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Open Access Case report

Type I Arnold-Chiari malformation with bronchiectasis, respiratory failure, and sleep disordered breathing: a case report

Raffaele Campisi12*, Nicola Ciancio1, Laura Bivona1, Annalisa Di Maria3 and Giuseppe Di Maria1

Author Affiliations

1 Pneumology Unit Policlinico “G. Rodolico”, University of Catania, Via Santa Sofia 78, 95123, Catania, Italy

2 Respiratory Diseases, Università di Catania, Catania, Italy

3 School of specialization in Respiratory Dideases, University of Catania, Catania, Italy

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Multidisciplinary Respiratory Medicine 2013, 8:15  doi:10.1186/2049-6958-8-15

Published: 22 February 2013

Abstract

Arnold Chiari Malformation (ACM) is defined as a condition where part of the cerebellar tissue herniates into the cervical canal toward the medulla and spinal cord resulting in a number of clinical manifestations. Type I ACM consists of variable displacement of the medulla throughout the formamen magnum into the cervical canal, with prominent cerebellar herniation.

Type I ACM is characterized by symptoms related to the compression of craniovertebral junction, including ataxia, dysphagia, nistagmus, headache, dizziness, and sleep disordered breathing. We report a case of a life-long non-smoker, 54 years old woman who presented these symptoms associated with bronchiectasis secondary to recurrent inhalation pneumonia, hypercapnic respiratory failure, and central sleep apnea (CSA).

CSA was first unsuccessfully treated with nocturnal c-PAP. The subsequent treatment with low flow oxygen led to breathing pattern stabilization with resolution of CSA and related clinical symptoms during sleep. We suggest that in patients with type I ACM the presence of pulmonary manifestations aggravating other respiratory disturbances including sleep disordered breathing (SDB) should be actively investigated. The early diagnosis is desirable in order to avoid serious and/or poorly reversible damages.

Keywords:
Arnold-Chiari malformation; Bronchiectasis; Central sleep apnea; Oxygen therapy; Respiratory failure; Sleep disordered breathing