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Melnick–Needles syndrome (osteodysplasty) in an older male–report of a case and a review of the literature

H. van der Lely, MD ^* S. G. F. Robben, MD M. Meradji, MD, PhD and G. Derksen-Lubsen, MD, PhD

Department of Radiology, Erasmus University, Sophia Children's Hospital, Gordelweg 160, 3038 GE Rotterdam, The Netherlands Department of Pediatrics, Erasmus University, Sophia Children's Hospital, Gordelweg 160, 3038 GE Rotterdam, The Netherlands

Correspondence: ^* Author's current address for correspondence: Department of Radiology, Zuiderziekenhuis, Groene Hilledijk 315, 3075 EA Rotterdam, The Netherlands.

This excerpt was created in the absence of an abstract.

In 1966 Melnick and Needles reported on a previously undiagnosed bone dysplasia. The diagnosis was based on characteristic radiographic findings: a delay in closure of the anterior fontanel, i.e. sclerosis of the skull base and mastoids. The long tubular bones are bowed with cortical irregularities and metaphyseal flaring. The ribs also show cortical irregularities and have a ribbonlike or wavy appearance. The vertebral bodies show an increase in height with a decrease in antero-posterior measurement and anterior scalloping. A decrease in disk space is also present. The pelvis has a typical constricted appearance with flaring of the iliac crest and tapering of the ischial and pubic bones. The clinical signs include exophthalmos, full prominent cheeks, mild hypertelorism, micrognatia, large ears and misaligned teeth. Females are affected almost exclusively and inheritance is in an X-linked dominant pattern. The condition is lethal in males born from Melnick–Needles syndrome (MNS) mothers (Donnenfeld, 1987). Three male patients (born from normal parents) are reported to have reached the age of 3 years.

We present the case of an 11-year-old boy, the oldest living male patient documented so far.

Key Words: Melnick–Needles syndrome • Osteodysplasty • Child • Male

Received for publication November 1, 1990. Revision received February 1, 1991.