Anterior midline defects: Association with ectopia cordis or vascular dysplasia defines two distinct entities

Superficial Hemangiomas: Associations and Management

“…As in our series, a number of other malformations, usually located close to the hemangioma, have been reported in various other systems, for example, sternal clefting, atresia or absence of the sternum (Fig. 2), supraumbilical abdominal raphe (26)(27)(28)(29), coarctation of the aortic arch and other vascular and cardiac defects (30,31), eye abnormalities (23), such as microphthalmos, congenital cataract, and optic nerve hypoplasia (Fig. 3), imperforate anus, bladder exstrophy, abnormal genitalia, renal anomalies, sacral tail, bony de- Figure 2.…”

“…formity of the sacrum, spinalipoma, occult spinal dysraphism and tethered cord, lipomyelomeningocele (10,32,33), and thyroid defect (25)(26)(27)(28)(29)(30)(31)(32)(33)(34). The presence of multiple anomalies in association with large cephalic hemangiomas is, according to Frieden et al (25), a new neurocutaneous syndrome; the PHACE(S) syndrome-Posterior fossa CNS malformations.…”

Supra- and infraumbilical raphe with pectus excavatum

“…Ventral midline defects represent distinct entities of different causes [Hersh et al, 1985;Kaplan et al, 1985]. However, the combination of ventral midline defects such as abdominal wall, sternum, and heart defects has been described before [Carmi and Bough-man, 1992].…”

“…Anterior abdominal midline defects may be seen with cleft sternum or other midline anomalies [Kaplan et al, 1985;Carmi and Boughman, 1992]. The upper abdominal segment is commonly involved in the form of a supraumbilical raphe.…”

“…According to Leiber [1982], Kaplan et al [1985], and Opitz [1985], ''the supraumbilical raphe represents a developmental field defect involving upper abdominal midline mesenchymatous structures.'' Abdominal wall defects result from a malformation involving midline mesenchymatosus tissue.…”