Abstract: TH-PO0102
Dietl Crisis in a Patient with Lacrimo-Auriculo-Dento-Digital Syndrome
Session Information
- AKI: Pathogenesis and Disease Mechanisms
November 06, 2025 | Location: Exhibit Hall, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Kae, Soo Hyun, Hospital of Central Connecticut at New Britain, New Britain, Connecticut, United States
- Niranjan, Sankar Narayan, Stanford University, Stanford, California, United States
Introduction
Obstructive uropathy accounts for 5-10% of all acute kidney injury (AKI) cases. Congenital malformation as the cause of the obstruction is not commonly thought of, let alone in adults. We present a unique case of an adult presenting with AKI due to UPJ (ureteropelvic junction) obstruction likely from a congenital malformation due to LADD (lacrimo-auriculo-dento-digital) syndrome in hopes of raising awareness for clinicians to identify and quickly pursue appropriate work-up and management.
Case Description
51 yo M with history of HTN, baseline serum creatinine (SCr) of 1.0-1.2 mg/dL, and LADD syndrome presented with acute-onset, episodic, right-sided flank pain and associated nausea/vomiting. He was in AKI with SCr 1.5 peaking at 1.8 mg/dL. His imaging showed high-grade right UPJ obstruction with no evidence of stone, mass, or stricture. He had a right nephrostomy tube placement as a temporary measure with improvement in renal function. He underwent an elective robotic-assisted right pyeloplasty after discharge. Intraoperatively, a narrow atrophic appearing portion of the proximal right ureter was identified as the culprit for the UPJ obstruction without any crossing vessels, so this area was resected and reconstructed. His SCr returned to his baseline and has been stable since without further episodes of UPJ obstructions.
Discussion
Our patient’s presentation is consistent with Dietl’s crisis, a clinical syndrome characterized by episodic acute abdominal pain with nausea/vomiting, often triggered by a UPJ obstruction, which can be congenital or caused by other factors like a crossing blood vessel. Although seen in children, it is rare in adults as the congenital aspect of it usually presents earlier in life. Our patient was known to have a mild form of LADD syndrome, which can be associated with congenital abnormalities of the genitourinary system that can cause hydronephrosis, nephrosclerosis, and renal agenesis in extreme cases. His LADD syndrome was thought to have played a role in the abnormal development of his proximal right ureter that eventually caused the UPJ obstruction. The patient did have a kidney ultrasound done 2 years prior to his presentation, which showed moderate fullness of the R renal pelvis with no obstructing calculi. He was started on a thiazide for HTN just before his presentation; so in retrospect, this could have been the precipitant for the UPJ obstruction to manifest clinically.