PubMed · 5715656
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P H Schurr. 1968. Craniosynostosis.. https://doi.org/10.1111/j.1469-8749.1968.tb02980.x
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Hennekam syndrome is a rare autosomal recessive syndrome which was described for the first time in 1989. Here, we present a girl with intestinal lymphangiectasia, severe lymphedema of limbs, seizures, mild mental retardation, and facial anomalies consistent with the diagnosis of Hennekam syndrome. In addition, she had an ectopic kidney and craniosynostosis of the coronal suture, 2 manifestations not previously reported in this syndrome. While the molecular basis of Hennekam syndrome remains, as yet, unknown, this report illustrates its variable clinical expression.
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Growing skull fractures or posttraumatic leptomeningeal cysts are a known complication of skull fractures sustained in infancy and childhood. They usually present as progressively enlarging cranial masses at the site of the linear skull fractures. The pathogenesis is believed to require an underlying dural tear that allows herniation of the growing pulsatile brain and leads to resorption of the overlying bone. Similar enlarging cranial masses have been reported following craniofacial surgery. Our case occurred following reconstruction of a stenosed metopic suture. The treatment of this unusual complication of craniofacial surgery requires sound dural repair. An understanding of the similarity between this complication and the more common growing skull fractures of childhood is essential to a sound approach to diagnosis and treatment.