Anesthesia for proteus syndrome

Abstract

Proteus syndrome is a complex disorder comprising malformations and overgrowth of multiple tissues. The disorder is highly variable affecting tissues in a mosaic pattern. A 2-year-old boy with Proteus syndrome, with epidermal verrucal naevus, hyperplastic lesions of connective tissue, hyperostosis, overgrowth of tubular bones, bilateral inguinal hernia, and juvenile intestinal polyposis was scheduled for vertebral magnetic resonance imaging (MRI) for further evaluation of malignancies. In addition to the pathological findings of this syndrome, potential complications such as difficult intubation, pulmonary hypertension, and pulmonary thromboembolism necessitate a careful preoperative and anesthetic preparation. MRI was performed under general anesthesia. There were no anesthetic complications. There are few previous reports on anesthesia in a patient with Proteus syndrome.

Proteus syndrome: An update

“…Cardoso et al [2003] reported a patient with neurofibromatosis as having ''Proteus syndrome.' ' Cekmen et al [2004] reported a patient with presumed ''Proteus syndrome;'' no clinical photographs were shown, but juvenile polyposis of the colon was a striking feature.…”

Bronchoscopic concerns in Proteus syndrome: a case report

“…Pradhan et al [ 6 ] and Sinha et al [ 7 ] reported on intubation using a McCoy levering laryngoscope for enlarged epiglottis. Cekmen et al [ 8 ] reported on a 2.5-year boy who underwent general anesthesia using LMA without complication. In our case, difficult intubation was anticipated because the patient had facial asymmetry and disproportion, abnormal teeth, limitation of neck movement due to torticollis, and suboptimal positioning due to thoracolumbar scoliosis ( Figs.…”

Ultrasound-guided neural blockade in Proteus syndrome

“…5 A small patent ductus arteriosus has been reported. 11 Cardiovascular illness secondary to pulmonary disease occurs and pulmonary hypertension should be excluded.…”