An unusual association: Jaffe-Linchtenstein syndrome, arcuate ligament syndrome and hepatic adenoma

A case of a woman affected by an unusual association of rare diseases, is presented. The patient was referred to our department for acute anaemia. Preoperative investigations revealed that the patient was affected by fibrous polyostotic dysplasia, so called Jaffè-Lichtenstein syndrome. The presence...

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Bibliographic Details
Published inMinerva chirurgica Vol. 51; no. 3; p. 167
Main Authors Fontana, D, Leli, R, Della Beffa, V
Format Journal Article
LanguageItalian
Published Italy 01.03.1996
Subjects
Adenoma - complications
Adenoma - pathology
Adenoma - surgery
Adult
Female
Fibrous Dysplasia, Polyostotic - complications
Fibrous Dysplasia, Polyostotic - diagnosis
Hemorrhage - complications
Humans
Liver - pathology
Liver - surgery
Liver Neoplasms - complications
Liver Neoplasms - pathology
Liver Neoplasms - surgery
Syndrome
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Summary:A case of a woman affected by an unusual association of rare diseases, is presented. The patient was referred to our department for acute anaemia. Preoperative investigations revealed that the patient was affected by fibrous polyostotic dysplasia, so called Jaffè-Lichtenstein syndrome. The presence of skin brown spot and endocrine disorders requiring pill administration, allows to classify the patient as carrier of Albright syndrome. Moreover, the angiography pointed out a celiac trunc stenosis (Dunbar syndrome). The long-standing administration of the pill could be the cause of bleeding adenoma. The patient underwent hepatic resection. We did not treat the Dunbar syndrome because of poor symptoms. From literature, we review some opinions on the fibrous dysplasia of the bone.
ISSN:0026-4733