Techniques for Toddlers: Linear Band Incision for Harlequin Ichthyosis with Associated Compartment Syndrome |
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| Authors: | Gramen Tontchev MD Nanette B Silverberg MD Edward Shlasko MD Carol Henry RDMS Jaclyn L Roberts MD Malcolm Z Roth MD |
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| Institution: | 1. Department of Surgery, Maimonides Medical Center, , Brooklyn, New York;2. Department of Dermatology, St. Luke's‐Roosevelt Hospital Center of the Icahn School of Medicine at Mount Sinai, , New York, New York;3. Department of Surgery, Icahn School of Medicine at Mt. Sinai, , New York, New York;4. Department of Obstetrics and Gynecology, Perinatal Testing Center, Maimonides Medical Center, , Brooklyn, New York;5. Division of Plastic Surgery, Albany Medical Center, , Albany, New York |
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| Abstract: | Harlequin ichthyosis (HI) is a rare autosomal recessive disorder of cornification in which children are born with an extremely thick stratum corneum that becomes a restrictive circumferential encasement around the orifices, limbs, chest, and abdomen, resulting in limb contractures. We present a neonate diagnosed in utero with HI. The infant was born with encasing bands of thickened skin creating strictures that were causing digital and limb cyanosis (compartment syndrome). We treated the child using a new technique of lysis of the encasing bands that we call linear band incision, using a new escharotomy‐like procedure while the infant was undergoing a 3‐week course oral acitretin therapy. The technique involved linear incision and lysis of encasements that resulted in reperfusion of the injured limbs and prevention of further digital necrosis. The child is currently a healthy 8‐year‐old boy with skin manifestations resembling congenital ichthyosiform erythroderma. He has use of all of the limbs that were released in the procedures and is maintained on frequent application of bland emollients. Linear band incision is a potentially life‐ and limb‐saving technique in children with HI. |
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