The Wunderlich's syndrome secondary to massive bilateral angiomyolipomas associated with advanced tuberous sclerosis. 2011

Dorothy Sparks, and Daniel Chase, and David Thomas, and Jon Arnott
Western Reserve Care System/Northeastern Ohio College of Medicine, OH, USA. Afirebrand@gmail.com

The Wunderlich Syndrome refers to spontaneous perirenal hemorrhage often associated with underlying renal pathology. We report the case of a 23-year-old female with advanced tuberous sclerosis who presented in hypovolemic shock secondary to rupture of one of her massive bilateral angiomyolipomas (AMLs). The patient was able to be managed conservatively and subsequently retained full renal function. A review of the literature with a discussion of types of clinical presentation, diagnostic criteria, and methods of management of AMLs is included.

UI MeSH Term Description Entries
D007680 Kidney Neoplasms Tumors or cancers of the KIDNEY. Cancer of Kidney,Kidney Cancer,Renal Cancer,Cancer of the Kidney,Neoplasms, Kidney,Renal Neoplasms,Cancer, Kidney,Cancer, Renal,Cancers, Kidney,Cancers, Renal,Kidney Cancers,Kidney Neoplasm,Neoplasm, Kidney,Neoplasm, Renal,Neoplasms, Renal,Renal Cancers,Renal Neoplasm
D005260 Female Females
D006470 Hemorrhage Bleeding or escape of blood from a vessel. Bleeding,Hemorrhages
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D001416 Back Pain Acute or chronic pain located in the posterior regions of the THORAX; LUMBOSACRAL REGION; or the adjacent regions. Backache,Back Ache,Back Pain with Radiation,Back Pain without Radiation,Vertebrogenic Pain Syndrome,Ache, Back,Aches, Back,Back Aches,Back Pains,Backaches,Pain Syndrome, Vertebrogenic,Pain Syndromes, Vertebrogenic,Pain, Back,Pains, Back,Syndrome, Vertebrogenic Pain,Syndromes, Vertebrogenic Pain,Vertebrogenic Pain Syndromes
D012187 Retroperitoneal Space An area occupying the most posterior aspect of the ABDOMINAL CAVITY. It is bounded laterally by the borders of the quadratus lumborum muscles and extends from the DIAPHRAGM to the brim of the true PELVIS, where it continues as the pelvic extraperitoneal space. Retroperitoneal Spaces,Space, Retroperitoneal,Spaces, Retroperitoneal
D012422 Rupture, Spontaneous Tear or break of an organ, vessel or other soft part of the body, occurring in the absence of external force. Ruptures, Spontaneous,Spontaneous Rupture,Spontaneous Ruptures
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes
D014402 Tuberous Sclerosis Autosomal dominant neurocutaneous syndrome classically characterized by MENTAL RETARDATION; EPILEPSY; and skin lesions (e.g., adenoma sebaceum and hypomelanotic macules). There is, however, considerable heterogeneity in the neurologic manifestations. It is also associated with cortical tuber and HAMARTOMAS formation throughout the body, especially the heart, kidneys, and eyes. Mutations in two loci TSC1 and TSC2 that encode hamartin and tuberin, respectively, are associated with the disease. Bourneville Disease,Epiloia,Phakomatosis, Bourneville,Adenoma Sebaceum,Bourneville Phakomatosis,Bourneville Syndrome,Bourneville's Disease,Bourneville's Syndrome,Bourneville-Pringle Disease,Bourneville-Pringle's Disease,Cerebral Sclerosis,Phacomatosis, Bourneville,Sclerosis Tuberosa,Tuberose Sclerosis,Tuberous Sclerosis Complex,Bourneville Phacomatosis,Bourneville Pringle Disease,Bourneville Pringle's Disease,Bourneville-Pringles Disease,Cerebral Scleroses,Disease, Bourneville-Pringle,Disease, Bourneville-Pringle's,Sclerosis, Cerebral,Sclerosis, Tuberose,Sclerosis, Tuberous,Syndrome, Bourneville,Syndrome, Bourneville's

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