Hirschsprung's disease in Japan: analysis of 3852 patients based on a nationwide survey in 30 years

Presented at the 35th Annual Meeting of the American Pediatric Surgical Association, Ponte Vedra, Florida, May 27-30, 2004.
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Abstract

Background/Purpose

The introduction of laparoscope and transanal endorectal pull-through has caused a revolution in the operative procedures for Hirschsprung's disease. To study the changing profile of Hirschsprung's disease in Japan, the authors carried out a national survey.

Method

Patient data were collected in 3 phases: group 1, 1628 patients between 1978 and 1982; group 2, 1121 patients between 1988 and 1992; and group 3, 1103 patients between 1998 and 2002, respectively.

Results

The incidence was 1:4697, 1:5544, and 1:5343 and the male/female ratio was 3.0:1, 3.4:1, and 3.0:1 in each group, respectively. Patients weighing less than 2500 g at birth increased to 10.4% in group 3, whereas they were 6.5% in group 2 and 5.5% in group 1. The patients with a family history also increased to 6.0% in group 3, in comparison with 2.8% in group 2 and 3.0% in group 1. The incidence of associated anomalies increased over time, 11.1% in group 1, 16.3% in group 2, and 21.2% in group 3. Mutations of genes were found in 4 of the 23 patients examined. The extent of aganglionosis was almost the same in each group. Regarding the definitive operation, the procedures without laparotomy, including transanal endorectal pull-through, increased up to 42.6% in group 3 but 0% in groups 1 and 2. The frequency that a primary operation without stoma was performed also increased. The age at definitive operation decreased in group 3. The incidence of preoperative and postoperative enterocolitis also decreased over time. The mortality was decreased over time, 7.1%, 4.9%, and 3.0% for groups 1, 2, and 3, respectively.

Conclusions

The authors analyzed 3852 Japanese patients over 3 decades. The ratio of patients with a low birth weight, associated anomalies, or a family history was increased in the last 10 years. A primary operation without laparotomy has thus become the procedure of choice for a definitive operation.

Section snippets

Patients and methods

Questionnaires consisting of an individual patient form relating to the general incidence, diagnosis, and treatment of Hirschsprung's disease were sent to 196 departments of pediatric surgery at major hospitals throughout Japan. The patients studied in this survey were limited to cases appearing during the 5-year period from 1998 to 2002. The completed forms of 1103 patients were sent back from 159 hospitals (81.1%), including almost all major services for pediatric surgery in Japan. These data

Results

The estimated incidence of Hirschsprung's disease based on the annual number of cases divided by the annual number of newborns was 1/4697, 1/5544, and 1/5343 and the male/female ratio was 3.0:1, 3.4:1, and 3.0:1 in groups 1, 2, and 3, respectively. The percentage of patients with a birth weight less than 2500 g significantly increased to 10.4% in group 3 as compared with 6.5% in group 2 and 5.5% in group 1. The patients with a family history also significantly increased to 6.0% in group 3 as

Discussion

There have so far been very few reports on multi-institutional surveys of Hirschsprung's disease. The members of a surgical section of the American Academy of Pediatrics collected 1196 cases and showed an estimated incidence of 1/5257 [7]. A recent report from Oman showed a higher incidence of 1/3070 [8], whereas a report from Australia showed a lower incidence of 1/7165 [9]. The incidence in Japan is almost the same as that in the United States and it is also similar to the generally accepted

Acknowledgment

The authors thank Mr Brian Quinn for reading the manuscript and Dr Naoko Kinukawa for helping with statistical analysis.

References (11)

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