Hemorrhagic polycystic liver disease without the presence of polycystic kidney disease, a serious life-threatening condition: A case report. April 2015, Peru
DOI:
https://doi.org/10.24265/horizmed.2018.v18n1.12Keywords:
Liver diseases, Polycystic liver, LaparoscopyAbstract
Polycystic liver disease without the presence of polycystic kidney disease is a rare clinical condition and its hemorrhagic complication is even rarer, with very few cases being reported. It can be life-threatening without a rapid intervention. Case report: A 40 year-old female who presented with moderate sharp right upper quadrant abdominal pain and nausea for one hour. Emergency department: blood pressure 130/80 mmHg, heart rate 100 beats per minute, temperature 37 °C, O2 saturation 99 %, respiratory rate 22 breaths per minute. On examination, soft and relaxed abdomen, right upper quadrant abdominal pain, no visceromegaly, positive Murphy’s sign and rebound sign. Labs: hematocrit 34 %, hemoglobin 11.4 g/dL, white blood cells 12190, amylase 70 U/L. Abdominal ultrasound: Polycystic liver disease with ruptured cyst, abundant free subcapsular fluid within cavity, without pancreas and kidneys injury. Procedures: emergency exploratory laparoscopy, fluid drainage and aspiration, and partial cystectomy. Favorable evolution. Appointment with patient´s direct relatives for further studies.
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Chanana L, Jegaraj M, Kalyaniwala K, Yadav B, Abilash K. Clinical profile of non-traumatic acute abdominal pain presenting to an adult emergency department. J Family Med Prim Care. 2015;4(3):422-5. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4535107/
Marrero J, Ahn J, Rajender K. American College of Gastroenterology. Clinical guideline: the diagnosis and management of focal liver lesions. Am J Gastroenterol. 2014;109(9):1328-47. Disponible en: https://www.nature.com/articles/ajg2014213
Peces R, González P, Venegas J. Enfermedad poliquistica hepatica no asociada a poliquistosis renal autosomica dominante. Nefrología. 2003;23(5):454-458. Disponible en: http://www.revistanefrologia.com/es-publicacion-nefrologia-articulo-enfermedad-poliquistica-hepatica-no-asociada-poliquistosis-renal-autosomica-dominante-X0211699503016241
Russell R, Pinson C. Surgical management of polycystic liver disease. World J Gastroenterol. 2007;13(38):5052-9. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4434633/
Patel A, Shah R. Polycystic liver disease presenting as acute abdomen. QJM. 2014;107(9):769. Disponible en: https://www.ncbi.nlm.nih.gov/pubmed/24453280
Janssen M, Waanders E, Te Morsche R, Xing R, Dijkman H, Woudenberg J, et al. Secondary, somatic mutations might promote cyst formation in patients with autosomal dominant polycystic liver disease. Gastroenterology. 2011;141(6):2056-63. Disponible en: https://www.ncbi.nlm.nih.gov/pubmed/21856269
Banales J, Munoz-Garrido P, Bujanda L. Somatic second-hit mutations leads to polycystic liver diseases. World J Gastroenterol. 2013;19(1):141-3. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3542753/
Van Keimpema L, De Koning D, Van Hoek B, Van Den Berg A, Van Oijen M, De Man R, et al. Patients with isolated polycystic liver disease referred to liver centres: clinical characterization 137 cases. Liver Int. 2011;31(1):92-8. Disponible en: https://www.ncbi.nlm.nih.gov/pubmed/20408955
Hogan M, Abebe K, Torres V, Chapman A, Bae K, Tao C, et al. Liver involvement in early autosomal-dominant polycystic kidney disease. Clin Gastroenterol Hepatol. 2015;13(1):155-64. Disponible en: https://www.ncbi.nlm.nih.gov/pubmed/25111236
Gevers T, de Koning D, Van Dijk A, Drenth J. Low prevalence of cardiac valve abnormalities in patients with autosomal dominant polycystic liver disease. Liver Int. 2012;32(4):690-2. Disponible en: https://www.ncbi.nlm.nih.gov/pubmed/22099398
Gigot J, Jadoul P, Que F, Van Beers B, Etienne J, Horsmans Y, et al. Adult polycystic liver disease: is fenestration the most adequate operation for long-term management?. Ann Surg. 1997;225(3):286-94. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1190679/
Abu-Wasel B, Walsh C, Keough V, Molinari M. Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases. World J Gastroenterol. 2013;19(35):5775-86. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3793132/
Grosso G, Gruttadauria S, Biondi A, Marventano S, Mistretta A. Worldwide epidemiology of liver hydatidosis including the Mediterranean area. World J Gastroenterol. 2012;18(13):1425-37. Disponible en: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3319938/
García P, Chabez A, Fernández M, Guerra M, Levano I, Quiroz S, et al. Características clínicas y epidemiológicas de hidatidosis en el Hospital Nacional Dos de Mayo. Revista de Medicina Humana-Universidad R Palma. 2006;6(2):26-30. Disponible en: http://aulavirtual1.urp.edu.pe/ojs/index.php/RFMH/article/viewFile/23/19
Temmerman F, Dobbels F, Ho TA, Pirson Y, Vanslembrouck R, Coudyzer W, et al. Development and validation of a polycystic liver disease complaint-specific assessment (POLCA). J Hepatol. 2014;61(5):1143-50. Disponible en: http://www.sciencedirect.com/science/article/pii/S0168827814004541
Spirli C, Okolicsanyi S, Fiorotto R, Fabris L, Cadamuro M, Lecchi S, et al. ERK1/2-dependent vascular endothelial growth factor signaling sustains cyst growth in polycystin-2 defective mice. Gastroenterology. 2010;138(1):360-71. Disponible en: https://www.ncbi.nlm.nih.gov/pubmed/19766642
Temmerman F, Vanslembrouck R, Coudyzer W, Bammens B, Laleman W, Cassiman D, et al. The reduction in liver volume in polycystic liver disease with lanreotide is dose dependent and is most pronounced in patients with the highest liver volume. J. Hepatol. 2012;56(2):S547.Disponible en: https://www.infona.pl/resource/bwmeta1.element.elsevier-7e1a775b-dd99-3813-9557-1873c893ad76
Duarte-Chang C. Avances en el manejo de la Poliquistosis Hepática. Rev méd cient. 2012;25(2):26-31. Disponible en: http://www.revistamedicocientifica.org/uploads/journals/1/articles/367/public/367-1556-1-PB.pdf
Ampuero J, Bañales J, Soriano G, Crespo J, Olcoz J, Diago M, et al. La poliquistosis hepática del adulto (PHA) en España: análisis de una encuesta estructurada analizando la experiencia y actitud de los especialistas de digestivo españoles. Rev Esp Enferm Dig. 2014;106(4):263-75. Disponible en: http://scielo.isciii.es/scielo.php?pid=S1130-01082014000400005&script=sci_arttext&tlng=en
Dietrich A, Ardiles V, Lendoire J, Raffin G, Moro M, Storck G, et al. Liver resection for non tumoral liver disease. Multicentric experience in Argentina. Actas Gastroenterol Latinoam. 2014;44(2):114-20. Disponible en: http://europepmc.org/abstract/med/25199305
Tseng J, Orloff SL. Management of symptomatic polycystic liver disease with hepatic resection. JAMA Surg. 2015;150(1):81-2. Disponible en: https://jamanetwork.com/journals/jamasurgery/fullarticle/1934726
Marion Y, Brevart C, Plard L, Chiche L. Hemorrhagic liver cyst rupture: an unusual life-threatening complication of hepatic cyst and literatura review. Ann. Hepatol 2013;12(2):336-9. Disponible en: http://www.medigraphic.com/pdfs/hepato/ah-2013/ah132w.pdf
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