A0027 Patient with Hurler's Syndrome for Emergency Ventriculoperitoneal Shunt: What an Anesthesiologist Must Know

Background: Patients with Hurler's syndrome pose a great challenge to anesthesiologists. The most common problem faced by anesthesiologist is difficult venous access and establishment and maintenance of difficult airway.

Case Description: A 22-year-old female patient, a known case of Hurler's syndrome, presented with complaint of headache. She was scheduled for insertion of VP shunt for acute hydrocephalus. History was significant for obstructive sleep apnea, and she used CPAP during sleep for the same. The patient was cooperative but slow in response to commands. General physical examination revealed short stature, dysmorphic face, macrocephaly, frontal bossing, short neck, depressed nasal bridge, dorsolumbar kyphosis, flexion deformity of elbows and wrists, and stubby fingers. Airway evaluation revealed large tongue and Mallampati grade IV. Echocardiography revealed mitral valve prolapse with trivial mitral regurgitation. Intravenous access was obtained with difficulty. Difficult mask ventilation was anticipated, in addition to difficult laryngoscopy and intubation. Awake fiberoptic intubation was performed successfully. Anesthesia was induced, and at the end of surgery, trachea was extubated awake.

Conclusions: The most important step in management of airway is to avoid a situation of “can't ventilate, can't intubate.” The basic aim is preservation of spontaneous ventilation and is of utmost importance. Recovery after general anesthesia is often slow in these patients and may be accompanied by breath-holding, apnea, and bronchospasm. The patient should be awake with adequate airway reflexes for extubation.