A term newborn was found to have a large
lympho-hemangioma on the left side involving the chest and abdomen (Fig.1).
Multiple small nevi were seen over the hemangioma. This was associated
with hyper-trophy on the left side. The baby’s left sole was bigger
than the right sole (Fig. 2). Width of left and right sole at
level of head of metatarsals was 4.5 cm and 4.2 cm, respectively. The
length of foot on the left and right was 9.3 cm and 8.2 cm,
respectively. Thickening, excessive wrinkling and furrow-ing of the
skin was also seen on the left sole. Congenital dislocation of hip was
present on the left side. Bilateral inguinal hernia was present.
Ultrasonography of the head, chest and abdomen were normal. Skull X-rays
and a skeletal survey did not reveal any abnormality. The initial
platelet count was 70,000/mm3
but subsequent counts were normal.
Proteus syndrome is a bizare clinical condition
characterized by overgrowth of various body parts, hemihypertrophy,
multiple cutaneous nevi, unusual skeletal malforma-tions, soft tissue
tumors and visceral anomalies. It is a congenital hamartomatous
disorder and hence is quite similar to other disorders of overgrowth.
However, the salient clinical features of massive overgrowth,
hamartomata, thickening of palms and soles and hemihypertrophy are
usually present to enable a confident diagnosis.
The lesions in Proteus syndrome tend to be
progressive and minor variations in size may become more apparent with
growth. Cosmetic and functional correction by surgery is generally
unrewarding since large scars and keloids are frequent. Delayed would
healing is an additional problem. For these reasons surgical
correction is generally avoided unless function is severely impaired
by tumor bulk. Careful follow up would be mandatory since almost all
congenital hamartomatous disorders display an increased tendency for
neoplasia.
Fig. 1. Infant with
a large lymphangioma with overlying hemangioma and black nevi on the
left side of the body.
Fig.2. Photograph showing
disparity in the size of the feet.
Amit Upadhyay,
Rajiv Aggarwal,
Division of Neonatology,
Department of Pediatrics,
All India Institute of Medical Sciences,
Ansari Nagar, New Delhi 110 029, India.
E-mail:
[email protected]
