The prune-belly syndrome (PBS) usually is described as a deficiency of the anterior abdominal muscle involving bilateral cryptorchidism and urinary tract malformations. In this report, we will present an eleven-month-old boy with PBS associated with a complex cardiac anomaly. A bilateral bidirectional Glenn shunt was performed with the diagnosis of isolated dextrocardia, single ventricle, pulmonary atresia, incomplete A-V septal defect, hemiazygos continuity, persistent right superior vena cava, patent ductus arteriosus-dependent pulmonary blood flow. The patient required special consideration for postoperative pulmonary care.
Key words:
Prune-belly syndrome - Congenital heart disease - Open heart surgery - Glenn procedure
References
-
1
Geary D, Mc Lusky I B, Churchill B M, Mc Lorie G.
A broader spectrum of abnormalities in the prune-belly syndrome.
J Urol.
1986;
135
324-326
-
2
Burbige K, Amodio J, Berdon W E, Hensle T W, Blanc W, Lattimer J K.
Prune-belly syndrome: 35 years of experience.
J Urol.
1987;
137
86-90
-
3
Reinberg Y, Shapiro E, Manivel J C, Manley C B, Pettinato G, Gonzales R.
Prune-belly syndrome in females: A triad of abdominal musculature deficiency and genital systems.
J Pediatr.
1991;
118
395-398
-
4 Yoshida M, Matsumoro M, Shintaku Y, Yura Y, Kanamori T, Matsushita K, Nonogaki T, Hayashi M, Tauchi K. Prenatally diagnosed female prune-belly syndrome associated with tetralogy of Fallot. 1995 39(2): 141-144
-
5
Kabakus N, Serhatlioglu S, Akfirat M, Kazez A, Aydinoglu H, Ozercan I, Aygun A D.
Prune-belly syndrome associated with extra-abdominal abnormalities in a 7-year-old boy.
Turk J Pediatr.
2000;
42
158-161
-
6
Karamanian A, Kravath R, Nagashima H, Gentsch H H.
Anaesthetic management of prune-belly syndrome.
Br J Anaesth.
1974;
46
897-899
-
7
Pagon R A, Smith D W, Shepard T H.
Urethral obstruction malformation complex: a cause of abdominal muscle deficiency and the prune belly.
J Pediatr.
1979;
94
900-906
-
8
Ewig J M, Griscom N T, Wohl M EB.
The effect of the absence of abdominal muscles on pulmonary function and exercise.
Am J Respir Crit Care Med.
1996;
153
1314-1321
Received for Publication: March 26, 2001
Prof. Rıza Doğan, MD
Department of Thoracic and Cardiovascular Surgery
Hacettepe University, Faculty of Medicine
06100 Ankara, Turkey
Telefon: 90 (312) 311-73 77
Fax: 90 (312) 311-09 95
eMail: rdogan@hacettepe.edu.tr
