Extraperitoneal laparoscopy-assisted percutaneous nephrolithotomy in a patient with osteogenesis imperfecta

Abstract

Osteogenesis imperfecta (OI) patients represent a challenge to all physicians, as they do for anesthetists and urologists, when they develop symptomatic stones in the urinary tract. We recently treated an OI patient with renal pelvic stone by extraperitoneal laparoscopy-assisted percutaneous nephrolithotomy (PCNL). To our knowledge, this combined treatment modality has not been reported previously in OI. An 18-year-old paraplegic girl with OI presented to our urology department because of right-sided flank pain. She pointed out that she had right kidney stone for the previous 2 years, and because of risks of general anesthesia and surgical procedures, surveillance was recommended. Intravenous pyelography was performed and an 11.9-mm stone at the pelvis of the right kidney and grade 1–2 hydronephrosis at the same side with normal kidney functions and severe left-sided scoliosis were detected. After explanation of risks of the treatment modality and general anesthesia to the patient, extraperitoneal laparoscopy-assisted PCNL was performed. No complications occurred due to general anesthesia or surgical procedure. The operation time was 95 min and no blood transfusion was required. The nephrostomy tube and retroperitoneal drain were removed 2 and 3 days after the procedure, respectively. The patient was doing well at a follow-up of 6 months. Extraperitoneal laparoscopy-assisted PCNL approach may decrease the risk of surgery as an alternative treatment modality for OI patients. Such cases should be operated on at centers with significant experience in the field of endourology, where all the equipment and specialized personnel are readily available.