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Acupoint Application Combined with Ear Plaster Therapy for Treating Sleep Disorders with Acute Exacerbation of Chronic Obstructive Pulmonary Disease
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A case of achondroplasia with severe pulmonary hypertension due to obstructive sleep apnea

Selman Vefa Yildirim1, Cemile Durmaz, Mir Ali Pourbagher

  • 1Division of Pediatric Cardiology, Adana Teaching and Research Center, Başkent University, Adana, Turkey.

European Archives of Oto-Rhino-Laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery
|May 4, 2006
PubMed

Insights

Severe pulmonary hypertension in achondroplasia is rare. This case study shows that upper-airway obstruction from achondroplasia can cause severe pulmonary hypertension, which resolved after surgery.

Area of Science:

  • Pediatrics
  • Genetics
  • Pulmonology

Background:

  • Achondroplasia is the most common skeletal dysplasia.
  • Patients with achondroplasia often exhibit midface hypoplasia, leading to airway complications.
  • Obstructive sleep apnea is a known cause of pulmonary hypertension (PH) via hypoxemia.

Observation:

  • A 5-year-old girl with achondroplasia presented with severe PH.
  • Her condition was attributed to upper-airway obstruction secondary to achondroplasia.
  • This is a rare presentation of severe PH in achondroplasia.

Findings:

  • The patient experienced severe pulmonary hypertension due to obstructive sleep disorder.
  • Adenotonsillectomy was performed to address the upper-airway obstruction.
  • Post-surgery, the patient showed gradual resolution of severe PH.

Implications:

  • This case highlights a rare but serious complication of achondroplasia.
  • Early identification and management of airway obstruction are crucial in achondroplasia patients.
  • Surgical intervention for airway obstruction may effectively treat severe PH in this population.

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