Last update:
24-May-2026

In complex congenital anomalies such as obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, electrolyte disturbances may have multifactorial etiologies, including renal anatomical abnormalities and hormonal dysfunction. This case report aims to present a case of refractory hypokalemia associated with subclinical hypothyroidism in a patient with OHVIRA syndrome. A 28-year-old woman with a history of OHVIRA syndrome since early life presented with recurrent episodes of muscle weakness and fatigue. She had persistent refractory hypokalemia that did not improve with oral potassium supplementation. Laboratory tests showed persistent hypokalemia (2.9–3.0 mmoL/L), sodium levels ranging from 139 to 150 mmoL/L, elevated thyroid stimulating hormone (TSH) (8.4 μIU/mL) with normal free T4, along with mild leukocytosis and thrombocytosis. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) confirmed unilateral renal agenesis on the same side as the obstructed hemivagina. She was treated with oral levothyroxine with gradual dose adjustments and potassium supplementation. Monthly laboratory follow-up showed improvement in potassium levels and resolution of symptoms in parallel with normalization of TSH. In conclusion, subclinical hypothyroidism may be an important contributing factor to refractory hypokalemia in patients with OHVIRA. A multi-organ evaluation is essential to identify the underlying cause and to design an optimal management strategy.

Key words: Electrolyte imbalance, Hypokalemia, Hypothyroidism subclinical, Kidney agenesis, Müllerian duct anomalies.