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Images in Clinical Practice

Indian Pediatrics 2001; 38: 677-678  

Proteus Syndrome

 

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:
rajivreema@hotmail.com

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