some families to death in utero with severe kidney, heart, eye, skeletal, brain and intestinal malformations in others. The variable severity is usually due to the presence or absence in each family of mutations in other genes with similar functions to CENPF. Intestinal Individuals with Strømme syndrome are typically born with intestinal atresia, in which parts of the intestine are narrow or missing, leading to neonatal bowel obstruction that must be operated on. The intestinal atresia is of the "apple-peel" type, an uncommon type in which the remaining portion of the intestine is found twisted around its main artery, and this usually affects the jejunum. Often, much of the bowel is missing in this form of atresia. There can sometimes also be intestinal malrotation. At least two individuals with the syndrome in literature have avoided intestinal atresia, one of which had a sibling with the same mutations who did not. In two siblings who did not survive to term, the intestinal atresia (which also included duodenal atresia) and malrotation were more severe. Eyes The eyes are often smaller and underdeveloped, usually more severely in one eye than the other. This can manifest as a coloboma (hole) in the iris, cataracts, opacity of the cornea (leukoma), sclerocornea (in which the white of the eye blends into the cornea), a small cornea (microcornea) and synechia (in which the iris adheres to the cornea or lens). This underdevelopment of the front of the eye, known as anterior segment dysgenesis (which includes Peters' anomaly), can lead to an increased risk of glaucoma from high intraocular pressure, due to impaired eye fluid drainage, though this hadn't been reported in any of the affected individuals . There may also be crossing of the eyes (esotropia), and less commonly there may be twisted retinal blood vessels or optic nerve hypoplasia. The eye anomalies can result in an inability to focus (astigmatism) as well as amblyopia, in which the brain begins to fail to process input from the weaker eye during childhood. Neurological Those affected with the syndrome usually have microcephaly. A large minority also have pachygyria (fewer ridges in the brain) or lissencephaly (shallower ridges). Developmental delay is usually present. It has usually been moderate-to-severe, but in some cases it has been mild. A few of those affected have had increased muscle tone. One individual had cortical heterotopia, which is a sign of impaired neuronal cell migration