Thorac Cardiovasc Surg 2001; 49(6): 378-379
DOI: 10.1055/s-2001-19012
Case Report
© Georg Thieme Verlag Stuttgart · New York

Bidirectional Glenn Shunt in
an Infant with Prune-Belly
Syndrome

R.  Doğan1 , M.  Yılmaz1 , Ü.  Duman1 , S.  Özkutlu2
  • 1Department of Thoracic and Cardiovascular Surgery,
    Hacettepe University, Faculty of Medicine, Ankara, Turkey
  • 2Department of Paediatric Cardiology, Hacettepe University, Faculty of Medicine, Ankara, Turkey
Further Information

Publication History

Publication Date:
17 December 2001 (online)

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.

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

Phone: 90 (312) 311-73 77

Fax: 90 (312) 311-09 95

Email: rdogan@hacettepe.edu.tr