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Child's Nervous System
Published in: Child's Nervous System 8/2018

01-08-2018 | Case Report

Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis

Authors: Cristina Barceló-López, Antonio L. López-Guerrero, Antonio García-López, José Hurtado-Marín, Helena Alarcón-Martínez, Juan F. Martínez-Lage

Published in: Child's Nervous System | Issue 8/2018

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Abstract

Case Report

A 4-year-old boy with kaposiform lymphangiomatosis (KLA) developed progressive headaches and papilloedema and was diagnosed with pseudotumor cerebri initially treated with acetazolamide. Clinical deterioration prompted placement of a ventriculoperitoneal shunt. After the surgery, the child’s condition has markedly improved.

Discussion and conclusions

 A network of intracranial lymphatics is presently being investigated. Neuroimaging excluded KLA infiltration of the skull and/or meninges, leaving as the most plausible explanation for the child’s pseudotumor cerebri the existence of an increase in intracranial venous pressure by venous compression at the thorax. To our knowledge, our case constitutes the first report of pseudotumor cerebri occurring in the context of KLA.
Literature
1.
2.
Goyal P, Alomari AI, Kozakewich HP, Trenor CC, Perez-Atayde AR, Fishman SJ, Greene AK, Shaikh R, Chaudry G (2016) Imaging features of kaposiform lymphangiomatosis. Pediatr Radiol 46(9):1282–1290CrossRefPubMed
3.
Ozeki M, Fujino A, Matsuoka K, Nosaka S, Kuroda T, Fukao T (2016) Clinical features and prognosis of generalized lymphatic anomaly, kaposiform lymphangiomatosis, and Gorham-Stout disease. Pediatr Blood Cancer 63(5):832–838CrossRefPubMed
4.
Croteau SE, Kozakewich HPW, Perez-Atayde AR, Fishman SJ, Alomari AI, Chaudry G, Mulliken JB, Trenor CC III (2014) Kaposiform lymphangiomatosis: a distinct aggressive lymphatic anomaly. J Pediatr 164(2):383–388CrossRefPubMed
5.
Fernandes VM, Fargo JH, Saini S, Guerrera MF, Marcus L, Luchtman-Jones L, Adams D, Meier ER (2015) Kaposiform lymphangiomatosis: unifying features of a heterogeneous disorder. Pediatr Blood Cancer 62(5):901–904CrossRefPubMed
6.
Patel MK, Mittelstaedt BR, Valentin FE, Thomas LP, Carlson CL, Faux BM, Hsieh DT (2016) Increased intracranial pressure in a boy with Gorham-stout disease. Case Rep Neurol 8(1):66–71CrossRefPubMedPubMedCentral
7.
Standridge SM (2010) Idiopathic intracranial hypertension in children: a review and algorithm. Pediatr Neurol 43(6):377–390CrossRefPubMed
8.
Suga K, Goji A, Inoue M, Kawahito M, Taki M, Mori K (2017) A case of generalized lymphatic anomaly causing skull-base leakage and bacterial meningitis. Brain and Development 39(5):444–447CrossRefPubMed
9.
Louveau A, Smirnov I, Keyes TJ, Eccles JD, Rouhani SJ, Peske JD, Derecki NC, Castle D, Mandell JW, Lee KS, Harris TH, Kipnis J (2015) Structural and functional features of central nervous system lymphatic vessels. Nature 523:337–341CrossRefPubMedPubMedCentral
Metadata
Title
Pseudotumor cerebri in kaposiform lymphangiomatosis: a case report and pathogenetic hypothesis
Authors
Cristina Barceló-López
Antonio L. López-Guerrero
Antonio García-López
José Hurtado-Marín
Helena Alarcón-Martínez
Juan F. Martínez-Lage
Publication date
01-08-2018
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 8/2018
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-018-3794-x

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