title: Hemihyperplasia-multiple lipomatosis syndrome (HHML): a challenge in spinal care.
abstract: A 15-year-old girl developed a progressive paraparesis over a period of six months, secondary to spinal cord compression by a lipomatous mass and anomalies of the vertebral column. Clinically, a right hemihyperplasia affecting the trunk and lower limb was evident, as well as a right convex lumbar scoliosis. CT and MRI demonstrated severe spinal cord compression resulting from intraspinal lipomatosis, overgrowth of right facet joints (T8 to L5), and kyphoscoliosis. Surgical decompression was undertaken. A lumbar scoliosis of 48 degrees was partially corrected by means of dual-rod instrumentation. The neurological deficit improved significantly, and ambulation was progressively restored. The patient carried the diagnosis of Proteus syndrome for several years, but reevaluation of clinical features prompted the diagnosis of Hemihyperplasia Multiple Lipomatosis syndrome (HHML). This rare sporadic disorder is often confused with Proteus syndrome. As in Proteus syndrome, spinal cord compression in patients with HHML can result from lipomatous infiltration and/or significant spinal abnormalities including kyphoscoliosis and overgrowth. HHML and Proteus syndrome are discussed and compared with special emphasis on spinal and orthopaedic pathologies.
authors.affiliation: Department of Orthopaedics, University Hospital, Albert Schweitzer Strasse 33, 48149 Münster, Germany. dr.tobias.schulte@web.de
authors.forename: Tobias L
authors.initials: TL
authors.lastname: Schulte
authors.forename: Ulf
authors.initials: U
authors.lastname: Liljenqvist
authors.forename: Heike
authors.initials: H
authors.lastname: Görgens
authors.forename: Lars
authors.initials: L
authors.lastname: Hackenberg
authors.forename: Viola
authors.initials: V
authors.lastname: Bullmann
authors.forename: Sigrid
authors.initials: S
authors.lastname: Tinschert
mesh_terms: D000293:Adolescent; D005260:Female; D006801:Humans; D006965:Hyperplasia; D008068:Lipomatosis / Q000150:complications*; D020335:Paraparesis / Q000209:etiology; D013117:Spinal Cord Compression / Q000209:etiology*; D013122:Spinal Diseases / Q000150:complications*; D013577:Syndrome