CASE REPORT M. S. Ismail Æ I. Sharaf Æ C. R. Thambidorai Æ A. Zainal S. Somasundaram Æ S. Adeeb Æ M. Y. Sajjad Æ B. Bilkis L. S. K. Felicia Cardiopulmonary bypass in surgery for complex-combined vascular malformation of the lower limb: case report Accepted: 11 February 2004 / Published online: 2 April 2005 Ó Springer-Verlag 2005 Abstract A 16-year-old boy was referred with features of Parkes Weber syndrome (PWS) involving the right lower limb. He had presented at birth with cutaneous vascular malformations (VM) in the right thigh and at the age of 7 years developed congestive cardiac failure, which was controlled with drugs. He received alpha interferon and steroids during this period without any benefit. He defaulted follow-up and at 12 years of age presented with further enlargement of the VM in the right thigh and leg with skin and soft tissue thickening. At this stage, embolization and subsequent excision of the VM were tried, but the surgery was abandoned be- cause of massive hemorrhage. Over the next 4 years, the boy became totally bedridden because of massive in- crease in the size of the limb, repeated hemorrhages, and secondary infection of the VM. Right hip disarticulation was considered the best option to improve his quality of life. To prevent uncontrollable hemorrhage during sur- gery, the disarticulation was done under cardiopulmo- nary bypass with low circulatory flow. Postoperatively, the patient required intensive care nursing for a week. He is presently ambulatory with crutches. Cardiopul- monary bypass with low flow has been used for treating posttraumatic arteriovenous malformations. However, its use in surgery for PWS has not been reported earlier. Keywords Vascular malformation Æ Parkes Weber syndrome Æ Arteriovenous shunts Æ Cardiopulmonary bypass Introduction Vascular anomalies are currently classified on the basis of their endothelial characteristics into hemangiomas and vascular malformations (VM) [1]. VM are devel- opmental errors composed of dysplastic vessels and are lined by quiescent endothelium. VM are usually present at birth and generally grow proportionately with the child’s development. They may also change in size be- cause of trauma and infection but do not have a pro- liferative phase or a tendency for spontaneous involution. In contrast, hemangiomas are benign tumors that have an early rapid growth phase during the 1st year of life, followed by steady involution over time. Hemangiomas are composed of endothelial cells that have a programmed growth or proliferation pattern, a static phase of normal cellular kinetics, and a prolonged regression phase [2]. The VM are subcategorized based on the predomi- nant type of channel involved and their flow character- istics [3]. The predominant channels involved may be capillaries, lymphatics, veins, or arteries. Complex- combined vascular malformations (CCVM) involve more than one of these channels with propensity for soft tissue and skeletal overgrowth. Eponyms such as Klip- pel–Trenaunay syndrome (KTWS) and Parkes Weber syndrome (PWS) have been used to describe CCVM but unfortunately do not indicate the lesion’s pathology. PWS is a fast-flow CCVM characterized by the presence of capillary-lymphaticovenous malformation with large arteriovenous (AV) shunts and overgrowth of soft tis- sues and bones. It involves the lower limbs more often C. R. Thambidorai (&) Æ S. Somasundaram Æ S. Adeeb M. Y. Sajjad Department of Surgery, Kebangsaan University, Jalan Yacob Latiff, 56000 Kuala Lumpur, Malaysia E-mail: tdorai@pc.jaring.my Fax: +60-3-91737831 A. Zainal Department of Surgery, General Hospital, Kuala Lumpur, Malaysia M. S. Ismail Æ I. Sharaf Department of Orthopaedics, Kebangsaan University, Kuala Lumpur, Malaysia B. Bilkis Department of Paediatrics, Kebangsaan University, Kuala Lumpur, Malaysia L. S. K. Felicia Department of Anaesthesiology, Kebangsaan University, Kuala Lumpur, Malaysia Pediatr Surg Int (2005) 21: 392–395 DOI 10.1007/s00383-005-1367-z