Prenatal sonographic appearance of diprosopus.

Congenital anomalies in the teratological collection of museum Vrolik in Amsterdam, the Netherlands. v: conjoined and acardiac twins

“…The relation to twinning of other midline defects that are frequently found in diprosopus with or without neural tube defects, such as (bi)lateral cleft lip with or without cleft palate [see Carles et al, 1995], diaphragmatic hernia [see Chervenak et al, 1985;Strauss et al, 1987], cardiac anomalies [see Changaris and McGavran, 1976;Sharony et al, 1993], and omphalocele [Amr and Hammouri, 1995], is less obvious. Carles et al [1995] postulated that at least some of these defects result from impaired migration of neural crest cells due to the (partially) duplicated neural tube and that diprosopus and agnathia-holoprosencephaly are two ends of the same neurocristopathy spectrum.…”

Prenatal Diagnosis of Monocephalic Bifacial Tetraophthalmic Diprosopus (Conjoined Twin)

“…Most of those gynecologists do not have high-quality ultrasounds and are not qualified to the highest standards of skills in ultrasound to perform screening in the first Table 1. Ultrasound and postmortem finding of a case of diprosopus tetraophthalmus Ultrasound finding -BPD 23 weeks, FL 19 weeks -Heart-shaped head, single occiput and two prominent frontal bones -Four hemispheres, two cerebella and two thalami -Three orbits, the middle one larger, two mouths, two noses Postmortem finding -Male fetus, length 155 mm, weight 450 g -Single head enlarged mainly in transverse diameter -Two oral cavities of normal shape connected in the medial corner with two completely separated tongues, two noses, two jaws and two chins, two ears -Four hemispheres, two thalami, and one brain stem with the fusion starting at mensencephalon; two hypoplastic cerebella, two frontal bones, duplication of nasal passages and two nasal cavities; doubled olfactory tracks, sellae turcici and hypo physes; two pairs of optic nerves with a funnel shaped single posterior fossa -Small poorly formed rudiment of a supernumerary digit next to the thumb of the right hand -Hypoplastic right lung with incomplete separation of left lung lobes -Heart pushed to the left without any abnormalities During an ultrasound examination abnormally shaped cranium and widened vertebral column should be considered as a suspicious ultrasound finding for anterior duplication of the faciocranial structures (as described by Strauss et al [10] ). The altered heart shape ( fig.…”

Diprosopus Associated with Neural Tube Defect and Facial Cleft in theFirst Trimester

“…Duplication of the brain is not always amenable to ultrasound in diprosopus since most cases are anencephalic [1-3, 5, 8-10]. Diprosopus can also be associated with spina bifida [7,8], hydrocephalus [6] and encephalocele [11], but often presents as a major craniorrhachischisis [1-3, 5, 9, 10]. Other commonly associated abnormalities include cleft lip and palate [1,12], diaphragmatic hernia [6][7][8] and cardiac defects [7,8].…”

“…Early diagnosis is difficult, although a few ultrasound features may help. In a case with a single intact skull, an abnormality of the cerebral hemispheres is usually the first element to be noted and has been associated with hydrocephalus [6], encephalocele [11], or a large posterior fossa [4,11,14].…”

“…Diprosopus can also be associated with spina bifida [7,8], hydrocephalus [6] and encephalocele [11], but often presents as a major craniorrhachischisis [1-3, 5, 9, 10]. Other commonly associated abnormalities include cleft lip and palate [1,12], diaphragmatic hernia [6][7][8] and cardiac defects [7,8]. A strong association between diprosopus and neural tube defects supports the hypothesis that conjoined twins and neural tube defects may share some pathological mechanism secondary to a failure of the rostral neuropore to close.…”