Leanne Foster and Claude Abdallah*
Division of Anesthesiology, Children’s National Medical Center, The George Washington University Medical Center, USA
Received: 23 February, 2016; Accepted: 16 March, 2016; Published: 18 March, 2016
Claude Abdallah, MD, MSc, Division of Anesthesiology, Children’s National Medical Center, The George Washington University Medical Center, 111 Michigan Avenue, NW. Washington D.C. 20010-2970, Tel: (202) 476-2025/2407: E-mail:
Foster L, Abdallah C (2016) Emergent Cervical Decompression in a Child with MURCS Association. Glob J Anesthesiol 3(1): 004-005. DOI: 10.17352/2455-3476.000021
© 2016 Foster L, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
MURCS Association; Cervical Decompression; Child
This case report describes an unusual presentation and anesthesia management of a rare genetic association. The MURCS association, a variant of Mayer-Rokitansky-Küster-Hauser syndrome is a rare (1/4500-1/50,000) congenital syndrome, of unknown etiology, consisting of mullerian duct (MU) aplasia, renal (R) aplasia, and cervicothoracic somite (CS) dysplasia.
A 3 years old female, with a history of MURCS association including vertebral anomalies, foramen magnum stenosis, upper cervical syrinx and central sleep apnea presented to an outside hospital following sinus congestion. She was initially found to have respiratory syncytial virus (RSV) bronchiolitis. The patient had breath holding spells associated with severe bradycardia, requiring tracheal intubation and hemodynamic resuscitation. Tracheal intubation was performed by the ear/nose/throat surgeon, as the patient had a critical airway due to cervical stenosis.
She was transferred to our institution for urgent decompression after an MRI of the brain and neck showed critical narrowing of the foramen magnum with severe mass effect on the medulla, Chiari I, as well as upper cervical syrinx and multiple cervical segmentation abnormalities with dysgenesis of the craniocervical junction (Figure 1). In the operating room, anesthesia was induced with propofol 3 mg/kg, fentanyl 1 mcg/kg and rocuronium 1 mg/kg after insuring the patency of the endotracheal tube, adequate intravenous access and an arterial line in place. The respiratory status was optimized with inhaled albuterol administration, endotracheal tube suctioning, and positive end expiratory pressure. Patient was placed in a prone position with the head supported by a cerebellar head rest. The initial phase of the surgery including tissue dissection and partial occipital craniotomy was well tolerated; however, repeated episodes of bradycardia and hemodynamic instability necessitated repetitive vasopressors including atropine 0.02mg/kg, epinephrine 5 mcg/kg followed by 10mcg/kg boluses and infusions of dopamine 10 mcg/kg/min and epinephrine starting at 0.1 mcg/kg/min and titrated to response; and correlated on occasion with minimal surgical manipulation.
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