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Results for “Hirschsprung disease”

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At least 19 recordsLinked to original sources
PubMed1979

The barium-enema examination and rectal manometry in Hirschsprung disease.

Barium-enema studies and rectal manometry were clearly shown complementary in diagnosing Hirschsprung disease. Of 27 patients studied, 19 had positive barium-enema and rectal manometric studies and were proved histologically to have Hirschsprung disease. Three had positive barium-enemas but normal rectal manometric studies in the first 10 days of life; later manometric studies were positive, and all 3 were subsequently proved to have Hirschsprung disease. Three patients had negative barium-enema and positive manometric studies and were proved to have low segment Hirschsprung disease. If both studies indicate the disease, rectal biopsy is not necessary before surgery.

Barium Sulfate↗
PubMed1979

Small intestinal changes in enterocolitis complicating Hirschsprung's disease.

Hirschsprung's disease is one of the more common causes of childhood bowel obstruction. The disease can cause enterocolitis which, untreated, may result in considerable mortality. We describe our evaluation of two infants who suffered from intractable diarrhea of infancy secondary to Hirschsprung's disease. We found that mucosal damage to the small bowel and disaccharidase deficiency are among the most important mechanisms producing this complications. Therefore, management of enterocolitis including parenteral alimentation should proceed as soon as the complication is observed.

Biopsy↗
PubMed2024

Dynamic Pathology of Enteric Neural Network Using Curcumin-assisted Multiphoton Laser Imaging in Hirschsprung Disease.

BACKGROUND: In living tissue, it has been difficult to make microscopic-level observations without damaging the tissue. We have invented a novel intravital fluorescent observation method (IFOM) for real-time tissue observation, combining multiphoton laser scanning microscopy with curcumin vital staining (CVS-IFOM). The aim of this study was to use CVS-IFOM to analyze the enteric nervous system (ENS) in mice and human patients with hypoganglionosis and Hirschsprung disease (HSCR). METHODS: In an initial viability study, we compared live ENS images from nonfluorescent C57BL6 mice stained with curcumin (n = 5) and green fluorescent protein mice (n = 5) using multiphoton laser scanning microscopy. We then explored CVS-IFOM for the live examination of resected colon tissues from 1 patient with hypoganglionosis and 3 patients with HSCR. RESULTS: In the viability study, detailed ENS histologic features were only observed in the curcumin-stained mice. In the patient with hypoganglionosis, CVS-IFOM provided ENS details that were not visualized under hematoxylin and eosin staining or calretinin immunohistochemistry, allowing the analysis of ENS size, neural bundle number, and neural cell number per plexus. In patients with HSCR, CVS-IFOM showed a gradual hypoplastic change in the ENS from the oral edge to the anal edge, detecting disproportionate changes in the ENS within the same intestinal level, supporting a circumferentially uneven distribution of the intestinal ENS. CONCLUSIONS: CVS-IFOM may be supportive for intraoperative pathologic diagnosis during surgeries for HSCR.

Hirschsprung Disease↗
PubMed1979

[Steiner-type neuroapudomatosis presented a pseudo-Hirschsprung disease (author's transl)].

A case of Steiner type neuroapudomatosis presented with a pseudo-Hirschsprung disease in an infant. Secondarily, the facies became typical, while hyperthyrocalcitoninaemia led to the discovery at the age of 2 years and 8 months of a medullary carcinoma of the thyroid. Despite early surgery in the absence of any clinical signs, it was not possible to avoid cervical lymphatic spread.

Apudoma↗
PubMed1979

Z shaped primary colorectal anastomosis using the GIA autosuture for Hirschsprung's disease.

The Duhamel operation for Hirschsprung's disease has won wide acceptance throughout the world. However, this procedure is not without difficulties, and the usual technique of employing various crushing clamps for division of the colo-rectal septum is inelegant, inconvenient and uncertain. This paper presents an operative technique with particular reference to the use of the GIA autosuture surgical stapling instrument in the Z shaped primary colorectal side-to-side anastomosis for Hirschsprung's disease advocated by Ikeda. This is a single and primary procedure, and makes the postoperative care easier and more comfortable for the patients. Experiences with twenty-five patients are reported.

Child↗
PubMed1978

A critical evaluation of the Duhamel operation for Hirschsprung's disease.

Eighty-nine patients with Hirschsprung's disease were treated from 1972 to 1977. Of the patients, 70 were boys, 85 were the result of full-term pregnancy, and four had Down's syndrome. There were 23 children, 32 infants, and 34 neonates including seven (20%) with enterocolitis. Early mortality was 8.8% in neonates and 3% in infants. After initial diversion (colostomy or enterostomy), 66 patients had "classic" (ten), modified (52), or "long" (four) Duhamel pull-through procedures at age 1 year. Operative mortality was zero. Three late deaths occurred; two of them were mongols. Fecaloma occurred in eight of ten classic cases that required revision. Postoperative enterocolitis occurred in three of four long procedures done for total colonic aganglionosis. The modified Duhamel procedure was associated with a low complication rate and good function. There were no instances of anastomotic leak, anal stricture, or genitourinary problems. These data indicate that the modified Duhamel operation is a highly acceptable procedure in the management of Hirsch sprung's disease.

Adolescent↗
PubMed1979

Hirschsprung's disease with skip area (segmental aganglionosis).

Hirschsprung's disease is characterized by a single aganglionic segment of colon extending distally to the anal margin. Well documented reports of segmental aganglionosis have been rare. We report a case of segmental aganglionosis in which there were two distinct aganglionic segments resected. The entire transverse colon between the two aganglionic segments was normally ganglionated, preserved, and utilized and functions in a normal fashion.

Colon↗
PubMed1979

A manometric assessment of anorectal pressures and its significance in the diagnosis of Hirschsprung's disease and idiopathic megacolon.

Anorectal manometric studies were performed on nine patients with Hirschsprung's disease and ten patients with idiopathic megacolon for the purpose of differentiation, comparing with those of the normal subjects. The anorectal reflex was absent in all nine of the patients with Hirschsprung's disease while in all of ten patients with idiopathic megacolon rectal distension produced a relaxation in the anal canal like that of the normal subjects. Manometric studies proved to be a reliable and non-invasive technique for the diagnosis of Hirschsprung's disease and were of particular value as simple screening tests in patients with a clinical suggestion of this disease. The manometric studies of the patients with Hirschsprung's disease or idiopathic megacolon in the present series also demonstrated that the pronounced internal sphincter contraction combined with a total absence of reflex relaxation or an inadequate response of the sphincter to rectal distension might be responsible for the obstructive symptoms in these diseases.

Adolescent↗
PubMed1979

An evaluation of the modified endorectal procedure for Hirschsprung's disease.

During the past 4 yr, 60 patients with Hirschsprung's disease have been treated at our institution. Twenty-eight patients within this group have undergone a modified endorectal pull-through procedure. There are 18 males and 10 females in the series. There are 5 children with total colonic involvement, 3 patients with aganglionosis to the mid-transverse colon, and one child with Hirschsprung's disease extending to the distal jejunum. The associated anomalies have consisted of four cases of Down's syndrome, one of whom also has transposition of the great vessels. There has been no operative-related mortality or late mortality in our series. Two major complications (anastomotic leak following a colostomy closure and incorrect pathological diagnosis of aganglionic level) and five minor complications have occurred. The average follow-up period is 31.3 mo. The functional results postoperatively have been excellent. The 18 patients over the age of 3 yr are completely toilet-trained. The remaining patients are too young to be toilet-trained, but are continent in the sense that they do not soil between bowel movements. Because of the satisfying functional results in this series, we have now expanded our indications for this procedure to patients requiring total colectomy for ulcerative colitis and familial polyposis. During the past 2 yr, 8 patients with ulcerative colitis have undergone the endorectal pull-through together with a total colectomy.

Anal Canal↗
PubMed1978

Viscoelastic properties of the rectal wall in Hirschsprung's disease.

Viscoelastic properties of the rectal wall were compared with Hirschsprung's disease. The elasticity of the rectal wall after accomodation to distension was found to be significantly greater (P less than 0.001) in patients, and the time taken by the rectum to accomodate was also found to be longer (P less than 0.001). The increased elasticity correlated well with severity of the illness, but none of the parameters correlated with length of aganglionic segment. Measuring elastic properties of the rectal wall may help to assess the severity of illness in patients with Hirschsprung's disease.

Adolescent↗
PubMed1978

Abnormal rectal immunoglobulin pattern in Hirschsprung's disease.

The immunoglobulin content of rectal biopsy tissue and secretions from twelve neonates in whom Hirschsprung's disease was suspected was determined by an organ-culture technique and radioimmunoassay. The immunoreactive IgG content of explanted rectal tissue and its secretions in those six children who proved to have Hirschsprung's disease was much higher than in those with other types of obstructive lower-intestinal disease. Increased amounts of IgG may represent maternally derived antibody associated with neonatal gut neural elements. This seems to be the first report of an immunological abnormality in Hirschsprung's disease.

Humans↗
PubMed1979

Manometry and histochemistry in the diagnosis of Hirschsprung's disease.

Acetylcholine esterase histochemistry and rectal manometry have been used sequentially to evaluate constipated children and to make the diagnosis of Hirschsprung's disease. When applied together, these modalities give a reliable diagnosis of Hirschsprung's disease, restricting the use of deep rectal biopsy to those patients in whom symptoms are most suggestive of Hirschsprung's disease. Acetylcholine esterase histochemistry may be more reliable than rectal manometry in the newborn and premature periods. It is hoped that the combined use of these techniques may make deep rectal biopsy obsolete except in patients with hypoganglionosis.

Acetylcholinesterase↗
PubMed1978

Ano-rectal manometry in the diagnosis of Hirschsprung's disease in infants.

Manometric recordings of internal sphincter activity were performed during distension of the rectum in 10 healthy control infants and in 9 infants with clinical signs of Hirschsprung's disease. In 8 of the healthy infants relaxations of the internal sphincter were obtained, which were maximal 4 to 7 secs after rectal distension. This was also the case in 5 of the patients who were later proven not to have Hirschsprung's disease. In 3 patients no relaxations of the internal sphincter could be recorded. Subsequent rectal biopsy revealed absence of ganglion cells, confirming the diagnosis Hirschsprung's disease. The remaining 3 infants (2 controls and 1 patient) could not be calmed during the examination and the results were inconclusive. It is concluded that ano-rectal manometry is a valuable method of examination in the diagnosis of Hirschsprung's disease in infants. No false results were obtained in this study. Furthermore, it is an easy procedure without risk or discomfort for the patient.

Age Factors↗
PubMed1979

Experience in the diagnosis of Hirschsprung's disease using the anomanometer.

Results of using electromanometry as the sole preoperative diagnostic test for Hirschsprung's disease are presented. Out of a total of 113 infants and children diagnosed to be suffering from the disease, manometry was the initial diagnostic test in 92 infants and children. In nine, manometry confirmed the diagnosis after a colostomy has been done. In 11, barium enema had diagnosed the condition at a peripheral hospital and manometry confirmed the diagnosis. In one, manometry was interpreted as normal, but laparotomy revealed Hirschsprung's disease. Histologic corroboration has been obtained in 97 instances. In 14, no operative treatment was undertaken and, hence, no histologic examination was made; in 2, histologic examination is still pending.

Adolescent↗
PubMed1978

Serum and erythrocyte acetylcholinesterase activity in Hirschsprung's disease.

Acetylcholinesterase activity was measured in the serum and erythrocytes from 22 children. The 12 patients in whom the diagnosis of Hirschsprung's disease was confirmed, had a significantly higher concentration of enzyme in both serum and erythrocytes than the 10 children in whom the diagnosis was excluded. This systemic manifestation, may prove of value in the diagnosis of Hirschsprung's disease.

Acetylcholinesterase↗