cordis with amniotic bands appears to be distinct from isolated EC.
George, "Hybrid SPECT/CT evaluation of dual ectopia
of thyroid in the absence of orthotopic thyroid gland," Clinical Nuclear Medicine, vol.
cordis has been known for 5 000 years, but its etiology and pathogenesis has not been elucidated yet (2, 6-8).
As shown in table 1, statistics for the ocular manifestation in that family are; lens ectopia
100% (12/12), flat cornea 30% (4/12), on gated eyeball 42% (5/12), hypo plastic iris or ciliary's muscle hypoplasia 50% (6/12), glaucoma and cataract 25% (3/12), retinal detachment 16%(2/12).
of testis- rare type where both the testes are in same hemiscrotum.
Different surgical techniques for the management of ectopia
lentis have been reported such as lensectomy and anterior chamber IOL implantation, open loop3,4 or scleral fixation of IOL implant(SF-IOL).5 However, the risks include iritis, pigment dispersion, corec-topia, glaucoma, corneal endothelial loss, and retinal detachment.
Posterior pituitary ectopia
: an MR feature of pituitary dwarfism.
B) in some cases, the contralateral testis and the tube are also in the hernia sac; transverse testicular ectopia
can also occur.
The incredibly rare defect, ectopia
cordis, affects only eight in every million births and ninety percent of these are stillborn or die within three days.
In regard to the aforementioned syndrome patients we have to note that they presented with a variety of the expected typical characteristics, more specifically MVP, arachnodactyly, muscular atrophy and ectopia
lentis for the Marfan patients, and MVP skin and joint hyperextensibility (8), ectopia
lentis and highgraded myopia for the Ehlers-Danlos patients.
Mullerian hypoplasia or aplasia - renal agenesis - cervicothoracic somite dysplasia known as the MURCS association (Mullerian duct aplasia; R: renal agenesis / ectopia
; CS: cervical somite dysplasia) represent the most rare and severe form of the syndrome.7 Ovarian abnormalities such as ectopic or polycystic ovaries have been described.