Literature DB >> 34356962

Gorham-Stout Disease with Multiple Bone Involvement-Challenging Diagnosis of a Rare Disease and Literature Review.

Alina Momanu1, Lavinia Caba2, Nicoleta Carmen Gorduza3, Oana Elena Arhire4, Alina Delia Popa5, Victor Ianole6, Eusebiu Vlad Gorduza2.   

Abstract

Gorham-Stout disease is a rare disorder, which may result in a poor prognosis. This disease, a rare lymphangiomatosis, is defined by progressive bone disappearance due to massive unicentric and multicentric osteolysis. Osteolytic lesions of the spine and pleura effusion are poor prognostic factors. Herein, we will present a case where the onset of disease occurred at the age of 18 with asthenia, myalgia, and major bone pain, followed by incomplete motor deficiency in the lower limbs and, later, in the upper limbs. Imaging studies (CT scan and MRI) of the patient revealed osteolytic lesions (cervical and thoracic vertebrae, rib, and clavicle) and a pathological fracture of the C7 vertebra. Surgical procedures undertaken involved replacing the affected vertebrae with bone grafting and prosthesis. The investigations performed allowed for the exclusion of inflammation, thyroid or parathyroid disease, lymphoma, neoplasia, or autoimmune disorders. A bone marrow biopsy showed osteolysis, the replacement of bone tissues with connective tissue, and chronic non-specific inflammation. The evolution was negative with almost complete osteolysis of the left clavicle, the emergence of new osteolysis areas in the lumbar vertebrae, pelvic bones, and the bilateral proximal femur, splenic nodules, chylothorax, and associated major neurological deficits. Unfortunately, this negative evolution resulted in the patient's death a year after onset.

Entities:  

Keywords:  Gorham-Stout disease; osteolysis; spine lesions

Year:  2021        PMID: 34356962     DOI: 10.3390/medicina57070681

Source DB:  PubMed          Journal:  Medicina (Kaunas)        ISSN: 1010-660X            Impact factor:   2.430


  2 in total

Review 1.  Current concepts from diagnosis to management in Gorham-Stout disease: a systematic narrative review of about 350 cases.

Authors:  Andrea Angelini; Nicolò Mosele; Elisa Pagliarini; Pietro Ruggieri
Journal:  EFORT Open Rev       Date:  2022-01-11

2.  Gorham-Stout disease: A multirod lumbar reconstruction with off-label suppression-remission therapy.

Authors:  Ajay Krishnan; Aditya Raj; Devanand Degulmadi; Shivanand Mayi; Raviranjan Rai; Shiv Kumar Bali; Vatsal Parmar; Prarthan Chirag Amin; Preety Krishnan; Mirant Dave; Bharat Dave
Journal:  Surg Neurol Int       Date:  2022-04-08
  2 in total

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