342. Famili�res c-zell-carcinom und megacolon congenitum hirschsprung ? eine seltene entit�t des men ii-syndroms

1987 ◽  
Vol 372 (1) ◽  
pp. 949-949
Author(s):  
A. Frilling ◽  
P. E. Goretzki ◽  
E. Heissen ◽  
H. D. R�her
Keyword(s):  
Megacolon Congenitum

Megacolon congenitum (Mb. Hirschsprung)

1993 ◽  
Vol 25 (6) ◽  
pp. 386-388
Author(s):  
R. Morger ◽  
M. Müller
Keyword(s):  
Megacolon Congenitum

Currarino Syndroma, initially misdiagnosed and treated like megacolon congenitum

Open Medicine ◽  
2011 ◽  
Vol 6 (6) ◽  
pp. 723-726
Author(s):  
Ivona Djordjevic ◽  
Danica Jovanovic ◽  
Blagoje Grujic
Keyword(s):  
Molecular Genetic ◽  
Genetic Diagnosis ◽  
Hereditary Disease ◽  
Postoperative Recovery ◽  
Presacral Mass ◽  
Currarino Syndrome ◽  
Radiologic Evaluation ◽  
Definitive Operation ◽  
Megacolon Congenitum ◽  
Sacral Defect

AbstractCurrarino syndrome was first described as a triad by Guido Currarino, an American radiologist in 1981. It is an autosomal dominant hereditary disease known by the triad of anorectal stenosis, anterior sacral defect, and a presacral mass that is most often an anterior sacral meningocele.We represent a 3 year boy with refractory constipation from birth, which had been wrongly diagnosed and treated as Hirschsprung’s disease since early childhood. The patient underwent urgent colostomy because of water intoxication due to bowel irrigation, and following investigations (CT, MRI) revealed anorectal anomaly and presacral mass compatible with Curarrino’s syndrome.Definitive operation was done with perineal approach, posterior sagittal anorectoplasty, with interval colostomy closure 2 months after perineal operation. An uncomplicated postoperative recovery ensued. The patient’s bowel habits successfully returned to normal soon after the operation. A multidisciplinary diagnostic and therapeutic protocol is presented. Main points are rectal examination, sacrum x-Ray, molecular genetic diagnosis, radiologic evaluation of every member of Currarino syndrome families, CT scan, magnetic resonance (MR) evaluation of patient spine and pelvis and suction rectal biopsies. If the mass is a meningocele, colostomy and neurosurgical exploration should precede anoplasty due to the risk of meningitis.

scholarly journals Hirschsprung's disease

2021 ◽  
Vol 32 (5-6) ◽  
pp. 457-461
Author(s):  
T. Z. Gurevich
Keyword(s):  
Hirschsprung's Disease ◽  
Hirschsprung’S Disease ◽  
Megacolon Congenitum ◽  
Apparent Reason

Under the name idiopathic enlargement of the large intestines or Hirschsprung's disease, Megacolon Congenitum, a kind of levitation is known, the essence of which is that the large intestines appear to be enlarged in one stretch or another, sometimes of colossal dimensions, for no apparent reason that could cause such an expansion. The walls of the dilated segment of the intestines are usually thickened, hypertrophied.

Sign in / Sign up

Export Citation Format

Share Document