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 noninvasive 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.     

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.     

Acetylcholinesterase activity in rectal mucosa and blood in the diagnosis of Hirschsprung's disease

Histochemical and biochemical examinations of rectal biopsy specimens were performed in 13 patients with Hirschsprung's disease and in seven with idiopathic constipation and no megacolon. Serum and erythrocyte acetylcholinesterase (AchE) activity in these two groups was compared with that in 30 normal controls. AchE staining of rectal biopsy specimens proved to be a reliable and convenient diagnostic approach in patients with Hirschsprung's disease. Biochemical assay of AchE activity in rectal biopsy specimens was also of value in the diagnosis, however, there was an overlap between Hirschsprung's disease and idiopathic constipation. Erythrocyte AchE activity correlated well with AchE activity in rectal biopsy specimens. Therefore, assays of erythrocyte AchE activity provided useful additional information, especially in screening tests for Hirschsprung's disease.     

SURGERY FOR CONSTIPATION

Over a period of 30 years a total of 27 patients have been subjected to partial or complete colectomy and anastomosis for constipation. Patients fall into four groups: (i) functional constipation; (ii) adult megacolon; (iii) megasigmoid and (iv) persistent Hirschsprung's disease. The first two groups comprised 17 patients with resistant constipation, with or without megacolon or dolichocolon. Seven (41%) of these patients subsequently required operation for acute small-bowel obstruction due to adhesions. In two patients a permanent ileostomy was necessary for persistent rectal inertia after colectomy. The functional results in these first two groups were good. The third and fourth groups had similar presenting features; five had megasigmoid, and in these resection of the sigmoid colon gave good results. The remaining five patients with proven Hirschsprung's disease responded well to a pull-through resection (4) and to colectomy and anastomosis (1).     

The enteric nervous system in chagasic and idiopathic megacolon

Chagas disease frequently causes megacolon. We investigated the enteric nervous systems in patients with chagasic megacolon compared to idiopathic megacolon and controls. Surgical specimens were obtained from 12 patients with chagasic megacolon (1 woman, 11 men, age range 41 to 72 y) and 9 patients with idiopathic megacolon (3 women, 6 men, age range 39 to 68 y), undergoing surgery for intractable constipation. A control group of 10 patients (9 women, 1 man, age range 43 to 75 y) undergoing left hemicolectomy for nonobstructing colorectal cancer was also studied. Colonic sections were investigated by conventional and immunohistochemical methods, also taking into consideration the presence of lymphocytes. Compared to controls, the 2 megacolon groups showed a decrease of enteric neurons (not due to increased apoptosis) and of enteric glial cells (all more important in chagasic patients). The interstitial cells of Cajal subtypes were decreased but not absent in megacolons, although an increase of the intramuscular subtype was found, suggesting a possible compensative mechanism. An increased amount of fibrosis was found in the smooth muscle and the myenteric plexus of chagasic patients compared to the idiopathic megacolon and the control group. A mild lymphocytic infiltration of the enteric plexuses (more evident in Chagas disease) was also found in megacolons but not in controls. Patients with chagasic megacolon display important abnormalities of several components of the enteric nervous system. Similar alterations, although of lesser severity, may be found in patients with idiopathic megacolon.     

The mild form of Hirschsprung's disease (short segment): fourteen-years experience in diagnosis and treatment.

From 1966 through 1980, 39 children were treated with anorectal myectomy for the mild form of Hirschsprung's disease. The mild form of Hirschsprung's disease is differentiated from the severe one mainly on clinical grounds. Of the entire group, only four patients needed a Duhamel procedure, one to three years after myectomy. One patient still uses occasional enema (11 years after operation). The remaining 34 patients are symptom-free (1--14 years after operation). Anorectal myectomy is the treatment of choice in the management of the mild form of Hirschsprung's disease.     

Role of anorectal myectomy in the treatment of short segment Hirschsprung's disease in young adults

Basic pathological disorder in Hirschsprung's disease (HD) comes into being by the abnormal innervations of the aganglionic distal intestine. The nonserious forms of this congenital disease may reach the young by proceeding with chronic, obstinate constipation that does not respond to diet. Myectomy of the short segment of aganglionic part may provide diagnostic and therapeutic value. Between 1993 and 2003, anorectal posterior myectomy was performed in 19 patients diagnosed with HD. The mean age of patients was 23 years. Previous or concomitant anterior resection was added to the procedure in seven cases due to dolicomegacolon. In addition to the deficiency of anorectal inhibitor reflex (ARIR) among all patients, the frequency of defecation was 12.5 days (range, 6-30 days) before operation. Anorectal length before widening was an average of 5.5 cm (range, 2-8 cm). All pathologic specimens were found to be aganglionic. Postoperatively, the mean of first regular defecation interval was 1.5 days (range, 1-3 days). Involution of rectum was evaluated with a rectal examination and barium enema X-rays during follow-up. ARIR of patients also returned to normal. In conclusion, anorectal posterior myectomy is an effective operation in diagnosis and treatment of short-segment HD. Furthermore, colon resection annexed to this operation, in the condition of dolicomegacolon, is also used to remove obstinate constipation.     

THE CURRENT PRACTICE OF PAEDIATRIC SURGERY IN MALAYSIA

This 10-year review of surgical conditions In Infants at the University Hospital, Kuala Lumpur, highlights some of the more common problems encountered and outlines their management. Anorectal agenesis and Hirschsprung's disease were seen relatively more frequently than other anomalies of the gastrointestinal tract. The management of these two conditions and the operation of colostomy and its complications are singled out and presented In some detail.     

Rectal myectomy in Hirschsprung disease: a decade of experience.

Short-segment aganglionosis is becoming more frequently recognized in view of more sophisticated diagnostic techniques, as well as the greater clinical awareness of the entity. A series of 37 patients who underwent rectal myectomy during a ten-year period was reviewed. Selected patients with short-segment aganglionosis, proved by muscle biopsy, responded well to a single transanal operation that required an overnight hospital stay. The complications were minimal, and the cure or marked improvement has persisted for as long as 11 years. When major surgery of an abdominoperineal pull-through type was required after the myectomy (four patients), no technical difficulties were encountered. Within the limits of its applicability, rectal myectomy has a definite place in the treatment of short-segment Hirschsprung disease.     

A simplified approach to diagnostic rectal biopsy in infants and children

During a two year period, ninety-four patients underwent 103 rectal biopsies using the suction capsule technic (71 patients for diagnosis of Hirschsprung's disease and 23 to aid in the diagnosis of various neurologic storage disorders). In nine prospective and eighteen retrospective cases the suction biopsy accurately determined the diagnoses of Hirschsprung's disease. No false-positive or false-negative results were encountered. The technic is simple and safe, requiring neither an operating room nor general anesthesia. No complications were seen after this procedure.