Is there a hidden mortality after one-stage transanal endorectal pull-through for patients with Hirschsprung’s disease?

Abstract

One-stage transanal pull-through (TAP) has become a standard definitive procedure for the treatment of Hirschsprung's disease (HD). Short-term results of this operation seem to be excellent, but long-term outcome is still obscure. We evaluated the morbidity and mortality of our patients with one-stage TAP, and we reviewed the literature. We performed a TAP without a colostomy in 21 patients with HD. The primary outcome measures are age, sex, complications during surgery, enterocolitis (EC) attacks after surgery, postoperative stooling problems and mortality. All patients were called over telephone, and their clinical and functional outcomes were obtained. Case series of TAP in the literature were also reviewed in terms of postoperative problems. Twenty-one patients with full thickness rectal biopsy-proven HD underwent one-stage TAP. Average follow-up was 28 months. One early postoperative EC and three more late attacks of EC were observed. All survived patients had normal bowel habits. Three patients had perianal excoriations, three patients soiling, seven cases required anal dilatations and four patients experienced a diarrhea after surgery. We have been informed that four patients died after discharge from hospital. Two of them were a sudden death (one patient had metabolic problems, the other might have had an EC attack). The cause of death of one patient with an associated Down syndrome was a severe pneumonia, and one other case died of a septic shock of unknown etiology. None of these patients had a diarrhea or abdominal distention, which could have been an evidence of an EC attack prior to their deaths. We observed similar fatal cases, when reviewed the published series in the literature. There might be a hidden mortality within the long-term period after TAP for HD. Therefore, we recommend a close follow-up for all patients with any associated health problem and those from low socioeconomic regions after one-stage pull-through.

Epidemiology of Hirschsprung's Disease in Taiwanese Children: A 13-year Nationwide Population-based Study

“…17 Unfortunately, we did not have the pathological information about the length of the aganglionic segment in our HD cases because the NHI reimbursement database was not originally designed for academic research. In addition, delayed complications or mortality occurring after a long period of time may be missed in data, 28 even though the observational period has been up to 13 years in this study. Despite the limitations above, we believe that the NHI database is still very representative and can provide useful epidemiological information about HD on a national scale.…”

Comparison of transanal endorectal pull-through and laparoscopic assisted transanal endorectal pull-through for Hirschsprung's disease

“…Mısır'da yapılan bir çalışmada da benzer sonuçlar elde edilmiştir (9) . Ülkemizden yayınlanan 21 hastalık bir seride ise üç hastada perianal ekskoriasyon, üç hastada soiling, yedi hastada anal dilatasyon gereksinimi ve dört hastada diyare olduğu görülmüştür (32) . Bu çalışmadaki sonuçlar ile çalışmamızdaki sonuçlar benzerdir.…”

Hirschsprung’s-associated enterocolitis (HAEC)

“…Yoğun bakım koşullarının düzelmesi HEK mortalitesinde de belirgin düzelme sağlamıştır (4) . Ancak HEK önemli bir morbidite nedeni olarak durmaktadır (2,3,9,10,11,73) . Sendromik ve uzun segment hastalarında mortalite riski fazladır.…”

“…HEK sıklığını belirgin fazla olarak (%50) saptamıştır (3) . Bu durum diğerlerince de doğrulanmıştır (10,13,(17)(18)(19)69,73) . Uzun segment HH: Bazı yazarlar belirgin farklı olduğunu ortaya koyarken (10,(19)(20)(21) bazıları bunu doğrulamamıştır (7,13,22) .…”