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Intrahepatic biliary cysts encompass a large lesional spectrum including hereditary diseases as polycystic liver disease or Caroli's syndrome, malformative lesions as non hereditary Caroli's disease or simple biliary cyst and true neoplastic lesions as cystadenoma or cystadenocarcinoma. The diagnostic approach of these lesions relies firstly on imaging. Nevertheless, the pathologist not exceptionally receives surgical specimens from cystic fenestration or liver specimen resection with one or several cystic lesions. The clues for pathological diagnosis of these lesions have to be known by pathologists. As regards neoplastic cystic lesions, true non-communicating cystic tumors and cystic variants of intraductal biliary tumors have to be distinguished; in both cases, the classification is now identical to the one of pancreatic cystic tumors., (Copyright © 2010 Elsevier Masson SAS. All rights reserved.)

Introduction: There are very few observations of filiform palmo-plantar hyperkeratosis reported. Nevertheless it's worth knowing this entity for his potential association with a visceral neoplasia., Case Report: We report the first case of filiform palmo-plantar hyperkeratosis associated with a digestive adenocarcinoma and a polycystic kidney disease., Discussion: After a review of palmar and plantar filiform hyperkeratosis in the literature, we will discuss the possible association with neoplasia or other pathologies. This pathology requires a strict clinical and paraclinical follow-up.

The prevalence of multicystic dysplastic kidney has increased since the development of antenatal ultrasound diagnosis. Even though, the rarity of symptomatic forms and case reports of spontaneous regressions antenatally or after postnatal ultrasound follow-up have reduced indications of nephrectomy which is less and less appropriate. However, discovery of ectopic ureteric implantation in boys makes certain additional investigations as intravenous pyelography, cystography and ultrasound, essential before making any therapeutic decision. Nevertheless, some cases remain undescriptible. In these cases, operative opacification of any patent ureter can provide more precise information about ureteric implantation. Lastly, only a dissection and resection as complete as possible of any patent ureter and pelvic cystic dilatation can prevent subsequent complications.

Twenty one cases of spontaneous urine extravasation during an intravenous urography have been analysed in order to determinate the radiological signs and the causes of this rare phenomenon. Related to the increased pressure of the excretory tract, these extravasations usually disappear without complications. No specific treatment is required except the treatment of the obstacle due generally to calculi. No infection nor development of retroperitoneal fibrosis nor sinusal lipomatosis were observed in the patients we studied.

Five cases of intracranial aneurysms with polycystic kidneys are reported: --15% of polycystic kidneys have an intracranial aneurysm, and about 4% - 5% of intracranial aneurysms are joined with polycystic kidneys; --the surgical treatment of intracranial aneurysm with this association doesn't present more risk than an intracranial aneurysm, and the surgical prognosis isn't bad; --the pathogeny isn't clear. This association is perhaps a congenital elastic system disease.

In 3 cases, total parathyroidectomy, with graft of fragments of one of the glands into the forearm gave rapid correction of the clinical and biological disorders associated with secondary hyperparathyroidism. The secretion of parathyroid hormone by the autograft was confirmed by radioimmunoassay on brachial vein samples. This technique appears to be more satisfactory than 7/8 parathyroidectomy, the postoperative course of which is not always free of complications, which involves a risk of necrosis of the gland fragment left in place and in which reoperation is difficult.

Congenital hepatic fibrosis is nearly always associated with ectasia of collecting tubules of the kidneys. This abnormality usually remains silent. In this study we report three cases of adult's CHF with associated renal failure treated by hemodialysis. In all three cases, renal injuries were indistinguishable from those found in adult-type of polycystic disease. The kidneys of our third patient, who underwent two nephrectomies at a 14-years interval, showed ectasia of the collecting tubules with only a few cortical cysts. The second one showed numerous large cysts and only a few ectatic tubules. Our data indicate that: renal failure can complicate the CHF course in adults. Uremia can be the pressenting feature; polycystic kidneys in CHF are microscopically different from those found in adult-type, they might be considered as the final stage in ectasia of collecting tubules.

Six cases of recessive hepatorenal polycystic disease in infants and young children were explored by intravenous urography and ultrasonography. Sufficient specific ultrasonography findings for making a diagnosis were obtained. The kidneys are large and produce strong echos, with inversion of the normal strength of medullary when compared to cortical echos, and poor definition of the renal sinus. Strong echos are also obtained from the liver, especially in the juvenile forms, because of the large amount of fibrosis. Large intrahepatic biliary ectasias were discovered in a 3-year-old child.