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Background: Liver cysts occur with a prevalence of 4%-7% in the general population. Laparoscopic surgery is effective for solitary cysts and in selected patients with polycystic liver disease (PLD). We present our experience in the laparoscopic management of dysontogenetic cysts.
Patients And Methods: Between 1994 and 2002, 36 patients were referred to our centre for the management of dysontogenetic cystic liver disease. Management was laparoscopic in 16 cases. Indications were solitary giant cysts (n=9) and PLD (n=7).
Results: Laparoscopic procedures were completed in 15 patients. Mean operating time was 90 min. There were no deaths. In one case there was an intraoperative complication: bleeding from a superficial hepatic vein necessitated conversion to an open procedure. There were two postoperative complications: one patient with biliary leakage, which was managed conservatively, and one patient with a pneumothorax caused by the cava catheter installed for anaesthesia. Median follow-up was 36 months. There was no symptomatic recurrence.
Conclusion: Laparoscopy can be recommended as the procedure of choice for symptomatic solitary giant cysts and PLD Gigot type I.
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http://dx.doi.org/10.1007/s00423-004-0506-7 | DOI Listing |
J Clin Endocrinol Metab
January 2021
Minneapolis, Minnesota, USA.
Context: Pituitary blastoma is a rare, dysontogenetic hypophyseal tumor of infancy first described in 2008, strongly suggestive of DICER1 syndrome.
Objective: This work aims to describe genetic alterations, clinical courses, outcomes, and complications in all known pituitary blastoma cases.
Design And Setting: A multi-institutional case series is presented from tertiary pediatric oncology centers.
J Maxillofac Oral Surg
June 2019
2Oral and Maxillofacial Surgery, Swami Devi Dyal Hospital and Dental College, Barwala, Panchkula, Haryana India.
The maxillofacial region can be affected by a number of subcutaneous pathologic conditions that include developmental, inflammatory, infective and neoplastic entities. Many of these lesions present as a soft to firm painless swelling. Differential diagnosis of such lesions requires thorough knowledge of maxillofacial pathology as well as anatomy to come to a correct diagnosis and provide effective treatment.
View Article and Find Full Text PDFViszeralmedizin
February 2015
Department of Internal Medicine, DRK Kliniken Berlin - Köpenick, Berlin, Germany.
Background: Pancreatic cystic lesions (PCL) are common. They are increasingly detected as an incidental finding of transabdominal ultrasound or cross-sectional imaging. In contrast to other parenchymal organs, dysontogenetic pancreatic cysts are extremely rare.
View Article and Find Full Text PDFViszeralmedizin
February 2015
Department of Medicine A, University Medicine, Ernst Moritz Arndt University Greifswald, Germany.
Background: Increased usage of computed tomography and magnetic resonance imaging has led to a large increase in identified pancreatic cysts of up to 25% in population-based studies. The clinical and economic relevance of identifying so many cystic lesions has not been established. Compared to other organs such as liver or kidney, dysontogenetic pancreatic cysts are rare.
View Article and Find Full Text PDFBr J Ophthalmol
July 2011
International Pleuropulmonary Blastoma Registry, Children's Hospitals and Clinics of Minnesota, Minneapolis, MN 55404, USA.
Background And Aims: Ciliary body medulloepithelioma (CBME) is a rare embryonal ocular tumour of children under age 10 years. Pleuropulmonary blastoma (PPB) is a rare embryonal lung tumour in young children and the sentinel disease of the PPB Family Tumour and Dysplasia Syndrome, a distinctive predisposition leading to unusual dysontogenetic-dysplastic and neoplastic conditions in PPB patients and their relatives. Germline mutations of DICER1 gene, a key regulator of gene silencing, underlie this syndrome.
View Article and Find Full Text PDF