RESUMO
Total colonic aganglionosis occurring together with malrotation is a rare occurrence and may pose diagnostic and management dilemmas for the pediatric surgeon. We report the case of a new born, who was operated at the age of three days for malrotation with volvulus, treated by Ladd procedure. Postoperatively, we noticed persistent abdominal distension and emission of a small amount of meconium every 4 to 5 days. The barium enema showed a non-functional microcolon. Surgical exploration on the 24th day found an ileo-ileal transition zone located 60âcm distal to the ligament of Treitz. Extemporaneous biopsies from the colon and mid-ileum confirmed the absence of ganglion cells. We performed an ileostomy at 50âcm from duodeno-jejunal flexure. Unfortunately, the patient succumbed to nosocomial infection at 33 days of age.This case was a challenging scenario for us where a diagnosis of complicated malrotation had obscured the Hirschsprung's disease.
Assuntos
Colo/anormalidades , Doença de Hirschsprung , Ileostomia , Doenças do Recém-Nascido , Obstrução Intestinal , Volvo Intestinal/cirurgia , Intestinos , Enema Opaco/métodos , Biópsia/métodos , Colo/diagnóstico por imagem , Colo/fisiopatologia , Infecção Hospitalar/diagnóstico , Infecção Hospitalar/etiologia , Evolução Fatal , Feminino , Doença de Hirschsprung/diagnóstico , Doença de Hirschsprung/fisiopatologia , Doença de Hirschsprung/cirurgia , Humanos , Ileostomia/efeitos adversos , Ileostomia/métodos , Recém-Nascido , Doenças do Recém-Nascido/diagnóstico , Doenças do Recém-Nascido/fisiopatologia , Doenças do Recém-Nascido/cirurgia , Obstrução Intestinal/diagnóstico por imagem , Obstrução Intestinal/etiologia , Obstrução Intestinal/fisiopatologia , Volvo Intestinal/diagnóstico , Volvo Intestinal/etiologia , Intestinos/anormalidades , Intestinos/inervação , Intestinos/patologia , Intestinos/fisiopatologiaRESUMO
INTRODUCTION: Angiolymphoid hyperplasia with eosinophilia (HALE) is a rare, benign proliferative vascular disorder of unknown etiology which typically presents as tumor-like nodules of the skin or subcutaneous tissue. It concerns mostly young female adults. We report a case of HALE located on the ear auricle diagnosed by biopsy. The outcome was total regression. OBSERVATION: An 81-year-old male patient presented with a lesion of the whole ear auricle. A diagnosis of HALE was confirmed histologically on biopsy. The evolution was marked by spontaneous and complete regression in 2 months. There was no recurrence after 1 year of follow-up. DISCUSSION: The etiology of this disease remains hypothetical. It poses real diagnostic problems with other types of proliferation, but its main differential diagnosis is Kimura disease. Its treatment has not been codified yet. Spontaneous regression is possible and justifies following the patient from 3 to 6 months, before a possible surgery.
