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.