Prenatal Diagnosis and Postnatal Findings of Cephalothoracopagus Janiceps Disymmetros: A Case Report

Abstract

Conjoined twins are rare variants of monozygotic twins, which result from an incomplete division of the embryonic disk. Cephalothoracopagus is a rare twin pregnancy described as imperfect fusion of the head and chest, but separated columns, limbs, and pelvis. They occur with incidence rates that range from 1 per 50,000 to 1 per 100,000 births; however, the incidence of the cephalothoracopagus variety is 1 per 58 conjoined twins. In the case of identical and symmetric faces caused by the orientations of the 2 notochordal axes that are perfectly ventroventral, they are called janiceps disymmetros. We present a prenatal diagnosis of a typical case of cephalothoracopagus janiceps disymmetros and the diagnostic confirmation by image and pathology exams.

Fetal intracranial immature teratoma: presentation of a case and a systematic review of the literature

“…It is the second most frequent location after the sacrococcygeal tumors (4). At intracranial level, mainly supratentorial, they originate in the hyophysis, the suprasellar region, third ventricle, and brain hemispheres, being impossible in the majority of cases to determine the exact tumor origin (as in the case presented here) (3,10,14,15). From their development in the mid-line, they often extend from the intracranial area to the third ventricle, causing a hydrocephalus typical of these (also present in our case).…”

“…Thus, a normal ultrasound at the beginning of gestation does not rule out the possibility of it presenting later (9). A high suspicion is going to be established with the presence of an echogenic mass in the ultrasound in the 2nd or 3rd trimester that leads to an increase in the biparietal diameter and / or a head circumference accompanied by frecuent of calcifications, cystic foci, changes in the normal brain structure, or other ultrasound signs (2,8,10 (4,13). They are the most frequent histological type, representing between 26 to 50% of fetal brain tumors (9).…”

“…Prior to establishing the definitive diagnosis, we should rule out possible hemorrhage, vascular malformation, or a rare entity, but we must not forget the so-called 'fetus in J U S T A C C E P T E D fetus', and that it consists of the presence of a mass with fetal characteristics within a normal fetus, and being able to show cardiac activity in its interior (4,10,15).…”

A rare case of cephalothoracopagus janiceps conjoined twins

“…11,12 Overall survival is reported to be 25%. 12 However, severe cases such as TCP are incompatible with survival since the surgical separation is unfeasible 13 . This case had a serious union with conjoined brains, complex vasculature and nested airways that make surgical separation impossible.…”

Enfoque das gestações múltiplas de diagnóstico difícil durante a autopsia perinatal

“…Como todos los eventos asociados con monocigosidad, ocurren de forma esporádica y no tienen tendencia a recurrir (22,23). Los gemelos siameses resultan de una incompleta división del disco embrionario, la cual después del día 13 es usualmente incompletay resulta en la fusión de los gemelos (14,24); además de los puntos de fusión hay diversos grados de duplicación. El sitio y la extensión de la fusión de los gemelos son variables.…”