ABSTRACT
ABSTRACT A 16-year-old female patient previously diagnosed with autosomal recessive polycystic kidney disease (ARPKD) presented with acute bilateral pneumonia, upper gastrointestinal bleeding caused by ruptured esophageal varices, ascites, and lower limb edema. She required intensive care and an endoscopic procedure to treat the gastrointestinal bleeding. The analysis of the differential diagnosis for chronic liver disease indicated she had a spontaneous splenorenal shunt. Ultrasound-guided biopsy revealed the patient had cirrhosis, as characteristically seen in individuals with ARPKD. She had no symptoms at discharge and was referred for review for a combined transplant.
RESUMO Relato de caso de uma paciente adolescente de 16 anos de idade com diagnóstico prévio de doença renal policística autossômica recessiva (DRPAR), que apresentou quadro agudo de pneumonia bilateral e hemorragia digestiva alta por ruptura de varizes esofágicas, bem como ascite e edema de membros inferiores. Necessitou de estabilização clínica intensiva e tratamento endoscópico do sangramento digestivo. Após investigação dos diagnósticos diferenciais da hepatopatia crônica, diagnosticou-se shunt esplenorrenal espontâneo, e realizou-se biópsia hepática guiada por ecografia com diagnóstico de cirrose, espectro típico da DRPAR. Recebeu alta hospitalar assintomática e foi encaminhada para avaliação de transplante duplo.
Subject(s)
Humans , Female , Adolescent , Arteriovenous Anastomosis/pathology , Polycystic Kidney, Autosomal Recessive/complications , Caroli Disease/complications , Liver Cirrhosis/complications , Arteriovenous Anastomosis/diagnostic imaging , Referral and Consultation , Renal Veins/diagnostic imaging , Biopsy , Brazil , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Intensive Care Units, Pediatric , Treatment Outcome , Polycystic Kidney, Autosomal Recessive/drug therapy , Polycystic Kidney, Autosomal Recessive/diagnostic imaging , Caroli Disease/pathology , Caroli Disease/drug therapy , Magnetic Resonance Angiography , Adrenergic beta-Agonists/therapeutic use , Diuretics, Potassium Sparing/therapeutic use , Liver Cirrhosis/pathology , Liver Cirrhosis/drug therapyABSTRACT
A 16-year-old female patient previously diagnosed with autosomal recessive polycystic kidney disease (ARPKD) presented with acute bilateral pneumonia, upper gastrointestinal bleeding caused by ruptured esophageal varices, ascites, and lower limb edema. She required intensive care and an endoscopic procedure to treat the gastrointestinal bleeding. The analysis of the differential diagnosis for chronic liver disease indicated she had a spontaneous splenorenal shunt. Ultrasound-guided biopsy revealed the patient had cirrhosis, as characteristically seen in individuals with ARPKD. She had no symptoms at discharge and was referred for review for a combined transplant.
Subject(s)
Arteriovenous Anastomosis/pathology , Caroli Disease/complications , Liver Cirrhosis/complications , Polycystic Kidney, Autosomal Recessive/complications , Adolescent , Adrenergic beta-Antagonists/therapeutic use , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Arteriovenous Anastomosis/diagnostic imaging , Biopsy , Brazil , Caroli Disease/drug therapy , Caroli Disease/pathology , Diuretics, Potassium Sparing/therapeutic use , Female , Humans , Intensive Care Units, Pediatric , Liver Cirrhosis/drug therapy , Liver Cirrhosis/pathology , Magnetic Resonance Angiography , Polycystic Kidney, Autosomal Recessive/diagnostic imaging , Polycystic Kidney, Autosomal Recessive/drug therapy , Referral and Consultation , Renal Veins/diagnostic imaging , Renal Veins/pathology , Treatment Outcome , Vena Cava, Inferior/diagnostic imaging , Vena Cava, Inferior/pathologyABSTRACT
The trigeminal artery (TA) is the most common embryonic carotid-vertebrobasilar anastomosis to persist into adulthood. It typically extends from the internal carotid artery to the basilar artery. Persistent primitive arteries are usually found incidentally, but are often associated with vascular malformation, cerebral aneurysm and, in case of TA, with trigeminal neuralgia. We present one patient with TA as a cause of trigeminal neuralgia and in other three as an incidental finding, on TC and MR angiograms.