Seiichi Goto

Diagnosis and treatment of Hirschsprung's disease in Japan. An analysis of 1628 patients.

A nationwide survey on Hirschsprungs disease was conducted to clarify the recent trends of diagnosis and treatment of Hirschsprungs disease seen in Japan from 1978 to 1982. A total number of 1628 cases were collected from 135 medical institutions. Estimated incidence of Hirschsprungs disease was 1/4697. In diagnosis, anorectal manometry was carried out in 64.7% of the patients and histochemistry in 28.7%. Barium enema with manometry was most frequently used in combination of the methods. A total of 48.7% of the patients were diagnosed in the first month of life. The overall rate of creation of colostomy was 61.3% in aganglionosis extended to the sigmoid colon. Ret-rorectal transanal pull-through was used most frequently at 57.4%, including Z-shaped anastomosis at 30.4%, then endorectal pull-through at 27.6% as the definitive operation. Considerably high incidence (12.1%-33.7%) of postoperative enterocolitis was noted after major operative procedures, but the mortality rate was low (1.8%-2.4%) except total colonic with or without small bowel aganglionosis. The large number of patients studied in the present survey reveals that there still is much room for improvement in the diagnosis and treatment of Hirschsprungs disease, especially in total colonic with or without small bowel aganglionosis.

Total colonic aganglionosis with or without small bowel involvement: An analysis of 137 patients

A total of 137 patients with total colonic aganglionosis involving or not involving the small intestine (TCSA) were analyzed to clarify the recent trends of TCSA in Japan. The percent of TCSA out of all types of Hirschsprungs disease was 8.4%. Male to female ratio in TCSA was 1.5:1. Open biopsy was relatively frequent for diagnosis, the rate being 40.9%. A total of 67.4% of the TCSA patients were diagnosed in the first month of life. Seventy-two percent of the patients underwent enterostomy in the neonatal period. Definitive operations were performed in 67 patients. Sixty underwent retrorectal transanal pull-through, most of which were Martins procedure (56 cases). Mortality rate after Martins procedure was 12.5%. Sixty-four percent of the patients with enterostomy alone did not survive. This study shows the recent trends of TCSA and indicates there is still much room for improvement in the clinical management of TCSA.

Reduction of lower esophageal sphincter pressure with stamm gastrostomy

Although recent clinical studies suggest an association between Stamm gastrostomy and gastroesophageal reflux (GER), no significant experimental data is available. This study evaluates alterations in lower esophageal sphincter (LES) pressure after Stamm gastrostomy in cats. Eight male cats (average weight 3 to 4 kg) were induced under general anesthesia using 20/mg/kg ketamine intramuscularly (IM). Esophageal manometrics were determined for each animal using a continuous perfusion catheter and recording system. Three measurements were taken for each animal. Stamm gastrostomy was then placed in the anterior wall of the stomach two thirds of the way down from the fundus. This was tacked to the anterior abdominal wall 3.0 cm lateral to the midline at the appropriate longitudinal level. Esophageal manometry was repeated after abdominal closure. The animals were awakened and returned to their cages for ad libidum feedings. The animals were reanesthetized with ketamine and manometrics repeated at 7 and 14 days. Each animal served as its own control. Five animals underwent barium esophagram 14 days postoperatively to evaluate for GER. Preoperative mean LES pressure measured 11.4 +/- 3.5 torr. This decreased to 7.8 +/- 2.8 torr immediately after Stamm gastrostomy (P less than .025). When evaluated at 1 and 2 weeks after gastrostomy, further decrease in LES pressures to 6.6 +/- 1.6 torr and 4.8 +/- 1.6 was observed (P less than .02 v preoperative). Three of five cats undergoing barium swallow demonstrated significant reflux radiographically. Stamm gastrostomy caused significant reduction of the LES pressure in all cats studied. This resulted in clinical GER as documented by barium swallow in three of five cats.(ABSTRACT TRUNCATED AT 250 WORDS)

Surgical treatment of total colonic aganglionosis: Efficacy of aganglionic patch enteroplasty in the rat*

This study evaluates ileo endorectal pull-through (IEP) with and without an aganglionic colon patch in an experimental rat model for the surgical treatment of total colonic aganglionosis (TCA). Animals were randomly assigned to Group 1 (no patch, No. = 6), Group 2 (right colon patch, No. = 8), Group 3 (transverse colon patch, No. = 7), Group 4 (left colon patch; No. = 6), sham operation (SH, No. = 6), and unoperated controls (UC, No. = 10). Change in percent body weight at 4 weeks after operation was -30.9 +/- 3.68% in Group 1, +5.1 +/- 1.67% in Group 2, -3.4 +/- 3.96% in Group 3, -1.8 +/- 4.17% in Group 4, and +12.7 +/- 1.54% in SH (Group 1 v other groups: P less than .001). Restoration of initial body weight occurred in 100% in Group 2 and SH (8/8 and 6/6, respectively), 50% in Group 4 (3/6), 42.9% in Group 3 (3/7), and 0% in Group 1 (0/6). Transit time (stomach to anus) was significantly shorter in Group 1. All rats in patched groups had a prolonged transit time compared with Group 1. Manometric studies in IEP rats showed favorable anal canal pressures, which were slightly lower than SH and UC. Water and Na+ absorption were significantly greater in patched groups. Rats with a right colon patch (Group 2) showed slightly greater absorption at 4 weeks. These data suggest that an aganglionic colon patch may be an important adjunct in the surgical treatment of TCA.

Distribution and localization of glia fibrillary acidic protein in colons affected by Hirschsprung's disease

The distribution and localization of glial fibrillary acidic (GFA) protein were examined by means of immunohistochemistry in normoganglionic, oligoganglionic, and aganglionic segments of colons from 25 patients with Hirschsprungs disease, including four cases of long segment aganglionosis. In normoganglionic segments, GFA protein-positive glial cells were densely distributed within the myenteric plexus, but sparse in the submucous plexus. Aganglionic segments were completely devoid of glial cells with GFA protein immunoreactivity, coinciding with the lack of enteric ganglia. Instead, GFA protein was found specifically in association with the hypertrophic nerve fasciculi and their branches, which were mainly located in the intermuscular zone and submucosal connective tissue in the distal aganglionic segment of diseased bowels. However, two types of short and long segment aganglionosis differed in the distribution pattern of GFA protein; the extrinsic nerve fasciculi in short segment disease extended toward the normoganglionic segment, but in long segment disease they did not reach this area. A moderate number of GFA protein-positive fasciculi were observed within the circular muscle layer of proximal aganglionic and oligoganglionic parts in short segment aganglionosis, while no immunoreactive fasciculi were encountered within the circular muscle layer of the corresponding parts in long segment aganglionosis. Immunohistochemistry for GFA protein can be of excellent diagnostic value for the aganglionic colon with Hirschsprungs disease, since GFA protein immunohistochemistry discloses exclusively extrinsic, hypertrophic nerve fasciculi, characteristic of the bowel in cases of Hirschsprungs disease.

Is preserving the entire aganglionic colon reasonable in the surgicaltreatment of total colonic aganglionosis

Clinical reports describe an increased incidence of severe enterocolitis in infants with total colonic aganglionosis (TCA) following the Martin extended Duhamel procedure using the entire aganglionic colon. This study evaluates the efficacy of this procedure in an experimental model of TCA in comparison with an antimesenteric aganglionic colon patch in rats. TCA was produced by serosal application of 0.1% benzalkonium chloride in 18 Sprague-Dawley rats (250 g). Five additional rats served as operated controls. Ileoanal pull-through was performed in 18 TCA rats, in conjunction with the Martin extended Duhamel procedure using the entire colon in six rats, with an aganglionic colon patch in nine (using the right [3 rats], transverse [3 rats] and left [3 rats] colon), and without other procedures in three rats. Animals were evaluated for survival, weight change, food intake, stool consistency and volume, barium enema, complete blood cell count (CBC), total protein, and serum electrolytes at 4 and 12 weeks. Survival was 83% (5/6) rats with the Martin procedure, 100% in the nine rats with various colon patches, zero in three rats with ileoanal pull-through alone, and 100% in controls. Rats with the Martin procedure gained 2.2 +/- 3.27% of preoperative weight, while controls gained 11.2 +/- 0.52% at 4 weeks. All other rats showed an early weight loss. At 12 weeks, right and transverse colon patched rats had weight gain. Blood count and laboratory studies were similar in each group. Barium enema showed rapid transit in rats with ileoanal pull-through, and slower transit in rats with colon patches or the Martin procedure.(ABSTRACT TRUNCATED AT 250 WORDS)

Renal Cell Carcinoma in Japanese Children

Renal cell carcinoma in children is extremely rare compared to Wilms tumor. We report on a 7-year-old Japanese boy with left renal cell carcinoma. Since the disease was clinical stage I, nephrectomy with tumor extirpation was performed after a short course of initial chemotherapy had been administered. The patient is free of disease 3 years postoperatively. In a review of the Japanese literature we found 71 reports of renal cell carcinoma in children less than 15 years old. Mean patient age at occurrence was 8.1 years, there were no differences in regard to the sex or the affected side, and the most frequent symptom was the presence of a mass. We stress the early establishment of diagnosis in children with a persistent abdominal mass, hematuria and flank pain, since surgical treatment leads to a favorable prognosis only in the early stage of renal cell carcinoma.

Chemically induced bowel denervation improves survival in short bowel syndrome

This study evaluates the effect of chemically induced bowel denervation on survival, weight gain or loss, transit time, and d-xylose absorption in rats following 80% small bowel resection. Forty-three male Sprague-Dawley rats (150 g) underwent 80% midsmall bowel resection and anastomosis. Twenty rats were short bowel controls (group I). In 23 rats (group II), a 2.0 cm segment of jejunum proximal to the anastomosis was denervated by application of 0.1% benzalkonium chloride (BK) for 30 minutes. Ten additional rats underwent sham laparotomy without bowel resection. Five remained untreated (group III) and in 5 (BK) denervation was added (group IV). Bowel denervation was confirmed by histologic study in all (BK) rats. Weight and daily food and water intake were measured for 30 days and the groups compared. Weight in group I was 43.8 +/- 52.9 g, group II 95.0 +/- 50.1, (P less than .005), group III 177 g, and group IV 175 g. Food intake was greater in group I than II (P less than .05) and was similar to groups III and IV. Water intake calculated as animal weight (g)/mL H2O ingested was lowest in group I (P less than .05). Mortality was 30% in group I (6/20) and only 8.6% in group II (2/23). No deaths were observed in unresected controls (III and IV). Twenty-four additional rats were evaluated for d-xylose absorption and transit time by bringing out a loop enterostomy 10.0 cm from the Ligament of Treitz. Twelve rats were ostomy controls (group V).(ABSTRACT TRUNCATED AT 250 WORDS)

Distribution of neuropeptide Y-like immunoreactivity in the normoganglionic and aganglionic segments of human colon

SummaryThe localisation and distribution of neuropeptide Y (NPY)-like immunoreactivity were studied by use of immunohistochemical methods in gut tissues from 19 patients with Hirschsprungs disease, including 4 cases of long segment aganglionosis. In the normoganglionic segment, immunoreactive cell bodies and nonvaricose processes were seen within both myenteric and submucous plexuses. A scarce supply of varicose fibres was found in the lamina propria mucosae, muscularis mucosae and longitudinal muscle layer. NPY fibres were more frequently encountered in the circular muscle layer, although with a weakly immunostaining intensity. In addition, blood vessels in the submucosal connective tissue were surrounded by a typical plexus of varicose, NPY-positive fibres. Immunoreactive endocrine cells could be detected in the colonic epithelium. In the aganglionic segment, numerous nerve fasciculi comprising a small to moderate number of NPY fibres with varicosities were observed throughout the entire layer of the colonic wall. A few varicose, NPY-positive fibres were also contained in the relatively large, hypertrophic nerve fasciculi located in the intermuscular zone and submucosal connective tissue. NPY-immunoreactive fasciculi were more densely distributed in the distal aganglionic segment than in the proximal aganglionic one. On the other hand, the distribution of NPY-positive fibres in long segment aganglionosis was quite different from that in short segment type; in cases of long segment type, no immunoreactive nerve fibres were detected within the circular muscle layer of the proximal aganglionic segment near the oligoganglionic segment and only a few fibres were observed within the hypertrophic nerve bundle of the intermuscular zone. The present results suggest that NPY-like immunoreactive nerves in the human colon have a dual origin of intrinsic and extrinsic elements. The origin and nature of extrinsic NPY nerve fibres in the human colon are discussed.

An improved staining technique for acetylcholinesterase activity using rubeanic acid in the diagnosis of Hirschsprung's disease

An improved staining technique for acetylcholinesterase (AChE) activity using rubeanic acid was used to make a clinical diagnosis in 54 children with constipation. Nineteen were thus confirmed to have Hirschsprungs disease. False positive or negative reactions were nil. The sites of AChE activities were in the form of black deposits and the contrast was sharp. This approach should find a wide application for the diagnosis of Hirschsprungs disease.