Tags

Type your tag names separated by a space and hit enter

Chronic respiratory failure in a patient with type I Arnold-Chiari malformation (ACM1) and syringomyelia.
Monaldi Arch Chest Dis. 1998 Apr; 53(2):138-41.MA

Abstract

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.

Authors+Show Affiliations

S. Maugeri Foundation I.R.C.C.S, Rehabilitation Institute of Montescano, Italy.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

9689798

Citation

Fanfulla, F, et al. "Chronic Respiratory Failure in a Patient With Type I Arnold-Chiari Malformation (ACM1) and Syringomyelia." Monaldi Archives for Chest Disease = Archivio Monaldi Per Le Malattie Del Torace, vol. 53, no. 2, 1998, pp. 138-41.
Fanfulla F, Eleftheriou D, Patruno V, et al. Chronic respiratory failure in a patient with type I Arnold-Chiari malformation (ACM1) and syringomyelia. Monaldi Arch Chest Dis. 1998;53(2):138-41.
Fanfulla, F., Eleftheriou, D., Patruno, V., Bruschi, C., & Rampulla, C. (1998). Chronic respiratory failure in a patient with type I Arnold-Chiari malformation (ACM1) and syringomyelia. Monaldi Archives for Chest Disease = Archivio Monaldi Per Le Malattie Del Torace, 53(2), 138-41.
Fanfulla F, et al. Chronic Respiratory Failure in a Patient With Type I Arnold-Chiari Malformation (ACM1) and Syringomyelia. Monaldi Arch Chest Dis. 1998;53(2):138-41. PubMed PMID: 9689798.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Chronic respiratory failure in a patient with type I Arnold-Chiari malformation (ACM1) and syringomyelia. AU - Fanfulla,F, AU - Eleftheriou,D, AU - Patruno,V, AU - Bruschi,C, AU - Rampulla,C, PY - 1998/8/5/pubmed PY - 1998/8/5/medline PY - 1998/8/5/entrez SP - 138 EP - 41 JF - Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace JO - Monaldi Arch Chest Dis VL - 53 IS - 2 N2 - 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. SN - 1122-0643 UR - https://www.unboundmedicine.com/medline/citation/9689798/Chronic_respiratory_failure_in_a_patient_with_type_I_Arnold_Chiari_malformation__ACM1__and_syringomyelia_ L2 - http://www.diseaseinfosearch.org/result/579 DB - PRIME DP - Unbound Medicine ER -