Pathophysiologic and anesthetic correlations

'Prune-Belly" is the name given to the disease which is characterized by a congenital wrinkled appearance of the abdomen. Usually, a triad of congenital anomalies highlights the components of the prune-belly syndrome. This triad consists of undescended testicles, abdominal musculature deficiency and urinary tract abnormalities. The previously described triad of the syndrome is by no means the total spectrum of the disease. Prune-belly syndrome is also associated with diseases of the respiratory, cardiovascular skeletal, gastrointestinal and central nervous systems. Associated diseases of major body systems which are caused by renal and cardiopulmonary anomalies, as well as the surgical procedure to be performed, influence the plan for the administration of anesthesia. The nature of the genitourinary diseases in the prune-belly baby may obviate the need for surgical intervention as early as the neonatal period of life. Although this congenital disease occurs with some rarity, the grvity of the syndrome demands an in-depth knowledge of its pathophysiology in order to assure uncomplicated anesthetic care. Astute surveillance during preanesthetic, anesthetic, and postanesthetic management is germane to the prevention of mishaps. The congenital deformity characterized by a wrinkled and deformed abdomen was originally described by Frohlich in 1839.14 In 1895, Parker noted a collection of three anomalies: the undescended testicles, urological obstructive diseases and abdominal wall deficiency.4 The term "prune-belly" was aptly coined for the syndrome by Osler.3-5 Within succeeding years and following numerous urological studies, a myriad of nomenclature evolved. Other names for the syndrome include the triad syndrome, abdominal musculature deficiency syndrome, Eagle-Barrett syndrome, mesenchymal syndrome dysplasia, 6 and congenital absence of the abdominal musculature syndrome.7 (See Figure 1.)