POSTER PRESENTATION Open Access

Temporary tracheostomy required as an infantmay be a risk factor for future centrally mediateddisordered sleep ventilationRobert Adderley*, David Wensley

From 54th Annual Meeting of the Society for Research into Hydrocephalus and Spina BifidaVancouver, Canada. 7-10 July 2010

BackgroundChildren with spina bifida experience lifelong complexmedical issues. The problems of locomotion, and neuro-genic bowel and bladder are well appreciated, and thenecessity of shunting obstructive hydrocephalus isequally well known. There are however, serious, lesscommon problems associated with control of ventilation.Most common is vocal cord dysfunction, with unilateralor bilateral vocal cord paresis, and as a consequence,upper airway obstruction. Rarer still, are patients with lifethreatening breath holding spells, central apnea, ormixed central and obstructive apnea. In many cases, sur-gical decompression of the posterior fossa can result inreturn of vocal cord function, and relief of obstructiveapnea. In a few cases tracheostomy is required. With aChairi II malformation, the upper medulla, where thenuclei of cranial nerves IX and X lie close to the rostraltracts of the respiratory centre, may have an abnormaland tenuous blood supply. The medulla may be compro-mised by bony pressure or by compromise of the bloodsupply (herniation or chronic arachnoiditis).

Materials and methodsThe Spinal Cord Program at British Columbia’s Children’sHospital has cared for 956 patients since 1982. Over thesame period, the Home Tracheostomy Care/ Home Venti-lation Program has cared for 346 children, eight with spinaldysrhaphism. Two girls with meningomyelocoele andArnold Chiari type II malformations, who had requiredtracheostomies as infants, presented as adolescents with

symptoms suggestive of disordered sleep ventilation. Bothhad required a tracheostomy despite timely posterior fossadecompression, but over time (years), gag and vocal cordfunction returned, and they were successfully decannu-lated. Patient 1, with a lumbosacral meningomyelocoelewas referred to the Home Ventilation Program at fourteenyears of age when her mother, a registered nurse reportederratic breathing at night, with weight loss, and deteriorat-ing school performance. Patient 2, with a lumber meningo-myelocoele presented at fifteen years of age complaining ofdaytime somnolence, but denied morning headaches. Inboth cases polysomnography showed a similar, chaotic pat-tern of respiration with frequent arousals and severely frag-mented sleep. Computerized Tomography showed nochange in ventricular size in either case.

ResultsBoth patients were started on nighttime noninvasiveventilation (NIPPV) with rapid resolution of symptoms.

ConclusionsChildren with meningomyelocoele, particularly thosewho appear to have had resolution of infantile bulbardysfunction, may present later in life with severely disor-dered sleep ventilation, and warrant careful lifelong fol-lowup, with a low threshold for polysomnography andinstitution of ventilatory support.

Published: 15 December 2010

doi:10.1186/1743-8454-7-S1-S51Cite this article as: Adderley and Wensley: Temporary tracheostomyrequired as an infant may be a risk factor for future centrally mediateddisordered sleep ventilation. Cerebrospinal Fluid Research 2010 7(Suppl 1):S51.

* Correspondence: radderley@cw.bc.caCritical Care Services The University of British Columbia Room 2L5 BritishColumbia’s Children’s Hospital 4480 Oak Street Vancouver, British ColumbiaV6H 3V4, Canada

Adderley and Wensley Cerebrospinal Fluid Research 2010, 7(Suppl 1):S51http://www.cerebrospinalfluidresearch.com/content/7/S1/S51

CEREBROSPINAL FLUID RESEARCH

© 2010 Adderley and Wensley; licensee BioMed Central Ltd. This is an open access article distributed under the terms of the CreativeCommons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, andreproduction in any medium, provided the original work is properly cited.