Anesth Analg 2008; 106:1928-1929
© 2008 International Anesthesia Research Society
doi: 10.1213/ane.0b013e31816f277d
LETTER TO THE EDITOR
Section Editor:
Lawrence Saidman
Anesthetic Management of a Child with Bannayan-Riley-Ruvalcaba Syndrome
Carlo Pancaro, MD,
Tonya Miller, MD, FAAP, and
R. Scott Dingeman, MD, FAAP
Department of Anesthesiology, Pain and Perioperative Medicine; Brigham and Women’s Hospital; Harvard Medical School; Boston, Massachusetts; carlopancaro{at}hotmail.com (Pancaro)
Department of Anesthesiology, Perioperative and Pain Medicine; Children’s Hospital Boston; Harvard Medical School; Boston, Massachusetts (Miller, Dingeman)
To the Editor:
A 7-yr-old girl presented for sclerotherapy of a flank hemangioma. She carried a diagnosis of Bannayan-Riley-Ruvalcaba syndrome, a rare autosomal dominant overgrowth disorder characterized mainly by macrocephaly and diffuse hemangiomas.1–3 Sevoflurane 8% in oxygen was administered before establishing an IV access. Blankets supporting patient’s shoulders and a foam pad underneath the patient’s head were necessary to facilitate airway manipulation. Before giving 100 mg of IV propofol, a brief laryngoscopy was performed to exclude any upper airway pathology. The trachea was easily intubated.
In Bannayan-Riley-Ruvalcaba syndrome, there is an exaggerated Weldeyer ring hypertrophy4 that might cause airway obstruction5 during anesthesia. Inhalation induction offers the advantage of performing laryngoscopy during spontaneous breathing to exclude upper airway pathology. When there is a high index of suspicion for airway obstruction, a laryngologist evaluation or imaging of the upper airway are advisable before anesthesia. Our patient’s spine MRI showed a venous malformation involving the paravertebral soft tissues and the neural foramina in the thoracic and lumbar regions. Spontaneously bleeding vertebral angiomas accompanied by paraparesis have been described in patients with Bannayan-Riley-Ruvalcaba syndrome.6 We suggest obtaining an MRI of the spine if neuraxial blocks are planned in these patients.
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