Craniotomy in Klippel–Trenaunay Syndrome: Concerns and Challenges

Background: Klippel–Trenaunay Syndrome (KTS) is a rare congenital disorder characterized by cutaneous capillary malformations, soft tissue and bone hypertrophy, and venous varicosities. Multisystem involvement of this syndrome mandates adequate preparation and planning, with meticulous conduct of anesthesia, and surgery in these patients to achieve favorable outcomes.

Case Description: A 17-year-old girl presented for excision of right frontal calvarial hemangioma measuring 8 cm × 9 cm × 5 cm with intracranial extension and associated Chiari-1 malformation. On examination, her neurocutaneous markers suggested classical KTS-presence of distinct, linear bordered port-wine stains. She had lymphoedema of bilateral lower limbs and disproportionately enlarged feet with club feet deformity. Solid organ involvement and congenital heart disease were ruled out. Adequate blood products were arranged in view of anticipated blood loss. Standard anesthetic induction was performed. In view of Chiari malformation and anticipated soft tissue changes in the airway, intubation was performed using C-MAC videolaryngoscope. Patient was positioned carefully and surgery was performed uneventfully.

Conclusion: Patients with KTS pose challenges during vascular access necessitating use of ultrasonography to diagnose and avoid inadvertent injury to superficial arteriovenous fistulous malformations. Similar concerns exist during scalp block regarding inadvertent local anesthetic injection into occult subcutaneous scalp hemangiomas. Presence of associated Chiari-1 malformation and proneness for pathological fractures requires care during intubation and positioning. Oropharyngeal neurofibromatosis lesions warrant preoperative airway examination using indirect laryngoscopy to understand the potential airway difficulty and formulate intubation plan. Calvarial hyperostosis can result in difficulty during craniotomy. Proximity of the hemangioma to the superior sagittal sinus places the patient at risk of venous air embolism.

Publication History

Article published online:
25 March 2020