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Nail-Patella syndrome: a case report and anesthetic implications

[L’ostéo-onychodysostose : présentation de cas et implications anesthésiques]

Trevor A. Hennessey, MD^*, Steven B. Backman, MD PhD^*, Sarkis H. Meterissian, MD MSc and Thomas Schricker, MD PhD^*

^* From the Department of Anesthesia, and
Surgery, Royal Victoria Hospital, McGill University Health Center, Montreal, Quebec, Canada.

Address correspondence to: Dr. Thomas Schricker, Department of Anaesthesia, Royal Victoria Hospital, 687 Pine Avenue West, Rm. C5.20, Montreal, Quebec H3A 1A1, Canada. Phone: 514-934-1934, ext. 34883; Fax: 514-843-1698; E-mail: thomas.schricker{at}mcgill.ca

Purpose: To report a case of asystole during combined epidural and general anesthesia occurring in a patient with Nail-Patella syndrome (NPS), and to review the management and anesthetic implications of this rare genetic syndrome.

Clinical features: A 64-yr-old male with NPS, renal impairment and coronary artery disease presented for right hemicolectomy for colon cancer. Following initiation of surgery and during insertion of a nasogastric tube there was sudden loss of the patient’s pulse oxymetry, and arterial pressure waveforms with an asystolic electrocardiogram signal. Atropine 0.6 mg iv was administered and after an asystolic period of 20–30 sec, myocardial activity commenced at 110 beats·min^–1 with return of normal vital signs and no further sequelae.

Conclusions: Nail-Patella syndrome can present with an array of anomalies that may be associated with perioperative complications. Glaucoma, nephropathy, vasomotor dysfunction, fragile teeth, abnormal muscle, skeletal and nerve anatomy as well as involvement of the central and/or peripheral nervous systems are common findings. In this setting it is postulated that a vasovagal reflex from esophageal stimulation by nasogastric tube placement may have caused the asystolic event. This response could have been exaggerated by the sympatholytic combination of neuraxial block, preoperative beta-blockade, and potential autonomic dysfunction secondary to NPS. Awareness of this uncommon disease and its presentation may serve to caution the anesthesiologist regarding the perioperative implications of patients with this syndrome.