Premium
Clinical challenges in the management of a prenatally diagnosed cloacal malformation
Author(s) -
Khuja Mehtap,
Nouri Aria,
Wilczyński Jan,
Dzieniecka Monika,
Grzesiak Mariusz,
Podciechowski Lech,
Finke Daria,
Majos Agata,
Stefańczyk Ludomir,
Nowakowska Dorota
Publication year - 2011
Publication title -
congenital anomalies
Language(s) - English
Resource type - Journals
eISSN - 1741-4520
pISSN - 0914-3505
DOI - 10.1111/j.1741-4520.2010.00292.x
Subject(s) - cloaca , omphalocele , oligohydramnios , cloacal exstrophy , medicine , anatomy , genitourinary system , hypoplasia , fetus , perineum , abdominal distension , biology , pregnancy , surgery , bladder exstrophy , genetics
Cloacal dysgenesis sequence is a severe malformation of the primitive cloaca and is characterized by a phallus‐like structure, smooth perineum and the absence of genitourinary and anal orifices. It is usually accompanied by oligohydramnios, kidney dysplasia, and pulmonary hypoplasia. We present a case of a 29‐year‐old woman who was referred at 26 weeks of gestation due to an enlarged fetal abdominal circumference. Investigations revealed the presence of fetal ascites, intrapelvic cysts, calcified meconium, severe oligohydramnios and a 46XX karyotype. Fetal abdominal parecentesis performed on several occasions failed to reduce intra‐abdominal pressure. To our knowledge this case represents the first variation of cloacal dysgenesis sequence to contain three dysmorphic structures along with the common findings of this anomaly.