This article is published in the forthcoming issue.

A rare cause of childhood chylothorax: Gorham-Stout disease

Gorham-Stout disease in children

Authors

Keywords:

Gorham Stout Disease, Chylothorax, Pleural Effusion, Sirolimus

Abstract

Gorham syndrome is an extremely rare bone disease, with only a limited number of cases reported worldwide. It is characterized by progressive bone resorption associated with abnormal proliferation of lymphatic vessels, making it a rare case in medical literature. This disorder can lead to serious complications including chylothorax, especially in children. Early diagnosis and treatment are crucial in the prevention of life-threatening outcomes. This report presents a case of a young child with recurrent pleural effusion and bone lesions, eventually diagnosed with Gorham's disease. A three-year-old patient was admitted with recurrent pleural effusion, chylothorax, and pneumothorax. Imaging revealed multiple lytic bone lesions, suggestive of bone destruction. A lung biopsy confirmed lymphatic dilation, supporting a diagnosis of Gorham's disease. The patient’s condition was evaluated through a multidisciplinary approach involving pulmonology, pathology, and radiology teams. Treatment included a combination of pharmacotherapy and dietary modifications, which successfully stabilized the patient’s condition. The child has since been closely monitored, with no significant recurrence of symptoms. The progression of the disease can be life-threatening, particularly when the thoracic duct is involved, leading to recurrent pleural effusions. In this particular case, early diagnosis through a collaborative medical approach allowed for prompt treatment. Ongoing monitoring is essential in preventing relapses and ensuring long-term health outcomes for the patient.

Downloads

Download data is not yet available.

Author Biography

Ayşe Nur Toksöz Yıldırım, Istanbul Medeniyet University, Department of Pathology, Istanbul, Turkey

Department of Pathology

References

Haines C, Walsh B, Fletcher M, Davis PJ. Chylothorax development in infants and children in the UK. Arch Dis Child. 2014;99(8):724–30.

Dellinger MT, Garg N, Olsen BR. Viewpoints on vessels and vanishing bones in Gorham-Stout disease. Vol. 63, Bone. Elsevier Inc.; 2014. p. 47–52.

Ozeki M, Fujino A, Matsuoka K, Nosaka S, Kuroda T, Fukao T. Clinical Features and Prognosis of Generalized Lymphatic Anomaly, Kaposiform Lymphangiomatosis, and Gorham-Stout Disease. Pediatr Blood Cancer. 2016 May 1;63(5):832–8.

Riley LE, Ataya A. Clinical approach and review of causes of a chylothorax. Vol. 157, Respiratory Medicine. W.B. Saunders Ltd; 2019. p. 7–13.

Büyükşahin HN, Emiralioğlu N, Özcan HN, Sunman B, Güzelkaş İ, Alboğa D, et al. Evaluation of Clinical Findings in Children with Chylothorax: A Descriptive Study. Turkish Archives of Pediatrics. 2023 Jan 1;58(1):28–33.

Kotecha R, Mascarenhas L, Jackson HA, Venkatramani R. Radiological features of Gorham’s disease. Clin Radiol. 2012;67(8):782–8.

Ozeki M, Fukao T. Generalized Lymphatic Anomaly and Gorham-Stout Disease: Overview and Recent Insights. Vol. 8, Advances in Wound Care. Mary Ann Liebert Inc.; 2019. p. 230–45.

Wiegand S, Dietz A, Wichmann G. Efficacy of sirolimus in children with lymphatic malformations of the head and neck. Vol. 279, European Archives of Oto-Rhino-Laryngology. Springer Science and Business Media Deutschland GmbH; 2022. p. 3801–10.

Downloads

Published

2025-07-13

Issue

Section

Case Report

How to Cite

1.
Mocan Cağlar Y, Can Oksay S, Toksöz Yıldırım AN, Bozbeyoğlu SG, Girit S. A rare cause of childhood chylothorax: Gorham-Stout disease: Gorham-Stout disease in children. J Surg Med [Internet]. 2025 Jul. 13 [cited 2025 Jul. 16];9(7):00-. Available from: https://jsurgmed.com/article/view/8059