exudates and Hirschsprung-Disease

The authors evaluated the safety and benefits of 1-stage pull-through in comparison with staged repair of Hirschsprung's disease under circumstances prevailing in a developing country.. Forty-nine patients were treated for Hirschsprung's disease during a 7-year period between January 1991 and March 1998 at our institution, which is a tertiary referral center. Nine patients were excluded from the study, and the medical records of the remaining 40 patients were reviewed.. Eighteen patients including 7 neonates underwent 1-stage pull-through, and 22 patients underwent staged correction. There was no mortality for patients undergoing one-stage treatment, but there was 1 death caused by anastamotic leak after a 2-stage repair. There was no substantial difference in the incidence of complications (38.8% v 45.45%) and the need for additional surgical procedures (33.5% v 45.45%) between the 2 groups. Seventy-one percent after 1-stage treatment and 80% after staged treatment had a satisfactory functional result, and the incidence of incontinence was 14% and 10%, respectively. Overall, the incidence of postoperative enterocolitis was low (7.5%).. One-stage correction of Hirschsprung's disease is a safe procedure in all age groups. It offers economical and social advantages to families in developing countries. Benefits of 1-stage treatment include avoidance of multiple operations, elimination of complications associated with a colostomy, shorter duration of hospital stay, and completion of treatment at an earlier age. It is advisable to continue postoperative anal dilatation for a minimum period of 6 months to 1 year to reduce the incidence of enterocolitis.

Topics: Anastomosis, Surgical; Child, Preschool; Colostomy; Developing Countries; Digestive System Surgical Procedures; Female; Follow-Up Studies; Hirschsprung Disease; Humans; Infant; Infant, Newborn; Malaysia; Male; Minimally Invasive Surgical Procedures; Rectum; Survival Rate; Treatment Outcome

Mowat-Wilson syndrome (MWS) is a recently delineated mental retardation; a multiple congenital anomaly syndrome characterised by a typical facial gestalt, Hirschsprung disease or severe constipation, genitourinary anomaly, congenital heart defects, agenesis of corpus callosum and eye defects. Some cases also present with epilepsy, growth retardation with microcephaly and speech impairment. MWS was first described in 1998 by Mowat et al, and approximately 180 cases have been reported as of August 2008. The syndrome occurs as a result of heterozygous mutations or deletions in the zinc finger E-box-binding homeobox 2 gene, ZEB2, previously called ZFHX1B (SIP1). Most cases reported so far were sporadic occurrences; however, rare cases of sibling recurrence have been cited. The facial phenotype is particularly important for the initial clinical diagnosis and provides the hallmark, warranting ZEB2 mutational analysis even in the absence of Hirschsprung disease. We present the first two molecularly confirmed Malaysian MWS patients, one of whom has a novel mutation.

Topics: Abnormalities, Multiple; Child; Child, Preschool; Constipation; Epilepsy; Female; Gene Deletion; Hirschsprung Disease; Homeodomain Proteins; Humans; Malaysia; Repressor Proteins; Zinc Finger E-box Binding Homeobox 2