Diprosopus: A Rare Case of Craniofacial Duplication and a Systematic Review of the Literature.

In 1990, Gorlin et al. described four types of craniofacial duplications: (1) single mouth with duplication of the maxillary arch; (2) supernumerary mouth laterally placed with rudimentary segments; (3) single mouth with replication of the mandibular segments; and (4) true facial duplication, namely diprosopus. We describe a newborn born with wide-spaced eyes, a very broad nose, and two separate mouths. Workup revealed the absence of the corpus callosum and the presence of a brain midline lipoma, wide sutures, and a Chiari I malformation with cerebellar herniation. We conducted a systematic review of the literature and compared all the cases described as diprosopus. In 96% of these, the central nervous system is affected, with anencephaly being the most commonly associated abnormality. Other associated anomalies include cardiac malformations (86%), cleft palate (63%), diaphragmatic hernia (13%), and disorder of sex development (DSD) (13%). Although the facial features are those that first strike the eye, the almost obligate presence of cerebral malformations suggests a disruptive event in the cephalic pole of the forming embryo. No major monogenic contribution has been recognized today for this type of malformation.

Symmetrical parapagus diprosopus tetrophthalmos in a bovine calf, with computed tomography imaging, and review of craniofacial duplications.

Diprosopia, a congenital defect process also known as craniofacial duplication, is well-recognized in humans and has also been reported in numerous animal species. Here we describe a case of diprosopia in a live mixed-breed beef calf. We used computed tomography imaging to characterize internal and external abnormalities which, to our knowledge, have not been reported in any diprosopic veterinary species. Additional diagnostic tools included postmortem examination and histopathology. This case highlights distinct anatomic features associated with diprosopia and underscores the unique challenges associated with classifying and managing fetal malformations.

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

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.

Registro de un Monstruo Diprósopo de Tiburón Azul (Prionace glauca) en Chile / Record of a Diprosopus Monster of Blue Shark (Prionace glauca) from Chile

Un espécimen de siameses de tiburón azul, Prionace glauca, fue recolectado durante actividades de pesca en el norte de Chile. Según una clasificación basada en el grado de duplicación y el sitio de fusión de los gemelos, el espécimen corresponde a un monstruo del tipo diprósopo tetroftalmo. El individuo tiene una duplicación craneofacial parcial con presencia de cuatro globos oculares e igual número de narinas. Las cavidades orales están interconectadas convergiendo en una única faringe. El tronco es simple; la columna presenta lordosis torácica y una torsión helicoidal en su porción caudal. Este es el primer reporte de siameses en condrictios chilenos y primer registro mundial de diprosopa en tiburones.

A conjoined twins specimen of blue shark Prionace glauca was collected during fishing activities in Northern Chile. According to a classification based on duplication degree and fusion site of the twins, it represents a monster of diprosopus tetrophthalmus type. The specimen has a partial craniofacial duplication with presence of four eyeballs and an equal number of nostrils. Oral cavities are interconnected, converging in a single pharynx. The trunk is simple; the vertebral column presents thoracic lordosis and helical torsion in its caudal portion. This is the first Chilean record of conjoined twins to chondrichthyan and first world record of diprosopia in sharks.

Diprosopia em bovino / Bovine diprosopus

This work describes a malformation in one newborn female bovine, with two faces and two skull fused, showing one single head. Duplications of the nasal and oral structures, tetraofthalmy, two brains, one single cerebellum, and pons were observed. The right thyroid was hypertrophic and the other organs had normal morphology. Every change observed in this case was compatibles with diprosopus.