Hirschsprung’s disease: 13 years’ experience in 112 patients from a single institution

Abstract

Although Hirschsprung's disease is curable, a low mortality and a certain morbidity have been described by several authors. We will present our experience with the treatment of Hirschsprung's disease at Gaslini Children's Hospital. All patients who underwent a pull-through procedure since January 1993 were included. Data were obtained from a comprehensive questionnaire and from the revision of the notes. The results were subsequently described and compared with regard to age and length of aganglionosis. A total of 151 patients underwent a pull-through. One hundred and twelve of these patients completed the forms and were reviewed. Eighty patients had a classic form, 22 an ultralong. Complications occurred in 28 patients (25%). Postoperative enterocolitis was complained by 25 patients (13% colonic and 59% ultralong forms) and constipation by 15 (equally distributed). Excellent to good continence was experienced by 84% of patients with classic forms and by 68% of patients with ultralong forms. A clear improving trend during growth was evident for patients with ultralong forms. Psychological self-acceptance, patients' perspective and cosmetic results proved to be significantly better for patients with classic forms of the disease. Our study confirmed the complications and long-term sequelae that patients with Hirschsprung's can experience. Early diagnosis can minimise morbidity and mortality and prompt and adequate treatment can reduce the incidence of postoperative complications. The parents should be acknowledged regarding the progressive improvements of function that patients gain during growth, particularly in case of ultralong forms, thus strengthening the need for continuative care and close follow-up.

Induction of RET Dependent and Independent Pro-Inflammatory Programs in Human Peripheral Blood Mononuclear Cells from Hirschsprung Patients

“…The diagnosis of HSCR was performed following all clinical and laboratory criteria, as recently revised by our group[76]. In particular, the examination of biological specimens of adequate rectal suction biopsies by the department of pathology included the Acetylcholinesterase (AChE), Lactate Dehidrogenase (LDH) and NADPH-diaphorase histochemical staining.…”

Quality of life and disease-specific functioning of patients with anorectal malformations or Hirschsprung's disease: a review

“…Additionally, a manual search of the references of the selected articles was carried out, which resulted in one more article 44. After thorough inspection of these 30 studies, we decided to exclude those25 30 33 35–37 41 43 that did not include sufficient data to calculate (A) an effect size of the generic QoL between the experimental and comparison groups (objective 1) or (B) a correlation coefficient between QoL and disease-specific functioning (objective 3C), leaving 22 eligible studies 3 8 10–14 23 24 26–29 31 32 34 38–40 42 44 45(see appendix A). We additionally searched in PsycINFO (EBSCOhost interface), Web of Science and ScienceDirect, none of which yielded eligible articles.…”

Per rectal endoscopic myotomy for the treatment of adult Hirschsprung's disease: First human case (with video)

“…The present case report describes the first human experience of PREM for successful treatment of adult Hirschsprung's disease. Surgical treatment of HD, especially the adult variant, remains unsatisfactory and has significant morbidity . PARM has been successfully used for treatment of ultrashort segment HD and for recurrences after pull‐through surgery; but can be used only for the distal 5–8 cm, understandably due to limitations of the transanal surgical approach .…”

“…The disease commonly affects the recto‐sigmoid region (short segment HD), although ultrashort or long variants of the disease have also been described . Standard treatment consists of single or multistage surgical or laparoscopic pull‐through procedures with or without diverting colostomy; and posterior anorectal myectomy (PARM) is a treatment option for ultrashort segment HD …”