with chronic constipation, and to evaluate the relation between patients' symptoms and rectal emptying rate. Thirty children (m: 22, f: 8, mean age: 8.9 +/- 0.9 year) suffering from chronic constipation were assessed by scintigraphic evacuation study. All patients underwent anal manometric and scintigraphic examinations before their therapy was started. During 3 months, they received conventional laxative treatment for constipation, and scintigraphic studies were repeated in 20 of 30 patients. Scintigraphic examinations consisted of instillation of artificial stool into the rectum up to the volume impending rectal evacuation, and acquisition of pre- and post-evacuation images. EVF values were then calculated from the images using a fixed region of interest. All of the patients had high resting anal canal pressure (75 +/- 12 mmHg) and had positive rectoanal inhibitory reflex. In 14/20 patients (70%) who benefited from therapy, mean EVF changed from 43.6% to 62.2% (p < 0.001), while decreasing in 6120 who did not benefit. The volume arousing rectal evacuation was significantly lower at the second scintigraphic study (261 +/- 98 ml) than the first study (354 +/- 138 ml) (p < 0.05). As a conclusion: 1) EVF was low in chronically constipated children, 2) it was improved after conventional laxative treatment and, 3) radionuclide EVF determination may be a useful method for follow-up.

Aicardi syndrome associated with palatal hemangioma.

An 1-day-old female newborn who had typical clinical features of Aicardi syndrome, such as agenesis of the corpus callosum, ocular abnormalities and infantile spasm associated with a palatal hemangioma is reported. The intraoral mass, which occluded incompletely the oropharynx and right side of the nasopharynx, was partially excised under general anesthesia. This is the first reported patient with Aicardi syndrome with palatal hemangioma, according to the med-line search.

Ruptured spleen due to blunt trauma in children: analysis of blood transfusion requirements.

Selective non-operative management of splenic injury in children is generally considered to be safe, and the majority of those with isolated injuries do not require blood transfusion. Eighty-four children were treated for blunt splenic trauma from 1988 to 1997 in the Department of Paediatric Surgery, The Medical Faculty of Uludag University, Bursa, Turkey. Management involved non-operative care in 56 cases (66.7%), splenectomy in 20 (23.8%) and splenorraphy in eight (9.5%) cases. There were no later complications related to splenic injury. The overall mortality was 9.5% (8/84) and the factors effecting mortality were additional intra-abdominal and/or other system injuries. Twenty-two children were not transfused during non-operative treatment. In our study, only 57.1% of the children in the non-operative group received blood transfusions. Some of the patients in this group received only small amounts of blood and probably would have recovered without it. It is concluded that, based on a very strict protocol in conservative management, the total amount of transfused blood could be reduced in children with splenic injuries due to blunt abdominal trauma.

Does maternal drug ingestion cause megacystis microcolon intestinal hypoperistalsis syndrome? I. Clomiphene trial.

PURPOSE: Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a congenital and lethal disease, and the etiology of the disease is not clear. It is speculated that maternal ingestion of some drugs during pregnancy may be an etiologic factor. In this study we aimed to investigate the effect of maternal ingestion of clomiphene on fetal bladder and colon in pregnant rats. METHODS: We separated animals into a control group including 14 rats and a clomiphene group with 30 rats. Nothing was given to the control group during pregnancy. Two mg/kg/day clomiphene intraperitoneally was given to the study group from the 6th to 12th day of pregnancy. All of them were sacrificed on the 20th day of pregnancy. Histopathological examination of the fetal colon and bladder was performed. RESULTS: In the clomiphene group a significant decrease in the thickness of the bladder wall, an increase in bladder epithelium, an increase in muscle atrophy of the colon and bladder wall, an increase in vacuoler degeneration of the muscles of the bladder and colon wall, a decrease in ganglion cell numbers in the myenteric plexus of the bladder and a decrease in the thickness of the bladder tunica muscularis were determined. CONCLUSION: In our rat model we found histological structural changes in the rats' colon and bladder walls as a result of using clomiphene on days 6-12 of pregnancy; a similar pathological finding to those found in some of the MMIHS patients' colons and bladders.

Jugular phlebectasia.

A patient, 5 years of age, presented with a swelling on the right side of the neck. Ultrasonography and computed tomography confirmed a diagnosis of phlebectasia of the right internal jugular vein (IJV). The patient underwent operation and the dilated IJV was excised. Dilatation of the IJV with a Valsalva maneuver suggested a mechanical obstruction in the neck or mediastinum. However, the exact cause is still unknown. Finally, we found the patient to have a thinning of the wall but no other associated abnormality.

Long-term outcome of the fetal adrenal gland transplantation in rats.

Three experimental groups have been constructed to evaluate the long-term outcome of fetal adrenal transplantation in rats. In Group 1 (n = 10), rats underwent bilateral adrenalectomy. In Group 2 (n = 10), rats underwent a sham procedure which included flank incision and arterial canulization. In Group 3 (n = 20), a left adrenalectomy was performed followed immediately by transplantation of a fetal adrenal graft into the greater omentum and marked with a prolene suture (Stage 1). One year later, the right adrenal gland of the recipient was removed followed by a determination of the fetal adrenal graft function (Stage 2). Graft function was evaluated by measuring ACTH and corticosterone levels; and a histologic examination of the transplanted fetal adrenal gland was obtained at autopsy. In Group 1, all the rats died within first 8 hours, following bilateral adrenalectomy. In Group 2, and Group 3, all rats survived after Stage 1 operation. During the Stage 2 operation, it was observed that three rats (15%) had neither fetal adrenal transplant nor prolene suture, seven rats (35%) had a well vascularized and developed fetal adrenal graft and a prolene suture. There was no visible fetal adrenal graft but the prolene suture was present in the remaining rats (50%) in Group 3. After removal of the right adrenal gland (6 and 12 hours later), the mean plasma level of ACTH increased with a decline in mean serum corticosterone level in Group 3 compared to the sham-operated animals (p < 0.001). In spite of visible, and viable transplants, all rats died within 48 hours following Stage 2 operation. The mean weight of the fetal adrenal transplant showed a sixteen-fold increase compared to the initial weight (p < 0.001) and histologic examination showed all 3 zones of adrenal cortex, but there were no medullary cells noted. Although the transplanted fetal adrenal grafts survived, their hormonal function was not enough to maintain host viability. Based on these results it is concluded that, insufficiency of the transplanted fetal adrenal gland may be secondary to either graft rejection or suppression of the transplanted tissue by the functional recipient adrenal despite the fetal adrenal transplant survival.

Rabdomyosarcoma of the biliary tree.

Rabdomyosarcoma of the biliary tree is one of the rare causes of biliary tract obstruction in childhood. Nevertheless it is the most common cause of obstructive jaundice due to neoplastic biliary obstruction. We present a two-year-old child with obstructive jaundice secondary to an embryonal rhabdomyosarcoma of the biliary tree. She underwent surgery and, after total excision of the mass, a hepaticojejunostomy and Roux-en-Y anastomosis were performed. She was referred to the Pediatric Oncology Group for follow-up. Rhabdomyosarcoma of the bilary tree, although rare, must be considered in the etiology of obstructive jaundice in children.

Does maternal drug ingestion cause megacystis microcolon intestinal hypoperistalsis syndrome? II. Bromide trial.

PURPOSE: Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a congenital disease, and the etiology of the disease is unclear. It is speculated that maternal ingestion of some drugs during pregnancy and degeneration of smooth muscle cells in the fetus may be an etiologic factor. In this study we aimed to investigate the effect of maternal ingestion of bromide on the fetal bladder and colon in pregnant rats. METHOD: We separated animals into a bromide group including 30 rats and a control group with 14 rats. Nothing was given to the control group during pregnancy. Intraperitoneally 8 mg/kg/day bromide was given to the study group from the 6th to 12th day of pregnancy. All of the rats were sacrificed on the 20th day of pregnancy. Histopathological examination of fetal colons and bladders was performed. RESULTS: In the bromide group, an increase in the colon and bladder diameter, an increase in muscle atrophy in the colon and bladder wall, an increase in vacuolar degeneration in the muscles of the bladder and colon wall, and a significant decrease in ganglion cell numbers in the myenteric plexus of the colon and bladder were determined. CONCLUSION: In our rat model, we found histological structural changes in the rats' colon and bladder walls as a result of using bromide on the 6-12th days of pregnancy similar to pathological findings found in some of MMIHS patients' bowels and bladders.

Does maternal drug ingestion cause megacystis microcolon intestinal hypoperistalsis syndrome? III. Ethanol trial.

PURPOSE: Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) is a congenital disease, and the etiology of the disease is unclear. It is speculated that maternal ingestion of some drugs during pregnancy may be an etiologic factor. In this study we aimed to investigate the effect of maternal ingestion of ethanol on the fetal bladder and colon in pregnant rats. METHODS: We separated animals into an ethanol group including 30 rats and a control group with 14 rats. Nothing was given to the control group during pregnancy. Orogastrically 2 cc/day 30% ethanol was given to the study group from the 6th to 12th day of pregnancy. All of them were sacrificed on the 20th day of pregnancy. Histopathological examination of the fetal colon and bladder was performed. RESULTS: In the ethanol group a significant decrease in the colon and bladder diameter, a significant decrease in the thickness of the colon and bladder wall, an increase in vacuolar degeneration in the muscles of the bladder wall, an increase in connective tissue proliferation among the muscles of the bladder, a significant decrease in the number of ganglion cells in the myenteric plexus of the colon and a significant decrease in the thickness of the bladder tunica muscularis were determined. CONCLUSION: In our rat model we found histological structural changes in the rats' colon and bladder wall similar to a pathological finding found in some of the MMIHS patients' bowel and bladder as a result of using ethanol on the 6th-12th days of pregnancy.

Giant omphalocele filled by a duplication cyst.

Ileal duplication cysts within a giant omphalocele are very rare. Only a few cases have been reported in the English literature (4). We report one case of giant omphalocele, which included a huge ileal duplication cyst, detected by prenatal US, and diagnosed at surgery after birth. This case illustrates the diagnostic and therapeutic problems occurring during pregnancy and the neonatal period.