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Cloves and newborn:

CLOVES syndrome - A congenital overgrowth disorder with vascular anomalies

Dr Sampurna Roy MD                      June  2016

   


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CLOVES syndrome, a rare and challenging disorder was first described in 2007 by Sapp et al.

He described it as "progressive, complex, and mixed truncal vascular malformations, dysregulated adipose tissue, varying degrees of scoliosis, and enlarged bony structures without progressive bony overgrowth."

CLOVES syndrome is characterized by:

C L O= Congenital lipomatous overgrowth,

V =Vascular malformations,

E = Epidermal nevi, and

S =Skeletal abnormalities (Scoliosis)

A newborn with CLOVES syndrome typically present with lipomatous masses of the thoracic and abdominal wall with extension to the anterior abdominal wall, groin, retroperitoneum, mediastinum, and gluteal area.

Skin lesions include vascular malformation, epidermal nevus and multiple small nevi.

Musculoskeletal abnormalities may present as scoliosis, leg-length discrepancy, dislocated knees, wide hands and feet, furrowed soles, sandal-gap toe and macrodactyly.

Differential Diagnosis: Patients with CLOVE syndrome should not be mistaken as Proteus syndrome which has strikingly similar manifestations. Klippel-Trenaunay syndrome is a rare limb overgrowth disorder with slow-flow vascular anomalies.

 

Further reading:

Newly delineated syndrome of congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE syndrome) in seven patients.

Complex spinal-paraspinal fast-flow lesions in CLOVES syndrome: analysis of clinical and imaging findings in 6 patients.

Newly delineated syndrome of congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE syndrome) in seven patients.

A Neonate with CLOVES Syndrome.

Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome: CNS malformations and seizures may be a component of this disorder.

Congenital lipomatous overgrowth, vascular malformations, and epidermal nevi (CLOVE) syndrome: CNS malformations and seizures may be a component of this disorder.

 

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Dr  Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

 


 

 

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