Screening Procedure for Hemihypertrophy: Preliminary Results of International Multicenter Prospective Study
Main Article Content
Abstract
Introduction: Isolated or congenital hemihypertrophy is a rare disorder characterized by asymmetric overgrowth of one side of the body. This article describes the protocol and preliminary results of a lateral body asymmetry (hemihypertrophy) screening procedure performed in healthy adolescents in a multicenter study. The reported incidence of hemihypertrophy varies between different publications and standardized protocols are needed to improve research in this area.
Methods: Our screening program is taking place in Australia, Israel, Mexico, Ukraine and USA. Procedure includes two steps: (1) “three measurements – three questions” screening, or assessment of face, palms, and shins; (2) in-depth assessment of selected cases in order to exclude localized, lesional, and syndrome-related cases as well as body asymmetry within normative range and to select suspected cases of isolated hemihypertrophy. This step includes measurements of various anatomical regions and a detailed questionnaire.
Results: At this stage, the screening procedure is completed and the selected participants are advised to refer to medical institutions for further clinical and genetic follow up to exclude possible tumors and other accompanying disorders.
Conclusion: We present an easy-to-use selection tool to identify children with suspected IH, which results in the selection of the risk group that may benefit from referral to a pediatrician and a clinical geneticist.
Article Details
Authors who publish with this journal agree to the following terms:
- The Author retains copyright in the Work, where the term “Work” shall include all digital objects that may result in subsequent electronic publication or distribution.
- Upon acceptance of the Work, the author shall grant to the Publisher the right of first publication of the Work.
- The Author shall grant to the Publisher and its agents the nonexclusive perpetual right and license to publish, archive, and make accessible the Work in whole or in part in all forms of media now or hereafter known under a Creative Commons Attribution 4.0 International License or its equivalent, which, for the avoidance of doubt, allows others to copy, distribute, and transmit the Work under the following conditions:
- Attribution—other users must attribute the Work in the manner specified by the author as indicated on the journal Web site;
- The Author is able to enter into separate, additional contractual arrangements for the nonexclusive distribution of the journal's published version of the Work (e.g., post it to an institutional repository or publish it in a book), as long as there is provided in the document an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post online a prepublication manuscript (but not the Publisher’s final formatted PDF version of the Work) in institutional repositories or on their Websites prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work. Any such posting made before acceptance and publication of the Work shall be updated upon publication to include a reference to the Publisher-assigned DOI (Digital Object Identifier) and a link to the online abstract for the final published Work in the Journal.
- Upon Publisher’s request, the Author agrees to furnish promptly to Publisher, at the Author’s own expense, written evidence of the permissions, licenses, and consents for use of third-party material included within the Work, except as determined by Publisher to be covered by the principles of Fair Use.
- The Author represents and warrants that:
- the Work is the Author’s original work;
- the Author has not transferred, and will not transfer, exclusive rights in the Work to any third party;
- the Work is not pending review or under consideration by another publisher;
- the Work has not previously been published;
- the Work contains no misrepresentation or infringement of the Work or property of other authors or third parties; and
- the Work contains no libel, invasion of privacy, or other unlawful matter.
- The Author agrees to indemnify and hold Publisher harmless from Author’s breach of the representations and warranties contained in Paragraph 6 above, as well as any claim or proceeding relating to Publisher’s use and publication of any content contained in the Work, including third-party content.
Revised 7/16/2018. Revision Description: Removed outdated link.
References
Hoyme HE, Seaver LH, Jones KL, Procopio F, Crooks W, Feingold M. Isolated hemihyperplasia (hemihypertrophy): report of a prospective multicenter study of the incidence of neoplasia and review. Am J Med Genet. 1998;79:274-8.
Urban PP, Bruening R. Congenital isolated hemifacial hyperplasia. J Neurol. 2009;256(9):1566-9. doi: 10.1007/s00415-009-5148-9.
van der Velpen I, Schendelaar P, van Pinxteren-Nagler E, Mouës-Vink CM. A 15-year-old Girl with an Asymmetric Hemitruncal Fat Distribution: Hemihypertrophy or Hemiatrophy? Plast Reconstr Surg Glob Open. 2016;4(4):e684. doi: 10.1097/GOX.0000000000000653. eCollection 2016 Apr.
Nyati A, Kalwaniya S, Agarwal P. Idiopathic hemihypertrophy with multiple fibroadenoma. Indian Dermatol Online J. 2016;7(4):316-7. doi: 10.4103/2229-5178.185481
Mark C, Hart C, McCarthy A, Thompson A. Fifteen-minute consultation: assessment, surveillance and management of hemihypertrophy. Arch Dis Child Educ Pract Ed. 2017 Aug 26. pii: edpract-2017-312645. doi: 10.1136/archdischild-2017-312645
Vaiman M, Shilco P, Roitblat Y, Nehuliaieva L, Rosenberg S, Leit A, Cleminson R, Shterenshis M. Hemihyperplasia/hemihypertrophy in adolescents: prospective international study. Int J Adolesc Med Health, 2018, in print.
Schook CC, Mulliken JB, Fishman SJ, Alomari AI, Grant FD, Greene AK. Differential diagnosis of lower extremity enlargement in pediatric patients referred with a diagnosis of lymphedema. Plast Reconstr Surg. 2011;127(4):1571-81. doi: 10.1097/PRS.0b013e31820a64f3.
Orbak Z, Orbak R, Kara C, Kavrut F. Differences in dental and bone maturation in regions with or without hemihypertrophy in two patients with Russell-Silver syndrome. J Pediatr Endocrinol Metab. 2005;18(7):701-10.
Jia HC, Sun XH. Dental arch characteristics of the facial asymmetry. Hua Xi Kou Qiang Yi Xue Za Zhi. 2005;23(5):412-4, 437.
Bjorklund SI. Hemihypertrophy and Wilms's tumour. Acta Paediatr. 1955;44(3):287-92.
Dumoucel S, Gauthier-Villars M, Stoppa-Lyonnet D, et al. Malformations, genetic abnormalities, and Wilms tumor. Pediatr Blood Cancer. 2014;61(1):140-4. doi: 10.1002/pbc.24709.
Arrigo S, Beckwith JB, Sharples K, D'Angio G, Haase G. Better survival after combined modality care for adults with Wilms' tumor. A report from the National Wilms' Tumor Study. Cancer 1990;66:827-30.
Breslow N, Beckwith JB, Ciol M, Sharples K. Age distribution of Wilms' tumor: Report from the National Wilms' Tumor Study. Cancer Res. 1988;48:1653-57.