Webb–Dattani syndrome in a 17-year-old girl

Webb-Dattani syndrome (WEDAS) is a rare autosomal recessive disorder caused by an ARNT2 pathogenic variant, presenting with a triad of congenital hypopituitarism, structural brain anomalies, and developmental delay. Adipsic diabetes insipidus is a hallmark complication in WEDAS and requires vigilant fluid management due to the absence of thirst sensation and persistent hypernatremia. Multisystem involvement - including renal anomalies and neurogenic bladder - may occur, expanding the known phenotypic spectrum of ARNT2-related disorders. Early recognition and genetic testing are essential for accurate diagnosis, especially in patients from consanguineous backgrounds presenting with multiple pituitary hormone deficiencies. Long-term survival, although rare, is possible with coordinated multidisciplinary care addressing endocrine, neurological, renal, and infectious complications. This case underscores the importance of individualized endocrine replacement therapy and caregiver education in managing complex syndromic conditions such as WEDAS.