Acrocyanosis Presentation in Postural Orthostatic Tachycardia Syndrome

Jane Abou-Diab, Dina Moubayed, Danielle Taddeo, Olivier Jamoulle, Chantal Stheneur


Postural orthostatic tachycardia syndrome (POTS) is a clinical chronic condition characterized by chronic fatigue and orthostatic symptoms associated with postural tachycardia. Early diagnosis and intervention can prevent significant functional consequences, although it can be very difficult to recognize. Two cases of POTS were presented in this article to help clinicians identify POTS more easily. Firstly, we present a 16-year-old adolescent female who was hospitalized for daily headaches, chronic fatigue, bilateral lower limbs discoloration and edema on standing position associated with orthostatic symptoms. All investigations were negative except for tilt table test, which showed heart rate increase of 80 beats per min, hypotension and discoloration of lower limbs, confirming POTS diagnosis. Non-pharmacological treatment showed no improvement. Midodrine was started because of beta-blockers intolerance with only moderate response. Secondly, we present a 17-year-old patient diagnosed with benign hypermobility syndrome who was referred for recurring episodes of palpitations associated with heat, nausea, headache and vertigo for the past year. She also reported dizziness and lower limbs discoloration on standing position. Previous investigations were negative. A clinical diagnosis of POTS was made based on history. Non-pharmacologic treatments with hydration, increase of salt intake and regular exercise were tested. However, no improvement was shown. Symptoms were controlled by a propranolol trial. These cases relate a clinical sign that can be easily identified by a clinician attempting to diagnose POTS. Acrocyanosis can be unquestioned or ignored. Clinicians should be attentive to this condition, and attempt to diagnose its underlying causes. This can result in lowering unnecessary testing and interventions. Patients with dependent acrocyanosis should be closely evaluated for the diagnosis of POTS.

Int J Clin Pediatr. 2018;7(1-2):13-16


Acrocyanosis; Adolescence; Orthostatic; Tachycardia

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