Cases reported "Hirschsprung Disease"

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1/51. A laparoscopic pull-through operation for Hirschsprung's disease: report of two infant cases.

    We describe herein a primary laparoscopic pull-through procedure that was successfully employed to treat two infants with Hirschsprung's disease. Mobilization of the rectum and sigmoid colon was performed laparoscopically, and the rectal mucosa was removed via a transanal submucosal resection. After inducing the rectal prolapse intussusceptically, the rectum was circumferentially transected, and the mobilized colon was pulled down through the rectal muscle cuff. Resection of the aganglionic bowel and the coloanal anastomosis, using the Soave-Denda method, was performed outside the anus. Both infants had an uneventful postoperative course with early recovery of bowel movement. Our experience demonstrates that this minimally invasive surgical procedure is feasible for young infants, and we believe that Hirschsprung's disease may be a definite indication for laparoscopic surgery.
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2/51. Repeat pull-through surgery for complicated Hirschsprung's disease: indications, techniques, and results.

    BACKGROUND: Most children with Hirschsprung's disease (HD) do well after a pull-through procedure. In the occasional child in which the operation fails, a repeat procedure may be necessary. methods: Nine children with HD aged 20 months to 9 years underwent repeat pull-through over a 4-year period. Original pull-throughs (six Soave, two Swenson, one Duhamel) were performed elsewhere 12 to 95 months earlier (median, 36 months). Indications for revision were stricture unresponsive to dilatation (n = 3) and acquired aganglionosis (n = 6). One of the latter had associated segmental intestinal neuronal dysplasia. One child with a stricture after a Swenson procedure underwent a repeat Swenson. The other eight underwent reconstruction using a Duhamel technique. Five had a defunctioning stoma before or at the time of repeat surgery. RESULTS: Median follow-up was 15 months (range, 4 to 40 months). Complications included wound infection (n = 2), anastomotic bleeding (n = 2), stoma leak (n = 1) or stenosis (n = 1), "kinking" at the top of the Duhamel (n = 1), and persistent septum (n = 1). Three patients have had a good outcome with normal stool patterns. One has intermittent soiling, and one has what is believed to be stool-holding behavior. Four have persistent obstructive symptoms caused by sphincter hypertonicity, which are being successfully managed nonoperatively. CONCLUSIONS: Repeat pull-through surgery can be performed safely in appropriately selected patients. Duhamel reconstruction usually is preferred for technical reasons, and a stoma is not always necessary. Outcome is generally favorable, but anal sphincter hypertonicity may cause persistent symptoms in some patients.
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3/51. Total colonic aganglionosis: diagnosis and management in a 12-year-old boy.

    Total colonic aganglionosis (TCA) is an unusual form of Hirschsprung's disease, and most of these present within the first few weeks and nearly all by the end of the first year of life. Very few cases presenting in older childhood or adulthood have been reported. Without a high index of suspicion these cases can easily be missed and, therefore, patients undergo repeated laparotomies for subacute intestinal obstruction without relief of symptoms. The diagnosis is suspected on operative findings and confirmed by histological evaluation of seromuscular biopsy specimens. Proper diagnosis will prevent loss of bowel length caused by inadvertent resection and will help in performing an optimal procedure to treat these patients. Discussed herein is another case of TCA in a 12-year-old boy with review of relevant literature.
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4/51. Near total intestinal aganglionosis with extreme short-bowel syndrome--a difficult surgical dilemma.

    Forty cases of total or near-total intestinal agangliosis (NTIA) were described to date in the English literature. Most cases had a lethal outcome. We describe the 41st case--a Beduin male neonate--who had only 30 cm of proximal hypoganglionic jejunum. He is presently almost one-year-old and thriving on home TPN, receiving one quarter of his caloric requirements orally using pregestamil, an MCT formula. The initial intricate course, diagnosis and several operative procedures, are elaborated. A review of the scant literature is discussed. The elusiveness of the correct diagnosis is pointed out and means to overcome these errors are described. Various surgical procedures have been suggested, none of which offer the perfect solution to the severe basic problem of short bowel. Long-term parenteral hyperalimentation is still the main modality of treatment. Based on our modest experience, we suggest saving every possible length of jejunum, even if hypoganglionic, since this bowel, following a few weeks of adaptation, starts to function fairly well, suggesting perhaps some neuro-muscular maturation. The best surgical approach is still pending. We present a report of a child with this disease and discuss the therapeutic dilemma.
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5/51. An ileostomy at the aganglionic ileum for total colon aganglionosis.

    For the initial management of total colon aganglionosis (TCA), an ileostomy is usually created at the distal end of the ganglionic bowel, which is associated with profuse diarrhea resulting in a water and electrolyte imbalance as well as malnutrition during the ileostomy period. An ileostomy at the aganglionic ileum several centimeters toward the anal side of the transition for TCA is thus expected to cause bowel retention without any bowel obstruction and to facilitate water reabsorption in the small bowel. We performed this procedure in 2 babies with TCA. The length of the aganglionic ileum was 50 cm and 10 cm, respectively. The ileostomies were made 5 cm distal to the transition at 1 month and 1 day after birth, respectively. Oral feedings were well tolerated, resulting in adequate weight gain and the stools were semisolid during the ileostomy periods. Definitive operations using the Duhamel procedure with a right colon patch graft without the creation of a new ileostomy were successfully performed at 7 and 3 months of age, respectively. The postoperative courses were satisfactory. An ileostomy at the aganglionic ileum appears to be the method of choice as initial management for patients with TCA.
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6/51. Sigmoid volvulus in children and adolescents.

    BACKGROUND: Sigmoid volvulus is an exceptionally rare and potentially life-threatening condition in the pediatric age group. STUDY DESIGN: We report our experience with three children treated for sigmoid volvulus and review the cases reported in the medical literature since 1940. RESULTS: Since 1940, 63 cases of sigmoid volvulus in children (including this series) have been reported. The median age was 7 years and the male to female ratio was 3.5:1. Two distinct presentations (acute and recurrent) were identified. Abdominal symptoms dominated the clinical picture. barium enemas either confirmed or were highly suggestive of sigmoid volvulus. Reduction by barium enema was successful in 77% (10 of 13) of the attempts. Forty-nine patients underwent operative treatment, with sigmoidectomy (with or without primary anastomosis) being the most common. The overall mortality rate was 6%, operative mortality was 8.1%, and neonatal mortality was 14%. Associated conditions were frequent. Particular emphasis should be placed on ruling out Hirschsprung's disease (present in 11 of 63 patients). CONCLUSIONS: Sigmoid volvulus remains a rare occurrence in children, but it should be included in the differential diagnosis of pain in children when colonic distention is present. An algorithm for treatment is proposed.
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7/51. Complex long-segment intestinal dysganglionosis.

    A case is reported with aganglionosis of the rectum, sigma, and descending colon; dysganglionosis with heterotopic ganglionic cells in the muscularis propria of the hypoganglionic transverse colon; and extreme hypoganglionosis (without detection of ganglionic cells) of the ascending colon and distal ileum. The ileum showed a transition zone with hypoganglionosis and intestinal neuronal dysplasia (IND) type B. As to the etiology of such complex intestinal innervation defects, pre- and perinatal perfusion deficits must be considered because their localization seems to be linked to the vascular anatomy of the colon. early diagnosis may be difficult, causing a delay in operative treatment and multiple operations. Different manifestations of dysganglionosis may be found in the same patient. The classical continuum of distal aganglionosis followed by proximal hypo- or dysganglionosis and then normally innervated bowel may not always be present. Therefore, in children with recurrent (sub-)ileus after resection of an aganglionic bowel segment, additional dysganglionosis such as IND or hypoganglionosis or even complex intestinal dysganglionosis should be excluded by full-thickness colon and small bowel biopsies.
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8/51. Clinical letter: epidural analgesia in a newborn with Hirschsprung's disease, associated with congenital central hypoventilation syndrome.

    A case is presented of a neonate with Hirschsprung's disease, associated with congenital central hypoventilation syndrome. After an ileostomy (at 2 days) and a stoma revision (at 10 days), postoperative pain management was established by continuous intravenous infusion of morphine, which caused severe postoperative respiratory depression. At 6 weeks a re-exploration and stoma revision was performed using postoperative epidural analgesia with bupivacaine. This caused no respiratory depression. A colectomy under epidural analgesia at 8 months was also uneventful. Respiratory difficulties in children with congenital central hypoventilation syndrome associated with Hirschsprung's disease are discussed in relation to the technique of choice for postoperative pain management.
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9/51. Laparoscopic full-thickness intestinal biopsies in children.

    BACKGROUND: laparoscopy may reduce postoperative pain and hospital stay, compared with laparotomy. The use of laparoscopic surgery to obtain full-thickness intestinal biopsies in children has not been previously reported. methods: Eleven children aged 1.6 to 19 years (median, 4.5 years) underwent laparoscopic full-thickness biopsy of the stomach, small bowel, colon, or a combination thereof. Each procedure used one 12-mm and two 5-mm ports. RESULTS: Eight children with obstructive symptoms after a pull-through for hirschsprung disease underwent multiple colon and small bowel biopsies (range, 3-6; median, 5); intestinal neuronal dysplasia was found in two. Two patients with cystic fibrosis had diffuse colonic narrowing; a diagnosis of enzyme-induced fibrosing colonopathy was made in one and nonspecific inflammation was found in the other. One child had a thickened stomach, and a gastroscopic-directed full-thickness biopsy revealed plasmacytoma. Nine of the 11 patients had a previous laparotomy, and ports were placed through preexisting scars. Median hospital stay was 2 days. No patient required more than 24 hours of narcotics. There were no leaks, and no other morbidity or mortality occurred. None of the patients required conversion to an open procedure. biopsy results significantly affected treatment for each patient. CONCLUSIONS: Laparoscopic full-thickness intestinal biopsy is safe and effective for a variety of gastrointestinal problems in children. This technique is associated with a short hospital stay, minimal pain, and a very low risk of complications and can be performed even in patients who have had a previous laparotomy.
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10/51. Fourteen-year experience of acetylcholinesterase staining for rectal mucosal biopsy in neonatal Hirschsprung's disease.

    BACKGROUND/PURPOSE: acetylcholinesterase (AChE) staining of rectal mucosal biopsy specimens is the most important and popular examination for making a definite diagnosis of Hirschsprung's disease. This examination often is performed for patients with constipation in the daily clinic. The results of this examination are reflected immediately in the treatment. However, the authors sometimes encountered difficult cases to diagnose, especially in neonates. Therefore, a retrospective investigation was conducted on the benefits and problems of AChE staining of rectal mucosal biopsy specimens in neonates. methods: The authors encountered 459 cases (91 neonates) of suspected Hirschsprung's disease, clinically, from April 1986 to March 2000. Mucosal specimens were taken by punch biopsies. Samples were stained by the modified Karnovsky Roots method using rubeanic acid as an amplifier and immediately examined with a light microscope. These results were collected and assessed mainly on neonatal cases. The authors also analyzed the 104 cases of Hirschsprung's disease diagnosed in patients less than 1 year of age to evaluate the relationship between the grade of proliferation of AChE positive fiber and age. RESULTS: Forty-one neonatal cases of Hirschsprung's disease were diagnosed based on the findings of AChE staining. A definite diagnosis of Hirschsprung's disease was confirmed based on the pathologic findings of operative samples. Forty-eight cases that were diagnosed as normal included 4 cases that turned out to be false-negative (3 Hirschspurung's disease cases and 1 case of an allied disorder of Hirschsprung's disease). There were no major complications in mucosal punch biopsy. In the cases of Hirschsprung's disease diagnosed in a patient less than 1 year of age, the grade of AChE-positive fiber tended to increase with the aging of patients. CONCLUSIONS: The specificity of AChE staining was high (100%), but its sensitivity was slightly low (91%). Careful long-term follow-up is required for any cases diagnosed as normal. Mucosal biopsies should be repeated in cases of persistent clinical symptoms.
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