Hemihypertrophy

Classification according to ICD-10
Q87.3	Congenital malformation syndromes with increased tissue growth in early childhood
ICD-10 online (WHO version 2019)

A hemihypertrophy is a special form of a hypertrophy , in which a one-sided overgrowth of the body or consists of parts thereof.

Often there are additional abnormalities of the skin and teeth, as well as endocrine disorders.

causes

Hemihypertrophy can be congenital, present at birth or develop during childhood, or it can be acquired later in inflammatory proliferative processes such as B. osteomyelitis .

Hemihypertrophy can occur in the context of syndromes such as Klippel-Trenaunay syndrome , neurofibromatosis , Proteus syndrome or Silver-Russell syndrome .

Classification

According to Hoyme et al., The following classification is possible:

The form in question face, as Friedreich's Auerbach syndrome called
Simple form (affecting only one limb)
Complex shape (affecting several limbs and / or one half of the trunk)
Isolated form as a sign of gigantism - Syndromes such as Beckwith-Wiedemann syndrome , neurofibromatosis and Klippel-Trenaunay-Weber syndrome

One form that affects the brain is hemimegalencephaly .

clinic

If the face is involved, facial scoliosis can develop, if the trunk is also involved, scoliosis can develop, and if the leg is involved, a leg length difference can develop .

treatment

Treatment options can be found in the articles on the complications mentioned.

Individual evidence

↑ F. Hefti: Pediatric Orthopedics in Practice. Springer 1998, p. 647, ISBN 3-540-61480-X .
↑ HE Hoyme, LH Seaver, KL Jones, F. Procopio, W. Crooks, M. Feingold: Isolated hemihyperplasia (hemihypertrophy): report of a prospective multicenter study of the incidence of neoplasia and review. In: American journal of medical genetics. Vol. 79, No. 4, October 1998, ISSN 0148-7299 , pp. 274-278, PMID 9781907 (review).
^ Who named it Friedreich Auerbach
^ N. Friedreich: About congenital unilateral head hypertrophy. In: Virchow's archive for pathological anatomy and physiology and for clinical medicine , Berlin, 1863, vol. 28, pp. 474–481.
↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .

[1] F. Hefti: Pediatric Orthopedics in Practice. Springer 1998, p. 647, ISBN 3-540-61480-X .

[2] HE Hoyme, LH Seaver, KL Jones, F. Procopio, W. Crooks, M. Feingold: Isolated hemihyperplasia (hemihypertrophy): report of a prospective multicenter study of the incidence of neoplasia and review. In: American journal of medical genetics. Vol. 79, No. 4, October 1998, ISSN 0148-7299 , pp. 274-278, PMID 9781907 (review).

[3] Who named it Friedreich Auerbach

[4] N. Friedreich: About congenital unilateral head hypertrophy. In: Virchow's archive for pathological anatomy and physiology and for clinical medicine , Berlin, 1863, vol. 28, pp. 474–481.

[Leiber-5] Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .