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Anesthetic Management of a Child with Bannayan-Riley-Ruvalcaba Syndrome

Pancaro, Carlo, MD; Miller, Tonya, MD, FAAP; Dingeman, R Scott, MD, FAAP

Section Editor(s): Saidman, Lawrence

doi: 10.1213/ane.0b013e31816f277d
Letters to the Editor: Letters & Announcements
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Department of Anesthesiology, Pain and Perioperative Medicine; Brigham and Women’s Hospital; Harvard Medical School; Boston, Massachusetts; carlopancaro@hotmail.com (Pancaro)

Department of Anesthesiology, Perioperative and Pain Medicine; Children’s Hospital Boston; Harvard Medical School; Boston, Massachusetts (Miller, Dingeman)

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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.

Carlo Pancaro, MD

Department of Anesthesiology, Pain and Perioperative Medicine

Brigham and Women’s Hospital

Harvard Medical School

Boston, Massachusetts

carlopancaro@hotmail.com

Tonya Miller, MD, FAAP

R. Scott Dingeman, MD, FAAP

Department of Anesthesiology, Perioperative and Pain Medicine

Children’s Hospital Boston

Harvard Medical School

Boston, Massachusetts

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REFERENCES

1. Lachlan KL, Lucassen AM, Bunyan D, Temple IK. Cowden syndrome and Bannayan Riley Ruvalcaba syndrome represent one condition with variable expression and age-related penetrance: results of a clinical study of PTEN mutation carriers. J Med Genet 2007;44:579–85
2. Cohen MM Jr. Bannayan-Riley-Ruvalcaba syndrome: renaming three formerly recognized syndromes as one etiologic entity. Am J Med Genet 1990;35:291–92
3. Gorlin RJ, Cohen MM Jr, Condon LM, Burke BA. Bannayan-Riley-Ruvalcaba syndrome. Am J Med Genet 1992;44:307–14
4. Sharma MR, Petty EM, Lesperance MM. Airway obstruction caused by PTEN hamartoma (Bannayan-Riley-Ruvalcaba) syndrome. Arch Otolaryngol Head Neck Surg 2007;133:1157–60
5. Omote K, Kawamata T, Imaizumi H, Namiki A. Case of Cowden’s disease that caused airway obstruction during induction of anesthesia. Anesthesiology 1999;91: 1537–40
6. Jenny B, Radovanovic I, Haenggeli CA, Delavelle J, Rüfenacht D, Kaelin A, Blouin JL, Bottani A, Rilliet B. Association of multiple vertebral hemangiomas and severe paraparesis in a patient with a PTEN hamartoma tumor syndrome. Case report. J Neurosurg 2007;107(4 Suppl):307–13
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