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.
Subject(s)
Compartment Syndromes/etiology , Compartment Syndromes/surgery , Ichthyosis, Lamellar/complications , Ichthyosis, Lamellar/surgery , Compartment Syndromes/diagnosis , Consanguinity , Humans , Ichthyosis, Lamellar/diagnosis , Infant, Newborn , Leg , Male , Prenatal DiagnosisABSTRACT
In the United States accredited residency programs in addiction exist only for psychiatrists specializing in addiction psychiatry (ADP); nonpsychiatrists seeking training in addiction medicine (ADM) can train in nonaccredited "fellowships," or can receive training in some ADP programs, only to not be granted a certificate of completion of accredited training. Information about ADP residency programs has been tabulated, but it is not available for ADM fellowships. The authors conducted a national survey to compile information about the location, structure, curriculum, and other characteristics of active ADM fellowships. Of the 40 accredited ADP residency programs, 7 offered training in addiction to nonpsychiatrists. The authors identified 14 nonaccredited ADM fellowships. In 2009 and 2010, there were approximately 15 nonpsychiatrists in ADP programs and 25 in ADM fellowships. Clinical experiences included inpatient services, outpatient treatment services such as methadone maintenance or buprenorphine maintenance, and providing addiction consult services. The most common academic activities included weekly lectures and the teaching of medical students.