Congenital hemihypertrophy with lymphangioma.

Congenital h-mihypertrophy is a condition in which the whole of one side of the body is increased in size from birth. In a complete review of the subject Ward and Lerncr (1947) state that more than 100 cases have been decribed since the first descrip:ion was recorded by Wagner in 1839. The incidence of the condition is difficult to assess. Males, according to Gesell (1927), are more commonly affected than females. Hypertrophy has been described in a 10 mm. embryo, but its early recognition depends upon the observer's awareness of the existence of such an entity. In some cases malformation is not noticed until after puberty. Various theories on the aetiology have been put forward. The hormonal theories, which postulate an abnormal function of the ductless glands, are difficult to reconcile with the one-sided nature of the condition, and in all but one of the post-mortem reports the endocrine glands have been found to be normal. The single exception is that of Hutchison (1915-16), who described enlarged adrenal glands on the involved side. Rugel (1946) mentions that hemihypertrophy is in some way associated with 'an embryonic defect of the vegetative nervous system,' and that the hypertrophy is caused by an excess of the trophic function of the nervous system. Rugel's case revealed hypertrophy of both cerebral hemispheres, but Gesell (1921) describes three cases in which there was an enlargement of the cerebral hemisphere on the same side as the somatic enlargement. According to Sheldon (1946) generalized dilatation of the lymph spaces of the whole of one side of the body is a cause of hemihypertrophy, and the patchy, bluish appearance of the skin in some of the cases indicates a mixture of naevoid and lymphangiectatic states. Pressure upon the developing ovum or foetus, or lesions of the central nervous system, would be expected to produce hemiatrophy rather than hemihypertrophy. Congenital syphilis and neurofibromatosis have also been suggested as causes of hemihypertrophy, but definite evidence is lacking. Reed (1925) reports the occurrence of hemihypertrophy in a brother and sister, and Scott (1935) its appearance in a mother and daughter. No further evidence of heredity as an aetiological factor is to be found.