Document Detail

Chronic respiratory failure in a patient with type I Arnold-Chiari malformation (ACM1) and syringomyelia.
MedLine Citation:
PMID:  9689798     Owner:  NLM     Status:  MEDLINE    
We present the case of a young adult with type I Arnold-Chiari malformation (AMC1) and syringomyelia who developed central sleep apnoea and chronic respiratory failure, successfully treated with nocturnal noninvasive positive pressure ventilation ventilation (NIPPV). An extensive syringomyelic cavity (from bulbar to L4 segment) with severe impairment of the IX cranial nerve was documented and remains, although reduced, after the neurosurgical treatment. At baseline evaluation, the patient showed a moderate restrictive ventilatory defect, severe hypercapnic respiratory failure, abnormal control of breathing characterized by the absence of response to hypoxia and hypercapnia, and severe nocturnal central apnoeas. Nocturnal NIPPV was then started in the A/C mode with an improvement in blood gas values. Further evaluations were performed 10 and 18 months later. A progressive significant improvement of lung volumes, both in sitting and supine position, associated with a slight improvement of blood-gas values were observed. Nonetheless, the breathing pattern abnormalities persisted. Polysomnographic evaluation during mechanical ventilation showed a normalization of breathing pattern with arterial oxygen saturation (SaO2) > 90% throughout the night.
F Fanfulla; D Eleftheriou; V Patruno; C Bruschi; C Rampulla
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace / Fondazione clinica del lavoro, IRCCS [and] Istituto di clinica tisiologica e malattie apparato respiratorio, Università di Napoli, Secondo ateneo     Volume:  53     ISSN:  1122-0643     ISO Abbreviation:  Monaldi Arch Chest Dis     Publication Date:  1998 Apr 
Date Detail:
Created Date:  1998-08-26     Completed Date:  1998-08-26     Revised Date:  2008-06-02    
Medline Journal Info:
Nlm Unique ID:  9307314     Medline TA:  Monaldi Arch Chest Dis     Country:  ITALY    
Other Details:
Languages:  eng     Pagination:  138-41     Citation Subset:  IM    
S. Maugeri Foundation I.R.C.C.S, Rehabilitation Institute of Montescano, Italy.
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MeSH Terms
Arnold-Chiari Malformation / complications*
Chronic Disease
Follow-Up Studies
Positive-Pressure Respiration
Respiratory Insufficiency / etiology*,  therapy
Sleep Apnea Syndromes / etiology*
Syringomyelia / complications*

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