Surgical Management of Gorham-Stout Disease in Cervical Compression Fracture with Cervicothoracic Fusion

Case Report and Review of Literature

Jung Hwa Kim, Do Heum Yoon, Keung Nyun Kim, DongAh Shin, Seong Yi, Jiin Kang, Yoon Ha

Research output: Contribution to journalArticle

Abstract

Background: Gorham-Stout disease (GSD) or “vanishing bone” disease is characterized by progressive osteolysis with intraosseous lymphangiomatosis (hemangiomatosis). Given its rarity, with about 300 reported cases, its pathophysiology, etiology, and treatment guidelines are not established yet. Case Description: A 22-year-old man was admitted to Severance Hospital with the chief complaint of neck pain from an injury due to falling. Initial cervical radiography showed a C4 burst fracture, and cervical magnetic resonance imaging revealed diffuse osteolytic lesions with coarse trabeculation with T2 hyperintensity and T1 enhancement in the entire cervical and upper thoracic area. He had a previous history of chylothorax that was still noticeable on a chest radiograph at the time of admission. A 2-stage operation was conducted. First, anterior corpectomy of C4 and anterior plate fixation of C3-5 were performed. Second, a week later, posterior fixation of C3-5 was performed. Thereafter, the patient was discharged without any neurologic complications. However, during the 1-month follow-up, asymptomatic progressive kyphosis was detected via radiography, and posterior cervical fusion of C2-T4 was performed. A minimal postoperative symptom of an intermittent left arm pain of 4–5 on the visual analog scale was experienced. No further deformity progression was noted until the last outpatient follow-up. Conclusions: Spinal GSD can cause severe deformity and neurologic deficits such as paralysis. Although treatment for GSD is not established, surgical treatment is recommended in severe deformity or aggravated neurologic deficit. The appropriate timing of surgery is after the arrest of osteolysis. Magnetic resonance imaging could be helpful in determining stable GSD.

Original languageEnglish
Pages (from-to)277-281
Number of pages5
JournalWorld Neurosurgery
Volume129
DOIs
Publication statusPublished - 2019 Sep 1

Fingerprint

Essential Osteolysis
Compression Fractures
Osteolysis
Neurologic Manifestations
Radiography
Thorax
Accidental Falls
Magnetic Resonance Imaging
Neck Injuries
Chylothorax
Kyphosis
Neck Pain
Bone Diseases
Visual Analog Scale
Paralysis
Nervous System
Arm
Outpatients
Therapeutics
Guidelines

All Science Journal Classification (ASJC) codes

  • Surgery
  • Clinical Neurology

Cite this

Kim, Jung Hwa ; Yoon, Do Heum ; Kim, Keung Nyun ; Shin, DongAh ; Yi, Seong ; Kang, Jiin ; Ha, Yoon. / Surgical Management of Gorham-Stout Disease in Cervical Compression Fracture with Cervicothoracic Fusion : Case Report and Review of Literature. In: World Neurosurgery. 2019 ; Vol. 129. pp. 277-281.
@article{6bae132408be450e9841b4596befab11,
title = "Surgical Management of Gorham-Stout Disease in Cervical Compression Fracture with Cervicothoracic Fusion: Case Report and Review of Literature",
abstract = "Background: Gorham-Stout disease (GSD) or “vanishing bone” disease is characterized by progressive osteolysis with intraosseous lymphangiomatosis (hemangiomatosis). Given its rarity, with about 300 reported cases, its pathophysiology, etiology, and treatment guidelines are not established yet. Case Description: A 22-year-old man was admitted to Severance Hospital with the chief complaint of neck pain from an injury due to falling. Initial cervical radiography showed a C4 burst fracture, and cervical magnetic resonance imaging revealed diffuse osteolytic lesions with coarse trabeculation with T2 hyperintensity and T1 enhancement in the entire cervical and upper thoracic area. He had a previous history of chylothorax that was still noticeable on a chest radiograph at the time of admission. A 2-stage operation was conducted. First, anterior corpectomy of C4 and anterior plate fixation of C3-5 were performed. Second, a week later, posterior fixation of C3-5 was performed. Thereafter, the patient was discharged without any neurologic complications. However, during the 1-month follow-up, asymptomatic progressive kyphosis was detected via radiography, and posterior cervical fusion of C2-T4 was performed. A minimal postoperative symptom of an intermittent left arm pain of 4–5 on the visual analog scale was experienced. No further deformity progression was noted until the last outpatient follow-up. Conclusions: Spinal GSD can cause severe deformity and neurologic deficits such as paralysis. Although treatment for GSD is not established, surgical treatment is recommended in severe deformity or aggravated neurologic deficit. The appropriate timing of surgery is after the arrest of osteolysis. Magnetic resonance imaging could be helpful in determining stable GSD.",
author = "Kim, {Jung Hwa} and Yoon, {Do Heum} and Kim, {Keung Nyun} and DongAh Shin and Seong Yi and Jiin Kang and Yoon Ha",
year = "2019",
month = "9",
day = "1",
doi = "10.1016/j.wneu.2019.05.235",
language = "English",
volume = "129",
pages = "277--281",
journal = "World Neurosurgery",
issn = "1878-8750",
publisher = "Elsevier Inc.",

}

Surgical Management of Gorham-Stout Disease in Cervical Compression Fracture with Cervicothoracic Fusion : Case Report and Review of Literature. / Kim, Jung Hwa; Yoon, Do Heum; Kim, Keung Nyun; Shin, DongAh; Yi, Seong; Kang, Jiin; Ha, Yoon.

In: World Neurosurgery, Vol. 129, 01.09.2019, p. 277-281.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Surgical Management of Gorham-Stout Disease in Cervical Compression Fracture with Cervicothoracic Fusion

T2 - Case Report and Review of Literature

AU - Kim, Jung Hwa

AU - Yoon, Do Heum

AU - Kim, Keung Nyun

AU - Shin, DongAh

AU - Yi, Seong

AU - Kang, Jiin

AU - Ha, Yoon

PY - 2019/9/1

Y1 - 2019/9/1

N2 - Background: Gorham-Stout disease (GSD) or “vanishing bone” disease is characterized by progressive osteolysis with intraosseous lymphangiomatosis (hemangiomatosis). Given its rarity, with about 300 reported cases, its pathophysiology, etiology, and treatment guidelines are not established yet. Case Description: A 22-year-old man was admitted to Severance Hospital with the chief complaint of neck pain from an injury due to falling. Initial cervical radiography showed a C4 burst fracture, and cervical magnetic resonance imaging revealed diffuse osteolytic lesions with coarse trabeculation with T2 hyperintensity and T1 enhancement in the entire cervical and upper thoracic area. He had a previous history of chylothorax that was still noticeable on a chest radiograph at the time of admission. A 2-stage operation was conducted. First, anterior corpectomy of C4 and anterior plate fixation of C3-5 were performed. Second, a week later, posterior fixation of C3-5 was performed. Thereafter, the patient was discharged without any neurologic complications. However, during the 1-month follow-up, asymptomatic progressive kyphosis was detected via radiography, and posterior cervical fusion of C2-T4 was performed. A minimal postoperative symptom of an intermittent left arm pain of 4–5 on the visual analog scale was experienced. No further deformity progression was noted until the last outpatient follow-up. Conclusions: Spinal GSD can cause severe deformity and neurologic deficits such as paralysis. Although treatment for GSD is not established, surgical treatment is recommended in severe deformity or aggravated neurologic deficit. The appropriate timing of surgery is after the arrest of osteolysis. Magnetic resonance imaging could be helpful in determining stable GSD.

AB - Background: Gorham-Stout disease (GSD) or “vanishing bone” disease is characterized by progressive osteolysis with intraosseous lymphangiomatosis (hemangiomatosis). Given its rarity, with about 300 reported cases, its pathophysiology, etiology, and treatment guidelines are not established yet. Case Description: A 22-year-old man was admitted to Severance Hospital with the chief complaint of neck pain from an injury due to falling. Initial cervical radiography showed a C4 burst fracture, and cervical magnetic resonance imaging revealed diffuse osteolytic lesions with coarse trabeculation with T2 hyperintensity and T1 enhancement in the entire cervical and upper thoracic area. He had a previous history of chylothorax that was still noticeable on a chest radiograph at the time of admission. A 2-stage operation was conducted. First, anterior corpectomy of C4 and anterior plate fixation of C3-5 were performed. Second, a week later, posterior fixation of C3-5 was performed. Thereafter, the patient was discharged without any neurologic complications. However, during the 1-month follow-up, asymptomatic progressive kyphosis was detected via radiography, and posterior cervical fusion of C2-T4 was performed. A minimal postoperative symptom of an intermittent left arm pain of 4–5 on the visual analog scale was experienced. No further deformity progression was noted until the last outpatient follow-up. Conclusions: Spinal GSD can cause severe deformity and neurologic deficits such as paralysis. Although treatment for GSD is not established, surgical treatment is recommended in severe deformity or aggravated neurologic deficit. The appropriate timing of surgery is after the arrest of osteolysis. Magnetic resonance imaging could be helpful in determining stable GSD.

UR - http://www.scopus.com/inward/record.url?scp=85068510305&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85068510305&partnerID=8YFLogxK

U2 - 10.1016/j.wneu.2019.05.235

DO - 10.1016/j.wneu.2019.05.235

M3 - Article

VL - 129

SP - 277

EP - 281

JO - World Neurosurgery

JF - World Neurosurgery

SN - 1878-8750

ER -