Hirschsprung Disease and Intestinal Neuronal Dysplasia Type B: Is There a Difference in the Clinical Presentation of These Two Diseases?

Autor: Cesar Gonçalves A; Department of Surgery - Division of Pediatric Surgery, Botucatu Medical School, São Paulo State University (UNESP), Botucatu, BRA., Camargo de Oliveira B; Department of Surgery - Division of Pediatric Surgery, Botucatu Medical School, São Paulo State University (UNESP), Botucatu, BRA., Patti Sanches Coelho M; Department of Surgery - Division of Pediatric Surgery, Botucatu Medical School, São Paulo State University (UNESP), Botucatu, BRA., Scalfi Caproni E; Surgery, Ouro Verde Hospital, Campinas, BRA., Tuccille Comes G; Department of Surgery - Division of Pediatric Surgery, Botucatu Medical School, São Paulo State University (UNESP), Botucatu, BRA., Marchesan Rodrigues MA; Department of Pathology, Botucatu Medical School, São Paulo State University (UNESP), Botucatu, BRA., Veruska Paiva Ortolan E; Department of Surgery - Division of Pediatric Surgery, Botucatu Medical School, São Paulo State University (UNESP), Botucatu, BRA., Toledo de Arruda Lourenção PL; Department of Surgery - Division of Pediatric Surgery, Botucatu Medical School, São Paulo State University (UNESP), Botucatu, BRA.
Jazyk: angličtina
Zdroj: Cureus [Cureus] 2023 Dec 16; Vol. 15 (12), pp. e50618. Date of Electronic Publication: 2023 Dec 16 (Print Publication: 2023).
DOI: 10.7759/cureus.50618
Abstrakt: Background: Although the signs and symptoms that comprise the clinical presentation of Hirschsprung disease (HD) and intestinal neuronal dysplasia type B (IND-B) are well established, no studies have specifically compared the clinical characteristics presented by patients with these diseases. We compared the clinical pictures of patients with HD and IND-B at the time of histopathological diagnosis.
Methods: This was a single-center, retrospective, analytical, and comparative study. We included 119 patients aged 0-15 years diagnosed with HD or IND-B. Information from the medical records of these patients was retrieved to obtain demographic and clinical information at the time of diagnosis. The data were compared statistically according to the characteristics of the variables.
Results: Sixty-nine patients (58.0%) were diagnosed with HD, and 50 (42.0%) had IND-B. The HD group had significantly more individuals with symptom onset in the neonatal period (p = 0.001), delayed meconium clearance (p < 0.001), failure to thrive (p = 0.02), and acute complications, such as enterocolitis (p = 0.049) or acute abdominal obstruction (p = 0.031), more commonly requiring emergency surgery (p < 0.001). Patients with IND-B were diagnosed at a significantly older age (p = 0.002). They more commonly had chronic constipation as their main symptom (p = 0.004), with local complications, such as evacuation bleeding (p = 0.007).
Conclusion: There were significant differences between the clinical pictures of patients with HD and IND-B. Knowledge of each disease's most common signs and symptoms can help direct diagnostic susception and initial management.
Competing Interests: The authors have declared financial relationships, which are detailed in the next section.
(Copyright © 2023, Cesar Gonçalves et al.)
Databáze: MEDLINE