Fetal diprosopus (double face): A case report

Onankpa, BO; Ukwu, E; Singh, S. D.; Au, Adoke; Tahir, Akbar

doi:10.5958/j.2319-5886.3.2.095

Cited by 4 publications

(4 citation statements)

References 9 publications

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“…Mutation of dlx homeobox genes expressed by neural crest cells (Young et al , ; Depew et al , ; Heude et al , ) might lead to duplication of craniofacial elements (Costa et al , ). In addition, it has been suggested that an increased production of sonic hedgehog (SHH) protein may be involved in the formation of diprosopus individuals (Onankpa et al , ). It is known that disruption in SHH signalling during facial morphogenesis can perturb mediolateral patterning at the neural plate, resulting in holoprosencephaly and cyclopia (Hu & Helms, ).…”

Section: Discussionmentioning

confidence: 99%

Dicephalous v. diprosopus sharks: record of a two‐headed embryo of Galeus atlanticus and review of the literature

Sans‐Coma

Rodrı́guez

López‐Unzu

et al. 2016

Journal of Fish Biology

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As far as is known, this paper gives the first description of a two-headed shark embryo belonging to an oviparous species, Galeus atlanticus (Carcharhiniformes: Scyliorhinidae). The specimen was detected among 797 embryos intended for cardiovascular studies, which represents a defect incidence of 0·13%. Each head had a mouth, two eyes, a brain, a notochord and five gill openings on each side. The two heads fused behind the gills. On the single body, there were four anticipated dorsal fins, two anterior, right and left and two posterior, right and left. Ventrally, the specimen possessed two pairs of pectoral fins, a pair of pelvic fins and one anal fin. Two adjacent notochords, two neural tubes and two dorsal aortas ran along the body, which bent 180° at its posterior portion. There were two hearts, two oesophaguses, two stomachs, two livers, but a single intestine with a spiral valve. Previous reports of conjoined twins in sharks are scarce and only refer to oviparous and ovoviviparous species. Seven dicephalous sharks reported so far were similar to the specimen described here, namely, with two totally separated heads on one body. Instead, only one case of diprosopus shark has been reported; it had a single body and a single head with partial duplication of the face. Two further cases described in the literature as dicephalous or simply as abnormal sharks should be better regarded as diprosopus, while another three cases, also considered dicephalous, showed a mixture of characteristics of diprosopia and dicephalia.

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Section: Discussionmentioning

confidence: 99%

Dicephalous v. diprosopus sharks: record of a two‐headed embryo of Galeus atlanticus and review of the literature

Sans‐Coma

Rodrı́guez

López‐Unzu

et al. 2016

Journal of Fish Biology

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“…Three cases of severe facial midline defects in association with true diprosopus have been observed thus far-in addition to our case 2 resulting in 4 HPEs out of 48 accessible diprosopus cases. In one of two cases presenting with discordant cyclopia, autopsy had been declined [13]. In the second case in which the individual showed cyclopia on the left face and a normal mouth, nose and right eye, but anophthalmia with a depression at the site of a lacking left palpebral fissure on the right face, the brain had been described as having 'two hemispheres'-although two 'holospheres' would have been the more likely diagnosis as in cas 2 [31].…”

Section: Discussionmentioning

confidence: 99%

“…1f ). A total of 81 scientific reports on human facial duplications have been documented since 1642, 38 early ones cited by Barr [6], 33 more recent ones cited by Bidondo et al, [1] and 10 additional cases [7][8][9][10][11][12][13][14][15][16]. The prevalence rate of Diprosopus has been calculated to be 2 per 1,000,000 births in Argentinia, accounting for 10% of all conjoined twins [1].…”

Section: Introductionmentioning

confidence: 99%

Brain malformations in diprosopia observed in clinical cases, museum specimens and artistic representations

Rehder

Kircher

Schoner

et al. 2023

Orphanet J Rare Dis

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Background Diprosopus is a rare malformation of still unclear aetiology. It describes a laterally double faced monocephalic and single-trunk individual and has to be distinguished from the variant Janus type diprosopus. Results We examined seven double-faced foetuses, five showing true diprosopus, and one each presenting as monocephalic Janiceps and parasitic conjoined twins. Four of the foetuses presented with (cranio)rachischisis, and two had secondary hydrocephaly. Three foetuses showed cerebral duplication with concordant holoprosencephaly, Dandy-Walker cyst and/or intracranial anterior encephalocele. In the Janiceps twins, cerebral duplication was accompanied by cerebral di-symmetry. In the parasitic twins the cyclopic facial aspects were suggestive of concordant holoprosencephaly. In one of the true diprosopus cases, pregnancy was achieved after intracytoplasmic sperm injection. Whole-exome sequencing, perfomed in one case, did not reveal any possible causative variants.The comparison of our double-faced foetuses to corresponding artistic representations from the Tlatilco culture allowed retrospective assignment of hairstyles to brain malformations. Conclusion Brain malformations in patients with diprosopus may not be regarded as an independent event but rather as a sequel closely related to the duplication of the notochord and neural plate and as a consequence of the cerebral and associated craniospinal structural instabilities.

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“…Laterally united conjoined twins share vast parts of their body (abdomen, thorax, neck, face and/or head) and classically consists of two phenotypes: parapagus dicephalus (two heads) and parapagus diprosopus (two laterally oriented faces in a single compound head). One of the more striking and relatively often occurring discordances found in dicephalic twins is anencephaly, with at least seven described cases appearing in the recent literature [9,14,15,[25][26][27][28]56]. These unilaterally occurring neural tube defects should be distinguished from concordant neural tube defects in parapagus diprosopus, which are the consequence of interaction aplasia, a phenomenon comprehensively described elsewhere [3].…”

Section: Discordance In Laterally United Twinsmentioning

confidence: 99%

Phenotypically Discordant Anomalies in Conjoined Twins: Quirks of Nature Governed by Molecular Pathways?

Boer,

Winter,

Gorissen

et al. 2023

Diagnostics

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A multitude of additional anomalies can be observed in virtually all types of symmetrical conjoined twins. These concomitant defects can be divided into different dysmorphological patterns. Some of these patterns reveal their etiological origin through their topographical location. The so-called shared anomalies are traceable to embryological adjustments and directly linked to the conjoined-twinning mechanism itself, inherently located within the boundaries of the coalescence area. In contrast, discordant patterns are anomalies present in only one of the twin members, intrinsically distant from the area of union. These dysmorphological entities are much more difficult to place in a developmental perspective, as it is presumed that conjoined twins share identical intra-uterine environments and intra-embryonic molecular and genetic footprints. However, their existence testifies that certain developmental fields and their respective developmental pathways take different routes in members of conjoined twins. This observation remains a poorly understood phenomenon. This article describes 69 cases of external discordant patterns within different types of otherwise symmetrical mono-umbilical conjoined twins and places them in a developmental perspective and a molecular framework. Gaining insights into the phenotypes and underlying (biochemical) mechanisms could potentially pave the way and generate novel etiological visions in the formation of conjoined twins itself.

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Fetal diprosopus (double face): A case report

Cited by 4 publications

References 9 publications

Dicephalous v. diprosopus sharks: record of a two‐headed embryo of Galeus atlanticus and review of the literature

Dicephalous v. diprosopus sharks: record of a two‐headed embryo of Galeus atlanticus and review of the literature

Brain malformations in diprosopia observed in clinical cases, museum specimens and artistic representations

Phenotypically Discordant Anomalies in Conjoined Twins: Quirks of Nature Governed by Molecular Pathways?

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