Insuficiencia hepática secundaria a enfermedad poliquística renal-hepática

La enfermedad poliquística renal autosómica dominante es considerada la enfermedad renal genética más frecuente y es la cuarta causa de enfermedad renal crónica a nivel mundial. Afecta a cerca de 1 por cada 1000 nacidos vivos. La enfermedad poliquística hepática es la manifestación extrarrenal más frecuente de la enfermedad poliquística renal. La cirrosis hepática constituye un hallazgo tardío en esta enfermedad y su presentación es más común en el anciano, y la descompensación de la enfermedad con insuficiencia hepática como causa de muerte. Presentamos el caso de un paciente masculino de 60 años con antecedentes patológicos personales de la enfermedad poliquística renal con ascitis a tensión, y edemas en ambos miembros inferiores con datos de insuficiencia hepática y evolución desfavorable hasta su fallecimiento. Aunque la presencia de la falla hepática se asocia a un pronóstico precario, la detección precoz puede suponer el inicio de un tratamiento oportuno y apropiado que puede ser beneficioso.

Autosomal dominant polycystic kidney disease is considered the most common genetic kidney disease and the fourth leading cause of chronic kidney disease worldwide. It affects about 1 in 1,000 live births. Polycystic liver disease is the most common extrarenal manifestation of polycystic kidney disease. Liver cirrhosis is a late finding in this disease and its presentation is more common in the elderly. Decompensation of the disease, with liver failure as the cause of death, is rare in polycystic liver disease. We report the case of a 60-year-old male patient with a personal pathological history of polycystic kidney disease with tense ascites and edema in both lower limbs, with evidence of liver failure and unfavorable evolution until his death. Although the presence of liver failure is associated with poor prognosis, its early detection may mean the initiation of timely and appropriate treatment that may be beneficial.

Enfermedad poliquística: a propósito de un caso

RESUMEN La enfermedad renal poliquística autosómica recesiva es una de las grandes causas de insuficiencia renal crónica en la población adulta. Se reporta el caso de un paciente con este padecimiento, que presentó, además, poliquistosis hepática y quistes pancreáticos, con escasas manifestaciones clínicas de la esfera renal y síntomas dispépticos; hígado y riñones muy aumentados de tamaño e irregulares, producto de los abundantes y grandes quistes; fosfatasa alcalina elevada y comprobación imagenológica de la enfermedad. Todo esto unido lo hace un caso infrecuente.

ABSTRACT Autosomal recessive polycystic renal disease is one of the great causes of chronic renal failure in the adult population. We present the case of a patient with this condition, who also had liver and pancreatic cysts, with few clinical manifestations of the renal sphere and dyspeptic symptoms; very enlarged and irregular liver and kidneys due to profuse and large cysts; high alkaline phosphatase and imaging checking of the disease. All of these together make it an infrequent case.

Cystic Disease of the Liver / 대한내과학회지

Cystic lesions of the liver in the adult can be classified as developmental, neoplastic, inflammatory, or miscellaneous. They occur in symptomatic or asymptomatic patients with or without preexisting liver disease. Many of the lesions represent a diagnostic dilemma for the clinician and usually a single imaging study is not diagnostic. The physician must understand the clinical, radiologic, and pathologic characteristics of these cystic lesions including benign lesion such as simple (bile duct) cyst, autosomal dominant polycystic liver disease, biliary hamartoma, Caroli disease, congenital hepatic fibrosis, and premalignant lesion such as biliary cystadenoma and cystadenocarcinoma. In this review, we review the clinical, radiological, and histologic features of the most common benign cystic liver disease and also present current management options. An understanding of these facts will allow more definitive diagnosis and shorten the diagnostic workup for focal cystic liver lesions.

Peribiliary Cysts with Intrahepatic Bile Duct Obstruction: A Case Report

Peribiliary cysts involve cystic dilatation and necroinflammatory change in the peribiliary glandular tissue of the larger biliary tree in association with portal hypertension or severe hepatobiliary disease. They are usually asymptomatic and found incidentally. However, rare cases causing symptoms or the mass effect of bile duct compression require differential diagnosis. They may be benign or malignant lesions resulting from narrowing of the bile duct and involving dilatation of the proximal intrahepatic bile duct. We recently encountered a case of peribiliary cysts associated with intrahepatic duct dilatation, and confirmed by surgery, and present the imaging and pathologic findings. Sonography and CT revealed the presence of multiple cystic lesions along the hepatic hilum, and the larger left portal tract and left intrahepatic ducts were dilated. Histopathologic examination indicated that the cysts were compressing the central bile duct.

Tetracycline Hydrochloride Sclerotherapy: Renal, Hepatic, Ovarian, and Perivesical cysts

PURPOSE: To assess the efficacy and resulting complications of tetracycline sclerotherapy in renal, hepatic, ovarian, and perivesical cysts. MATERIALS AND METHODS: We retrospectively reviewed 23 cases of benign cysts (16 renal, 4 hepatic, 2 ovarian, and 1 perivesical) in 22 patients in whom the condition was diagnosed or confirmed by either ultrasound, CT, or cytology, and who underwent percutaneous tetracycline sclerotherapy. Using a 21-gauge Chiba needle, the target cyst was punctured under ultrasound guidance. Prior to the injection of 1500 mg of tetracycline diluted in 5 ml of normal saline, almost all the cystic content was aspirated, and at the end of the procedure the tetra-cycline was left in the cyst. During a period of between 3 and 22 months, 18 of the 23 cases were followed up. RESULT: In six of the 18 cases followed up, the cysts either decreased in size by 10%, or collapsed completely. In seven cases a collapse of over 50% was noted, and in the remaining five the cyst recurred. In one of these, complete collapse occurred after retreatment at ten months, and the patient with a perivesical cyst underwent surgery six months after recurrence. Thus, treatment was effective (a collapse of at least 50%) in 13 of 18 cases (72.2%). This total of 13 comprised ten of 12 renal cysts (83.3%), two of two ovarian (100%), and one of three hepatic (33.3%). Percutaneous therapy was unsuccessful in five cases (two hepatic cysts, one renal, one para-pelvic and one perivesical). Complications occurring during the procedure or follow-up period included discomfort or mild pain, vomiting, and transient fever, though these subsided within 24 hours. In one patient with severe pain, this subsided after four days. CONCLUSION: As single-shot injection of tetracycline provides safe and effective treatment for renal and ovarian cysts, but for hepatic cysts is unsuccessful.

CT Findings of Ciliated Hepatic Foregut Cyst Mimicking Metastasis: A Case Report

Ciliated hepatic foregut cyst (CHFC) is a rare benign lesion consisting of ciliated pseudostratified columnar epithelium, loose subepithelial connective tissue, a smooth muscle layer, and an outer fibrous capsule. We encountered a patient with retroperitoneal and posterior mediastinal neurilemmoma and additional CHFC mimicking metastasis. Abdominal CT examination demonstrated that the posterior mediastinal and retroperitoneal lesions were lobulated, well-defined tumors with a neural foraminal extension that were pathologically confirmed as neurilemmomas. Unenhanced CT indicated that the additional lesion was a slightly hypodense mass relative to surrounding parenchyma at the medial segment of the left lobe of the liver, and after the IV ad-ministration of contrast material, the lesion did not show enhancement.