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Skeletal involvement in children with Langerhans cell histiocytosis: healing, complications, and functional outcome

Introduction: Skeletal involvement in children with Langerhans cell histiocytosis (LCH) is a common feature of the disease. Several options for the treatment of these skeletal lesions have been reported. We describe our experience in the treatment of skeletal involvement of LCH in this retrospective...

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Autores principales: Abdelaal, Ahmed H. K., Sedky, Mohamed, Gohar, Seham, Zaki, Iman, Salama, Asmaa, Hassanain, Omayma, El Ghoneimy, Ahmed M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: EDP Sciences 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7364762/
https://www.ncbi.nlm.nih.gov/pubmed/32672151
http://dx.doi.org/10.1051/sicotj/2020024
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author Abdelaal, Ahmed H. K.
Sedky, Mohamed
Gohar, Seham
Zaki, Iman
Salama, Asmaa
Hassanain, Omayma
El Ghoneimy, Ahmed M.
author_facet Abdelaal, Ahmed H. K.
Sedky, Mohamed
Gohar, Seham
Zaki, Iman
Salama, Asmaa
Hassanain, Omayma
El Ghoneimy, Ahmed M.
author_sort Abdelaal, Ahmed H. K.
collection PubMed
description Introduction: Skeletal involvement in children with Langerhans cell histiocytosis (LCH) is a common feature of the disease. Several options for the treatment of these skeletal lesions have been reported. We describe our experience in the treatment of skeletal involvement of LCH in this retrospective case series study, entailing anatomic distribution, pattern of healing, skeletal deformities, and functional outcome of skeletal LCH. Methods: A retrospective analysis was conducted for patients diagnosed with LCH and having skeletal lesions in the period between 2007 and 2015. Out of a total of 229 cases, 191 (83.4%) had skeletal involvement. Bone healing was divided into partial and complete based on the size of lesion and cortical changes in plain radiograph. Skeletal deformities were serially measured. Time to pain control, resumption of weight bearing, and the final functional status of the patient were reviewed. Results: The mean age at presentation was 4.4 years (3 m–14.8 y) and the mean follow-up period was 53.3 months (0.2–120.7). After screening of skeletal and extra-skeletal lesions, 59 patients (31%) had M-S (Multisystem) LCH and 132 (69%) had S-S (Single system) LCH. Unifocal bone lesions were found in 81 (42.5%) patients, and multifocal bone lesions in 110 patients (57.5%). Single or multiple bone lesions were found in the craniofacial bones in 152 patients (79.5%), femur in 19 patients, (10%), ribs in 18 patients (9.4%), spine in 15 patients (8.1%), pelvis in 14 patients (7.3%), scapula in 8 patients (4.1%), humerus in 6 (3.1%), clavicle in 6 patients (3.1%), tibia in 3 patients (1.5%), radius in 3 patients (1.5%), and the ulna in 2 patients (1%) patients. No lesions were found in the fibula, hand, or foot. Out of all skeletal lesions, 179 (93.7%) patients were treated either medically or conservatively and 12 patients (6.2%) were treated surgically. The mean time to complete healing was 5.2 months (2–12). Skeletal complications included: pathologic fractures (9 vertebra plana, 5 long bone, 1 iliac bone), deformities (9 thoracolumbar kyphosis, 2 cervical spine subluxations, 2 coxa vara deformity of the proximal femur and one flattening of iliac bone). Conclusion: Non-operative treatment can lead to adequate bone healing in few months period. Partial or complete remodeling of bone deformities can be observed without surgical correction. However, surgical intervention might be indicated when cervical spine affection may lead to instability and subsequent neurological affection. Functional impairment is rarely caused by skeletal lesions in LCH.
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spelling pubmed-73647622020-07-17 Skeletal involvement in children with Langerhans cell histiocytosis: healing, complications, and functional outcome Abdelaal, Ahmed H. K. Sedky, Mohamed Gohar, Seham Zaki, Iman Salama, Asmaa Hassanain, Omayma El Ghoneimy, Ahmed M. SICOT J Original Article Introduction: Skeletal involvement in children with Langerhans cell histiocytosis (LCH) is a common feature of the disease. Several options for the treatment of these skeletal lesions have been reported. We describe our experience in the treatment of skeletal involvement of LCH in this retrospective case series study, entailing anatomic distribution, pattern of healing, skeletal deformities, and functional outcome of skeletal LCH. Methods: A retrospective analysis was conducted for patients diagnosed with LCH and having skeletal lesions in the period between 2007 and 2015. Out of a total of 229 cases, 191 (83.4%) had skeletal involvement. Bone healing was divided into partial and complete based on the size of lesion and cortical changes in plain radiograph. Skeletal deformities were serially measured. Time to pain control, resumption of weight bearing, and the final functional status of the patient were reviewed. Results: The mean age at presentation was 4.4 years (3 m–14.8 y) and the mean follow-up period was 53.3 months (0.2–120.7). After screening of skeletal and extra-skeletal lesions, 59 patients (31%) had M-S (Multisystem) LCH and 132 (69%) had S-S (Single system) LCH. Unifocal bone lesions were found in 81 (42.5%) patients, and multifocal bone lesions in 110 patients (57.5%). Single or multiple bone lesions were found in the craniofacial bones in 152 patients (79.5%), femur in 19 patients, (10%), ribs in 18 patients (9.4%), spine in 15 patients (8.1%), pelvis in 14 patients (7.3%), scapula in 8 patients (4.1%), humerus in 6 (3.1%), clavicle in 6 patients (3.1%), tibia in 3 patients (1.5%), radius in 3 patients (1.5%), and the ulna in 2 patients (1%) patients. No lesions were found in the fibula, hand, or foot. Out of all skeletal lesions, 179 (93.7%) patients were treated either medically or conservatively and 12 patients (6.2%) were treated surgically. The mean time to complete healing was 5.2 months (2–12). Skeletal complications included: pathologic fractures (9 vertebra plana, 5 long bone, 1 iliac bone), deformities (9 thoracolumbar kyphosis, 2 cervical spine subluxations, 2 coxa vara deformity of the proximal femur and one flattening of iliac bone). Conclusion: Non-operative treatment can lead to adequate bone healing in few months period. Partial or complete remodeling of bone deformities can be observed without surgical correction. However, surgical intervention might be indicated when cervical spine affection may lead to instability and subsequent neurological affection. Functional impairment is rarely caused by skeletal lesions in LCH. EDP Sciences 2020-07-16 /pmc/articles/PMC7364762/ /pubmed/32672151 http://dx.doi.org/10.1051/sicotj/2020024 Text en © The Authors, published by EDP Sciences, 2020 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Original Article
Abdelaal, Ahmed H. K.
Sedky, Mohamed
Gohar, Seham
Zaki, Iman
Salama, Asmaa
Hassanain, Omayma
El Ghoneimy, Ahmed M.
Skeletal involvement in children with Langerhans cell histiocytosis: healing, complications, and functional outcome
title Skeletal involvement in children with Langerhans cell histiocytosis: healing, complications, and functional outcome
title_full Skeletal involvement in children with Langerhans cell histiocytosis: healing, complications, and functional outcome
title_fullStr Skeletal involvement in children with Langerhans cell histiocytosis: healing, complications, and functional outcome
title_full_unstemmed Skeletal involvement in children with Langerhans cell histiocytosis: healing, complications, and functional outcome
title_short Skeletal involvement in children with Langerhans cell histiocytosis: healing, complications, and functional outcome
title_sort skeletal involvement in children with langerhans cell histiocytosis: healing, complications, and functional outcome
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7364762/
https://www.ncbi.nlm.nih.gov/pubmed/32672151
http://dx.doi.org/10.1051/sicotj/2020024
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