Ambiguous External Genitalia Presenting With Chronic Renal Failure In Port Harcourt

A 16-year old presented with ambiguous external genitalia (AEG) and difficulty in passing urine since birth. She developed vomiting, generalized convulsion and reduced urine output three days prior to presentation. This case highlights management challenges and a rare complication of a case of (AEG).The patient was small for age, restless, with a blood pressure of 170/100mmHg. Genital examination revealed clitoral hypertrophy, fusion of labia majora and minora with no urethral opening. There was no palpable gonad in the inguinal region. Pubic and axillary hair development was at Tanner stage II and III respectively. Investigations revealed anaemia, renal failure with a Glomerular Filtration rate of 4.72ml/min/1.73m. Abdominal ultrasound scan showed echogenicity and poor corticomedullary differentiation of both kidneys with obstructive changes marked on the right. She has had no previous evaluation for the genital ambiguity. She was managed conservatively for chronic renal failure with end stage renal disease and hypertensive encephalopathy using antihypertensive. She was unable to commence dialysis and the cause of genital ambiguity could not be confirmed due to financial constraints before her demise. Conclusion: End stage renal disease is a rare preventable complication of AEG. The late presentation, ignorance and poverty were major contributing factors to poor outcome.