KLIPPEL-TRENAUNAY SYNDROME: A TROUBLESOME CONDITION

BACKGROUND

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder characterized by the triad of

port-wine stain, varicose veins and bony/soft tissue hypertrophy involving an extremity. Beside

the limbs, other areas may be affected, such as spinal cord and neck/oropharynx, which poses

important challenges to the anesthesiologist: airway management, controversial about

neuraxial techniques and potential blood loss. Because of its rarity and the important

anesthetic management considerations it requires, we report a case at our institution.

CASE REPORT

A 47-year- old woman, ASA III, with past medical history of KTS, pulmonary thromboembolism,

anticoagulated with rivaroxaban, hemythyroidectomy and neoadjuvant radiotherapy, was

scheduled for excision of a popliteal sarcoma. On preoperative evaluation she presented no

signs of difficult airway, normal echocardiogram, blood analysis including coagulation profile

were normal. She interrupted rivaroxaban 2 days before surgery.

Surgery was conducted under general anesthesia, induced with propofol and maintained with

sevoflurane. Endotracheal intubation was performed under direct laryngoscopy without

difficulties. Neuraxial technique was not done because we didn’t have any preoperative

imaging study to exclude neurovascular involvement. Hemodynamic stability and pain control

was maintained and no significant blood loss was recorded.

DISCUSSION

Few cases of KTS are published. KTS incidence is low (1:27500) 1 and its anesthetic management

is still a matter of controversy. Despite previous reports of difficult airway management, a

recent study 1 didn’t encountered significant airway difficulties, which was also the case of our

patient. Even if blood loss is a potential problem, especially because these patients are

frequently anticoagulated, our patient didn’t registered significant haemorrhage. As

recommended, we didn’t performed any neuraxial technique because none neuroimaging

study was made before surgery. Still, this possibility can be considered in specific conditions,

even if it’s controversial 2 .

REFERENCES

1. Barbara D, Wilson J. Anesth Analg. 2011; 113: 98-102

2. Holak E, Pagel P. J Anesth. 2010; 24: 134-8

LEARNING POINTS

Key aspects must be kept in mind in the management of these patients: potential difficult

airway (even if rare), massive blood loss that should be anticipated and neuraxial techniques

that may be considered only if exclusion of vascular malformations was confirmed by

neuroimaging.

Klippel-Trenaunay Syndrome: a troublesome condition