Case
We report the case of an 8-year-old woman, native from Spain, with no
personal history of interest. She has a family history of a mother with
a diagnosis of severe OSA in treatment with nocturnal CPAP, a 14 years
old sister with a diagnosis of severe OSA and in treatment with
nocturnal CPAP and 11 years old brother with a diagnosis of moderate
OSA.
The patient went to the Guadalajara University Hospital´s Sleep Unit
consultations of the Pneumology Service for restless sleep symptoms,
apnea witnessed accompanied by persistent snoring, headache and bruxism.
He did not refer to nocturnal enuresis, and presented a good school
performance. Physical examination highlights tonsillar hypertrophy III /
IV with normal palate and cardiopulmonary auscultation without
alterations. In December 2017, with 6 years, hospital CRP was performed
with a result of severe OSA of obstructive predominance (hypopnea apneas
index-AHI 16 / h: obstructive AHI: 15.3; central AHI: 0.7 / h).
Tonsillectomy was decided, after which the patient presented clinical
improvement with decreased daytime hypersomnolence.
Postamigdalectomy CRP was performed in October 2018 with a diagnosis of
moderate mixed OSA of central predominance (AHI: 9.3 / h: obstructive
AHI: 3.3 / h; central AHI 6 / h). We request a new evaluation by
Otolaryngology, with endoscopy with small non-obstructive adenoid
remains. Given the results of post-migralectomy CRP, it was decided to
perform hospital PSG presenting a change in the sleep architecture for
the patient’s age, increased surface sleep and frequent daydreams, very
frequent respiratory events were recorded accompanied by desaturations
of relevance and long duration (with no hypoventilation observed) .
These events are about sleep apnea with a clear central predominance
(AHI: 26.7 / h, obstructive AHI: 8.4 / h, central AHI: 18.3 / h) (Image
1).
Given this diagnosis, an etiological study of the CSAS was initiated,
performing cerebral-cervical-dorsal MRI with contrast. In that MRI, a 9
mm descent of the cerebellar tonsils was observed below the plane of the
great hole compatible with Arnold Chiari type I malformation, with
association to cervical syringomyelia that extended from C5 to D9 (Image
2). The patient was referred to the neurosurgery department for
evaluation of surgical treatment.