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Organo Oficial de la Sociedad Mexicana de Urología

2018, Number 4

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Rev Mex Urol 2018; 78 (4)

Wunderlich syndrome in tuberous sclerosis: Conservative management

Martínez-López HI, León-Vilchis F, Jiménez-López LA, Ricardez- Espinoza AA

Language: Spanish
References: 32
Page: 303-309
PDF size: 415.95 Kb.


ABSTRACT

Background: Wunderlich syndrome, also called spontaneous retroperitoneal hemorrhage, is a very rare clinical entity. Its primary kidney tumor etiology is angiomyolipoma and accounts for 60% of the cases.
Clinica case: A 22-year-old woman presented with sudden onset of pain in the right renal fossa. Upon hospital admission, the patient was conscious, lucid, and had mild hypotension. Physical examination revealed signs and symptoms of tuberous sclerosis, a soft, depressible abdomen that was painful upon palpation, a palpable mass in the right renal fossa, and no signs of peritoneal irritation. A tomography scan identified a right well-defined subcapsular hematoma with multiple bilateral angiomyolipomas. Wunderlich syndrome was diagnosed and tuberous sclerosis was confirmed through major criteria. Because the patient was hemodynamically stable she was managed conservatively, with serial hematocrit surveillance, intravenous fluids, and complete bedrest. The patient had satisfactory progression.
Conclusion: Treatment of Wunderlich syndrome should begin with an evaluation of the hemodynamic status of the patient. In case of instability, the indication is urgent surgery. We present herein the first report in the medical literature of an exceptional form of Wunderlich syndrome, concomitant with tuberous sclerosis, that was managed conservatively.


REFERENCES

  1. Wunderlich. Handbuch der patholie und therapie. 2nd ed. Stuttgart: Ebner and Seubert, 1856.

  2. Polkey and Vynalek. Spontaneous nontraumatic perirrenal and renal hematomas. An experimental and clinical study. Arch Surg 1993;26:196

  3. McDougal W, Kursh E, Persky L. Spontaneous rupture of kidney with perirrenal haematoma. J Urol 1975;114:181-4.

  4. Zhang JQ, Fielding JR, Zou KH. Etiology of spontaneous perirenal hemorrhage: a meta-analysis. J Urol 2002;167(4):1593-6.

  5. Pove D, Caine M. Spontaneous retroperitoneal hemorrhage. J Urol 1992;147:311-8.

  6. Stewart B, McLaughlin SJ, Thompson GA. Spontaneous retroperitoneal hemorrhage: a general surgeon’s perspective. Aust N Z J Surg 1998;68(5):371-3.

  7. Pinilla R, López S, Quintana JC, Al-Ezzi Al-Malahi A. Síndrome de Wunderlich: presentación de un caso y revisión de la bibliografía. Rev Colomb Cir 2009;24:56-61.

  8. Daliakopoulus SI. Spontaneous retroperitoneal hematoma: a rare devastating clinical entity of a pleiada of less common origins. doi: 10.4103/2006-8808.78462.

  9. De Luca S, Terrone C, Rossetti SR. Management of renal angiomyolipoma: a report of 53 cases. BJU Int 1999;83(3):215-8.

  10. Machuca Santa-Cruz FJ, Barros Rodriguez JM, Guate Ortiz JL, Mata Varela J, Ojea Calvo A, Figueiredo Godoy L, et al. Cirugía conservadora de urgencia por rotura de angiomiolipoma renal. Arch Esp Urol 1990;43:179-81.

  11. Pode D, Metetik S, Shapiro A, Lame M, Frcs MS. Diagnosis and management of renal angiomiolipoma. Urology 1985;5:461-7.

  12. Gimeno-Argente V, Bosquet-Sanz M, Ramírez-Backhaus M, Trassierra-Villa M, Arlandis-Guzmán S, Jiménez-Cruz JF. Hemorragia retroperitoneal espontánea: nuestra experiencia en los últimos 10 años. Actas Urol Esp 2007;31:521-7.

  13. López-Cubillana P, Hita-Rosino E, Asensio Egea L, Rigabert Montiel M, Hita Villaplana G, Pérez Albacete M. Síndrome de Wünderlich. Revisión diagnóstica y terapéutica. A propósito de siete observaciones. Actas Urol Esp 1995;19:772-6.

  14. Alonso A, Domínguez F, Verez M, Rodríguez B, Benavente J, Barros JM, et al. Síndrome de Wünderlich por metástasis de sarcoma gástrico en riñón. Aportación de un caso. Actas Urol Esp 1996;20:395-8.

  15. Albi G, Del Campo L, Tagarro D. Wünderlich´s syndrome: causes, diagnosis and radiological management. Clin Radiol 2002;57:840-5.

  16. Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria. J Child Neurol 1998;13:624-8.

  17. European Chromosome 16 Tuberous Sclerosis Consortium. Identification and characterization of the tuberous sclerosis gene on chromosome 16. Cell 1993;75:1305-15.

  18. Northrup H, Koenig MK, Au KS. Tuberous sclerosis complex. In: Adam MP, Ardinger HH, Pagon RA, Wallace SE, et al., editores. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2014, 13 juillet 1999 [mise à jour 23 novembre 2011].

  19. Rouvière O, Nivet H, Grenier N, Zini L, Lechevallier E. Kidney damage due to tuberous sclerosis complex: management recommendations. Prog Urol 2012; 22:367-79.

  20. Jozwiak S, Schwartz RA, Janniger CK, Bielicka-Cymerman J. Usefulness of diagnostic criteria of tuberous sclerosis complex in pediatric patients. J Child Neurol 2000;15:652-9.

  21. Rakowski SK, Winterkorn EB, Paul E, Steele DJ, et al. Renal manifestations of tuberous sclerosis complex: Incidence, prognosis, and predictive factors. Kidney Int 2006;70:1777-82.

  22. Ballanger F, Quéreux G, Stalder JF, Schmitt S, Jacquemont S. Sclérose tubéreuse de Bourneville. EMC (Elsevier Masson SAS, Paris), Dermatologie, 98-760-A-10, 2005:8.

  23. Bissler JJ,. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. doi: 10.1056/NEJMoa063564.

  24. Krueger DA, Northrup H, International Tuberous Sclerosis Complex Consensus Group. Tuberous sclerosis complex surveillance and management: recommendations of the 2012 International Tuberous Sclerosis Complex Consensus Conference. Pediatr Neurol 2013;49:255-65.

  25. Serra A, Bonny O, Bürki S, Dorn T, Fuster D, Guzman R, et al. La sclérose tubéreuse de Bourneville: pathogenèse, clinique et nouvelles options thérapeutiques. Forum Med Suisse 2013;13:696-702.

  26. Northrup H, et al. Tuberous sclerosis complex diagnostic criteria update: recommendations of the 2012 international tuberous sclerosis complex consensus conference. doi: 10.1016/j.pediatrneurol.2013.08.001.

  27. Schwartz RA, Fernández G, Kotulska K, Józwiak S. Tuberous sclerosis complex: advances in diagnosis, genetics, and management. J Am Acad Dermatol 2007;57:189-202.

  28. Grasso M, Blanco S, Fortuna F, Crippa S, Di Bella C. Spontaneous rupture of renal leiomyosarcoma in a 45-year-old woman. Arch Esp Urol 2004;57:870-2.

  29. Hao LW, Lin CM, Tsai SH. Spontaneous hemorrhagic angiomyolipoma presents with massive hematuria leading to urgent nephrectomy. Am J Emerg Med. 2008;26:249e3-5.

  30. Sales R, Villa V, Caballé J, Mas A, Valencoso O, Ballús L, et al. Síndrome de Wünderlich. Hemorragia renal espontánea. Cir Esp 2000;68:493-5.

  31. Moratalla MB. Wunderlich’s syndrome due to spontaneous rupture of large bilateral angiomyolipomas. doi: 10.1136/ emj.2008.062091.

  32. Hernandez F, Ong AM, Rha KH, Pinto PA, Kavoussi LR. Laparoscopic renal surgery after spontaneous retroperitoneal hemorrhage. J Urol 2003;170(3):749-51.




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