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Chiari type 1 malformation associated with central sleep apnea after high dose growth hormone (GH) therapy in a

Toshihiko Mori1, Eri Nishino1, Tomomi Jitsukawa1

  • 1Department of Pediatrics, NTT East Sapporo Hospital, Hokkaido, Japan.

Clinical Pediatric Endocrinology : Case Reports and Clinical Investigations : Official Journal of the Japanese Society for Pediatric Endocrinology
|February 7, 2018
PubMed

Insights

Growth hormone (GH) therapy in a short-statured boy led to Chiari type 1 malformation and severe central sleep apnea. Surgical decompression resolved the sleep apnea, highlighting the need for vigilance during GH treatment.

Area of Science:

  • Pediatric Endocrinology
  • Neurology
  • Sleep Medicine

Background:

  • Growth hormone (GH) therapy is used to treat short stature in children.
  • Potential adverse effects of GH therapy require careful monitoring.
  • Chiari malformation and sleep-related breathing disorders can impact patient health.

Purpose of the Study:

  • To report a rare case of Chiari type 1 malformation and central sleep apnea in a child receiving high-dose GH therapy.
  • To investigate the potential association between GH therapy and the development of these conditions.
  • To evaluate the efficacy of neurosurgical intervention for sleep apnea in this context.

Main Methods:

  • Case report of a 12-year-old boy with short stature treated with GH.
  • Detailed clinical evaluation including otolaryngology examination and polysomnography.
  • Magnetic resonance imaging (MRI) to diagnose Chiari type 1 malformation.
  • Assessment of treatment outcomes with continuous positive airway pressure (CPAP) and foramen magnum decompression.

Main Results:

  • The patient developed severe central sleep apnea (apnea-hypopnea index 46.5/h) and Chiari type 1 malformation (tonsillar herniation 15 mm below foramen magnum) during GH therapy.
  • CPAP therapy was ineffective in managing the sleep apnea.
  • Foramen magnum decompression resulted in normalization of breathing during sleep.

Conclusions:

  • High-dose GH therapy may be associated with the development of Chiari type 1 malformation and central sleep apnea.
  • Neuroimaging, specifically sagittal MRI, should be considered in patients on GH therapy who develop sleep apnea.
  • Surgical decompression is an effective treatment for sleep apnea secondary to Chiari malformation in this setting.

Keywords:
Chiari type 1 malformationGH therapyMRIcentral sleep apnea

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