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Diagnosis of Hirschsprung's Disease by Immunostaining Rectal Suction Biopsies for Calretinin, S100 Protein and Protein Gene Product 9.5
Published on: April 26, 2019
Hirschsprung's disease: 13 years' experience in 112 patients from a single institution
Alessio Pini Prato1, Valerio Gentilino, Camilla Giunta
1Department of Pediatric Surgery, IRCCS G. Gaslini Institute, Largo G. Gaslini, 5, 16147, Genoa, Italy. apinipr@tin.it
Insights
Hirschsprung's disease treatment outcomes vary by disease length. Classic forms show better continence and psychological results, while ultralong forms improve with growth, highlighting the need for ongoing care.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Clinical Outcomes Research
Background:
- Hirschsprung's disease, though curable, presents significant morbidity.
- Varied outcomes are reported, necessitating further investigation into treatment efficacy.
Purpose of the Study:
- To evaluate the outcomes of the pull-through procedure for Hirschsprung's disease.
- To compare results based on age at surgery and length of aganglionosis.
Main Methods:
- Retrospective review of 151 patients undergoing pull-through surgery since 1993.
- Data collection via questionnaires and medical record review.
- Analysis of complications, continence, and patient-reported outcomes.
Main Results:
- 25% of patients experienced complications, including enterocolitis (13-59%) and constipation (15%).
- Excellent to good continence was achieved in 84% of classic and 68% of ultralong forms.
- Classic forms had better psychological and cosmetic outcomes; ultralong forms showed functional improvement with growth.
Conclusions:
- Early diagnosis and prompt treatment are crucial for minimizing morbidity and complications.
- Long-term follow-up is essential, especially for ultralong forms, to manage progressive functional improvements.
- Treatment outcomes for Hirschsprung's disease are influenced by disease extent, underscoring the need for tailored care.
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.
