Total colonic aganglionosis with or without small bowel involvement: a 30-year retrospective nationwide survey in Japan.
OBJECTIVE
We conducted a nationwide survey in Japan, to identify recent trends in the diagnosis and treatment of patients who had total colonic aganglionosis with or without small bowel involvement (TCSA). These data were compared with 2 similar studies conducted within the last 30 years.


METHODS
Patient data were collected in 3 phases; phase I included 135 patients between 1978 and 1982; phase II, 107 patients between 1988 and 1992; and phase III, 101 patients between 1998 and 2002.


RESULTS
The incidence of TCSA was 1:59,059, 1:58,084, and 1:58,375, and the male-female ratios were 1.5:1, 1.5:1, and 2.2:1 in each phase, respectively. Patients with associated anomalies increased from 15.2% (phase I) to 22.8% (phase III). The incidence of preoperative enterocolitis decreased over time. For treatment, Duhamel's procedure and ascending colon patch methods have increased over time, whereas Martin's procedure has decreased. The mortality rate dropped from 40.9% to 15.8%; however, a high mortality rate persists in those cases with small bowel involvement (35.5%).


CONCLUSIONS
A marked decrease in the overall mortality rate was observed during the study period. However, further efforts are still required especially in cases involving aganglionosis extending orally to 75 cm from Treitz's band.
Ieiri
Satoshi
S
Department of Pediatric Surgery, Reproductive and Developmental Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan. satoshi@med.kyushu-u.ac.jp
Suita
Sachiyo
S
Nakatsuji
Takanori
T
Akiyoshi
Junko
J
Taguchi
Tomoaki
T
D000015:Abnormalities, Multiple / Q000453:epidemiology; D000714:Anastomosis, Surgical / Q000379:methods / Q000706:statistics & numerical data; D002423:Cause of Death; D015897:Comorbidity; D004314:Down Syndrome / Q000453:epidemiology; D004760:Enterocolitis / Q000453:epidemiology / Q000209:etiology; D004766:Enterostomy / Q000379:methods / Q000706:statistics & numerical data; D005260:Female; D006306:Health Surveys; D006330:Heart Defects, Congenital / Q000453:epidemiology; D006627:Hirschsprung Disease / Q000175:diagnosis / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery; D006801:Humans; D015994:Incidence; D007231:Infant, Newborn; D007421:Intestine, Small / Q000002:abnormalities*; D007564:Japan / Q000453:epidemiology; D010535:Laparoscopy / Q000379:methods / Q000706:statistics & numerical data; D008297:Male; D009026:Mortality / Q000639:trends; D011183:Postoperative Complications / Q000401:mortality; D012189:Retrospective Studies