NM-330
A Case of Chiari Type 1 Decompression in a Patient with Costello Syndrome
Patel J, Patel R
Albert Einstein-Montefiore Medical Center, Bronx, NY, USA
Costello syndrome is a rare genetic disorder causing cell overgrowth and abnormal cell division that has been described as a faciocutaneousskeletal syndrome. It involves short stature, metabolic dysfunction, cardiac defects, and neurologic manifestations. We present a patient who successfully underwent suboccipital craniectomy and cervical laminectomy for Chiari Type 1 decompression and syringomyelia.
The patient, a 10 year old female with Costello syndrome, was found to have a chiari malformation during routine workup for precocious puberty. A prior echo revealed trivial mitral regurgitation and normal biventricular systolic function. Her past history also included severe developmental delay and well controlled GERD. Preoperative physical exam revealed limited neck mobility, a Mallampati class III airway, and an unremarkable cardiopulmonary evaluation. She was premedicated with oral midazolam, received an inhalational induction for IV placement and was intubated with video laryngoscopy. Intraoperatively, the patient developed atrial arrhythmias but remained hemodynamically stable. She was extubated and transferred to the PICU in stable condition.
There are significant anesthetic considerations in patients with Costello syndrome due to its multisystem involvement. Patients present with a short neck, macroglossia, airway papillomata, and hypertrophied airway tissues. As a result, intubating conditions must be optimized which involves proper positioning, use of muscle relaxants, and appropriate airway equipment. Despite concern for airway obstruction, severe developmental delay often requires premedication. In our case, the decision was made to intubate using a Mcgrath after demonstrating successful mask ventilation. Cardiac abnormalities have also been described in this patient population. Although an old echo was within normal limits in our patient, she was lost to follow up and had not been re-evaluated by cardiology. Intraoperatively, she experienced variable P-wave morphology unrelated to the brainstem manipulation by the surgeon. Electrolyte abnormalities must be ruled out due to the potential for metabolic dysfunction. The perioperative use of TEE and precordial Doppler in addition to invasive monitoring must be considered depending on the surgical procedure. Dermatologic manifestations include excessive skin wrinkling with deep creases in the palms and soles, hyperpigmentation, and acanthosis nigricans. The increased skin laxity in our patient made obtaining IV access difficult. Neurologic findings include seizures, cerebral atrophy, dilated ventricles, and postnatal cerebellar overgrowth which may lead to development of a Chiari I malformation. Patients with Costello syndrome presenting for other surgical procedures may benefit from neurologic evaluation including brain MRI. Additionally, they often exhibit hypotonia that can impact the use of muscle relaxants or increase the risk of post-operative respiratory complications.
Successful anesthetic management of patients with Costello syndrome requires a comprehensive preoperative evaluation and intraoperative plan to minimize the risk of potential complications.
Katcher K, Tobias J. Anesthetic implications of Costello syndrome. Paediatric Anaesthesia. 2003;13:257-262.
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