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.

Cephalothoracoomphalopagus - a rare type of conjoined twins from the pathologists perspective.

Objective: Case description and autopsy fi nding in conjoined twins dia gnosed in the 24th week of pregnancy. Results: We reporta case of a 31-year-old primigravida who was referred to the Department of Gynecology and Obstetrics at our hospital with a presumptive dia gnosis of conjoined twins. The ultrasound examination with subsequent three-dimensional (3D) image reconstruction demonstrated twin gestation complicated by cephalothoracoomphalopagus. Observations demonstrated that the twins were joined over an area that extended from the head to the thoraces down to the central abdomen. In view of multiple congenital malformations incompatible with postnatal life, the pregnancy was terminated. The twins then underwent an autopsy at the Department of Pathology and the autopsy confi rmed previous diagnosis. Conclusion: Cephalothoracoomphalopagus is one of the rarest forms of conjoined twins with unknown incidence due to a very small number of documented cases.

[Thoracopagus conjoined twins: case study conducted in Libreville and literature review]. / Jumeaux conjoints thoracopages: à propos d'un cas à Libreville et revue de la littérature.

Conjoined twins are a rare form of monozygotic and monoamniotic twins. Fetal prognosis is very poor and, if the diagnosis is made early, it most often requires therapeutic abortion. We here report a case of three-hour old thoracopagus conjoined twins who were born vaginally, at term, at 40 weeks + 3 days. They were born to non-consanguineous parents, without any significant pathological history. Their mother was young and primiparous. She received 5 antenatal visits and 3 obstetric ultrasounds were performed by an inexperienced physician. The diagnosis of thoracopages twins was suspected based on clinical examination and computerized tomography (CT) results. This test showed cardiovascular abnormalities incompatible with life. The twins died after 23 hours of age due to acute respiratory distress. Conjoined twins are a rare congenital abnormality. Early diagnosis based on ultrasound would help to determine common anatomical structures, investigate associated congenital abnormalities, schedule delivery in a suitable structure and multidisciplinary neonatal management.

Fetus in fetu: use of intraoperative ultrasound for safe excision of a rare entity.

A prenatally diagnosed abdominal mass at 36 weeks and 0 days was further characterised by postnatal ultrasound and MRI to be likely a rare case of fetus in fetu in an otherwise healthy male. Due to close proximity to both the coeliac axis and superior mesenteric artery (SMA), surgical excision was delayed for several months. Interim CT with intravenous contrast performed at 2 months of age demonstrated the SMA travelling through the posterior aspect of the mass. Surgery proceeded at 2 months of age. Intraoperative ultrasound was used to definitively identify both the coeliac axis and SMA in order to facilitate a safe excision. The patient recovered well with an uneventful discharge to home on postoperative day 8. Pathology confirmed the diagnosis of fetus in fetu.

Conjoined twins after single blastocyst transfer: a case report including detailed time-lapse recording of the earliest embryogenesis, from zygote to expanded blastocyst.

Conjoined twins are estimated to occur in 1:50 000 pregnancies. Eighteen cases of pregnancies achieved by ART have been published of which three were achieved after single embryo transfer, allowing discussion of embryo characteristics. We report, to the best of our knowledge, the first case of parapagus conjoined twins after ART. Furthermore, this is the first report of conjoined twins with detailed morphokinetics of the earliest embryogenesis from zygote to expanded and hatched blastocyst stage. The case zygote had three refractile bodies, which were all allocated to one blastomere at first cleavage following an asynchronous pronuclei fading. Within 2 h, this blastomere cleaved to four and fragmented. The remaining blastomere cleaved symmetrically and regularly and a blastocyst (score: 4AB) was vitrified 120 h after IVF. Pregnancy was achieved following a frozen-thawed single blastocyst transfer. The etiopathogenetic mechanism of the origin of conjoined twins is unknown and several hypotheses exist. The morphokinetics in the present case and morphology of other reported cases will be discussed in this context.

Thoracopagus: Autoptic and radiologic study of 2 cases.

Conjoined twins are a very rare complication of monozygotic twinning, most common being thoracopagus. Here we report about two cases of thoracopagus male twins illustrating the autopsy details of one case and the prenatal magnetic resonance imaging (MRI) details of another case. While the first case was misdiagnosed as separate twins on antenatal USG, only to be later confirmed as thoracopagus twins after birth, the antenatal MRI done in the second case helped in accurate detection of thoracopagus twins. Bilateral peripheral cortical cysts with dysplasia was noted in one of the twins of the first case, which has not been reported earlier in conjoint thoracopagii. Early prenatal diagnosis of conjoined twins is essential for better counselling of parents regarding post natal surgical management or termination of pregnancy. Importance of prenatal MRI for accurate detection of these cases is thereby highlighted.

Early unexpected diagnosis of fetal life-limiting malformation; antenatal palliative care and parental decision.

INTRODUCTION: Conjoined twins are an infrequent occurrence in obstetric practice. Live-conjoined twins on a late preterm triplet pregnancy is an even rarer event. OBJECTIVE: The objective of this study is to emphasize the critical importance of perinatal palliative care and non-directive parental counseling, informed decision making and respect for autonomy following full disclosure of findings, fetal life-limiting diagnosis, treatment alternatives, maternal-fetal potential complications, and most likely perinatal outcomes. METHODS: Early surprise prenatal diagnosis, comprehensive parental counseling, palliative care, and perinatal care of a set of conjoined twins and a singleton. RESULTS: Cesarean delivery of a set of conjoined twins and a singleton at 34 weeks' gestation. Immediate neonatal death of the conjoined twins, intact survival, and discharge of the singleton. Review of the database on previously reported similar cases. It is very important to utilize simple and direct language for parents to understand the grave prognosis to the pregnancy. Care alternatives in view of the maternal physical risks and psychological impact of carrying a high order abnormal multiple pregnancy, along with the possible side effects on the singleton.

Prenatal and postnatal radiologic evaluation of conjoined twins.

Imaging of conjoined twins requires the use of multiple modalities, in both the prenatal and postnatal setting. Prenatal imaging, including both ultrasound and magnetic resonance imaging, benefits from an organized, worksheet-based approach, careful labelling of all images, and adoption of supplemental techniques. Postnatal imaging should be informed by the prenatal imaging findings and should be thoughtfully planned prior to performance. Complete evaluation prior to planned surgical separation will require the use of complementary modalities and multidisciplinary review and discussion.

Parasitic rachipagus conjoined twin: case report.

A parasitic twin represents a rare developmental anomaly in which an asymmetrical, nonviable conjoined twin is attached to the host body at the time of birth. Rachipagus is among the rarest of conjoined twin subtypes and typically features a parasitic twin mass attached at the spine. Herein, the authors review the literature and describe the case of a 9-month-old girl presenting with a rachipagus parasitic twin consisting of a fully developed set of lower extremities originating from the midline upper back. After a complete workup to delineate parasite and autosite anatomy, the parasitic twin mass was successfully excised by a multidisciplinary team of surgeons, and the resulting defect was closed in a single stage.

Separation of pygopagus, omphalopagus, and ischiopagus with the aid of three-dimensional models.

BACKGROUND: The three-dimensional (3D) technique provides with accurate anatomical information. We present the separation surgeries for three different kinds of conjoined twins with the aid of three-dimensional techniques. METHOD: For the pygopagus twins, a pelvic and lower vertebral model was made. For the omphalopagus and ischiopagus, their enhanced computed tomography (CT) scan images were transferred to the Computer-Assisted Surgery Planning System (CASP) (Hisense, Qingdao, China) to generate the 3D models. RESULT: In the case of the pygopagus twins, the 3D model clearly showed that their coccyges were joined at a 120°angle from each other horizontally which suggested that the blind-end orifice on their back was a pilonidal sinus, which separated the normal sphincter into two halves. In the omphalopagus, the 3D model revealed one of the branches of each twin's hepatic vein was connected with the other's. The 3D model of the ischiopagus twins revealed that both of the twins had duplicated bladders and each baby's duplicated bladders united with one of the bladders of the other baby and a single rectum passing through the middle of the four bladders. CONCLUSION: 3D techniques could provide more detailed anatomical information, which is helpful in planning procedures for such complicated separation surgery. LEVELS OF EVIDENCE: Level IV.

Canine conjoined twinning: A pathoanatomical study of a Lhasa Apso symmetrical cephalothoracopagus.

A female pair of conjoined twins of the Lhasa Apso canine breed was subjected to tomographic and anatomical examinations. The twins had only one head and neck. The two ribcages were joined, extending to the umbilicus, with duplicated structures thereafter. They had three thoracic limbs and two pelvic girdles with four limbs, as well as a number of abnormalities in their internal organs. The data obtained were compared with the rare canine cases reported so far and with human cases.

Presentación de un caso de siameses cefalópagos / A case report of cephalopagus siamese

Se denominan siameses a aquellos gemelos que comparten estructuras anatómicas en grado variable. La incidencia varía entre 1:50 000 a 1:100 000 embarazos, 1:4 de los casos fallecen in utero y 40-60 por ciento mueren poco después del parto.Los hallazgos en autopsias médico-científicas de gemelos unidos cefalópagos, que son aquellos que presentan fusión cefálica con dos hemicaras, son el cuarto tipo de siameses en ocurrencia y su incidencia está estimada en 1 por cada 3'000.000 de embarazos o 1/58 siameses. El objetivo es presentar los hallazgos de una autopsia médico-científica de gemelos unidos cefalópagos. Se trata de gemelos cefalópagos, con terminación de embarazo por incompatibilidad con la vida extrauterina. Presentan cordón umbilical único de cinco vasos, tenían cráneos fusionados a nivel frontofacial con dos caras en lados opuestos de la cabeza (Janiceps), con fusión ventro-ventral de los ejes somáticos hasta debajo del ombligo sobre la pelvis, con sistemas respiratorios, cardiaco y genitourinario independientes, el sistema digestivo superior esta fusionado hasta el intestino medio, con duplicación a partir de la tercera porción del duodeno. Los gemelos siameses cefalópagos no son los más infrecuentes, pero es difícil encontrar series de casos y revisiones sistemáticas de sus características y patogénesis en Latinoamérica. Son escasas las estadísticas sobre siameses y no se ha descrito previamente ningún caso como éste. Se hace necesaria la realización de estudios de autopsias para mejorar la descripción y caracterización de estos casos y poder determinar si existen otras condiciones asociadas además de las teorías de unión embriológica(AU)

It is denominated siamese to those twins that share anatomical structures to a variable degree. The incidence varies between 1/50.000 and 1/100.000 pregnancies, 1:4 of the cases die in the uterus and 40-60 percent die shortly after birth. Findings in a medical-scientific autopsies of conjoined cephalopagus twins, those that presenting cephalic fusion with two hemi faces, are the fourth type of Siamese in occurrence and their incidence is estimated in 1 every 3´000.000 pregnancies or 1/58 siamese. It is about cephalopagus twins, with termination of pregnancy due to incompatibility with extrauterine life. They presented a five vessels common umbilical cord, fused craniums at frontofacial level with two faces in opposed sides of the head (Janiceps), ventro-ventral fusion of the somatic axis until below the umbilicus and above the pelvis, independent respiratory, cardiac and genitourinary systems. The superior digestive system was fused until the medium intestine, with duplication parting from the third portion of the duodenum. Conjoined cephalopagus twins are not the most frequent but it is hard to find a series of cases and systemic revisions of their characteristics and pathogenesis in Latin America. Statistics on Siamese twins are scarce and it has not been previously described any case like this one. Autopsy studies becomes necessary to improve the description and characterization of these cases and to determine the existence of any other associated conditions different to those of the embryologic union theory(AU)

Presentación de un caso de siameses cefalópagos / A case report of cephalopagus siamese

Se denominan siameses a aquellos gemelos que comparten estructuras anatómicas en grado variable. La incidencia varía entre 1:50 000 a 1:100 000 embarazos, 1:4 de los casos fallecen in utero y 40-60 por ciento mueren poco después del parto.Los hallazgos en autopsias médico-científicas de gemelos unidos cefalópagos, que son aquellos que presentan fusión cefálica con dos hemicaras, son el cuarto tipo de siameses en ocurrencia y su incidencia está estimada en 1 por cada 3'000.000 de embarazos o 1/58 siameses. El objetivo es presentar los hallazgos de una autopsia médico-científica de gemelos unidos cefalópagos. Se trata de gemelos cefalópagos, con terminación de embarazo por incompatibilidad con la vida extrauterina. Presentan cordón umbilical único de cinco vasos, tenían cráneos fusionados a nivel frontofacial con dos caras en lados opuestos de la cabeza (Janiceps), con fusión ventro-ventral de los ejes somáticos hasta debajo del ombligo sobre la pelvis, con sistemas respiratorios, cardiaco y genitourinario independientes, el sistema digestivo superior esta fusionado hasta el intestino medio, con duplicación a partir de la tercera porción del duodeno. Los gemelos siameses cefalópagos no son los más infrecuentes, pero es difícil encontrar series de casos y revisiones sistemáticas de sus características y patogénesis en Latinoamérica. Son escasas las estadísticas sobre siameses y no se ha descrito previamente ningún caso como éste. Se hace necesaria la realización de estudios de autopsias para mejorar la descripción y caracterización de estos casos y poder determinar si existen otras condiciones asociadas además de las teorías de unión embriológica(AU)

It is denominated siamese to those twins that share anatomical structures to a variable degree. The incidence varies between 1/50.000 and 1/100.000 pregnancies, 1:4 of the cases die in the uterus and 40-60 percent die shortly after birth. Findings in a medical-scientific autopsies of conjoined cephalopagus twins, those that presenting cephalic fusion with two hemi faces, are the fourth type of Siamese in occurrence and their incidence is estimated in 1 every 3´000.000 pregnancies or 1/58 siamese. It is about cephalopagus twins, with termination of pregnancy due to incompatibility with extrauterine life. They presented a five vessels common umbilical cord, fused craniums at frontofacial level with two faces in opposed sides of the head (Janiceps), ventro-ventral fusion of the somatic axis until below the umbilicus and above the pelvis, independent respiratory, cardiac and genitourinary systems. The superior digestive system was fused until the medium intestine, with duplication parting from the third portion of the duodenum. Conjoined cephalopagus twins are not the most frequent but it is hard to find a series of cases and systemic revisions of their characteristics and pathogenesis in Latin America. Statistics on Siamese twins are scarce and it has not been previously described any case like this one. Autopsy studies becomes necessary to improve the description and characterization of these cases and to determine the existence of any other associated conditions different to those of the embryologic union theory(AU)

Leftward Flow Determines Laterality in Conjoined Twins.

Conjoined twins fused at the thorax display an enigmatic left-right defect: although left twins are normal, laterality is disturbed in one-half of right twins [1-3]. Molecularly, this randomization corresponds to a lack of asymmetric Nodal cascade induction in right twins [4]. We studied leftward flow [5, 6] at the left-right organizer (LRO) [7, 8] in thoracopagus twins in Xenopus, which displayed a duplicated, fused, and ciliated LRO. Cilia were motile and produced a leftward flow from the right LRO margin of the right to the left margin of the left twin. Motility was required for correct laterality in left twins, as knockdown of dynein motor dnah9 prevented Nodal cascade induction. Nodal was rescued by parallel knockdown of the inhibitor dand5 [9, 10] on the left side of the left twin. Lack of Nodal induction in the right twin, despite the presence of flow, was due to insufficient suppression of dand5. Knockdown of dand5 at the center of the fused LRO resulted in asymmetric Nodal cascade induction in the right twin as well. Manipulation of leftward flow and dand5 in a targeted and sided manner induced the Nodal cascade in a predictable manner, in the left twin, the right one, both, or neither. Laterality in conjoined twins thus was determined by cilia-driven leftward fluid flow like in single embryos, which solves a century-old riddle, as the phenomenon was already studied by some of the founders of experimental embryology, including Dareste [11], Fol and Warynsky [12], and Spemann and Falkenberg [13] (reviewed in [14]).

Frederik Ruysch (1638-1731): Historical perspective and contemporary analysis of his teratological legacy.

The Peter the Great Museum of Anthropology and Ethnography (Kunstkamera) in Saint Petersburg is the oldest museum in Russia. It keeps the remains of the anatomical collection of the world-famous 17th century Dutch anatomist Frederik Ruysch. This unique collection was bought and shipped in 1717 by Czar Peter the Great, and presently still comprises more than 900 specimens, a modest number of which concerns specimens with congenital anomalies. We searched for teratological clues in the existing collection and in all his descriptions and correspondence regarding specimens and cases he encountered during his career as doctor anatomiae and chief instructor of the surgeons and midwives in Amsterdam. A total of 63 teratological specimens and case descriptions were identified in this legacy, including some exceedingly rare anomalies. As it turns out, Ruysch was the first to describe several of the conditions we encountered, including intracranial teratoma, enchondromatosis, and Majewski syndrome. Although his comments pose an interesting view on how congenital anomalies were scientifically perceived in early 18th century Europe, Ruysch mostly refrained from explaining the causes of the conditions he encountered. Instead, he dedicated himself to careful descriptions of his specimens. Almost 300 years after his demise, Ruysch's legacy still impresses and inspires both scientists and lay men. © 2016 The Authors. American Journal of Medical Genetics Part A Published by Wiley Periodicals, Inc.

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.

Virtopsy in conjoined ischiopagus twins.

PURPOSE OF INVESTIGATION: To propose a multidisciplinary protocol for postmortem disclosure of complex fetal malformations, comparing ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), and autopsy in a case of conjoined ischiopagus twins. MATERIALS AND METHODS: A screening second-trimester ultrasound diagnosed ischiopagus twins at 20 gestational weeks in a 31-year-old woman without any previous ultrasound examination. The couple decided for pregnancy termination. The formalin-fixed fetuses underwent full-body CT, MRI, and autopsy. RESULTS: ultrasound accurately diagnosed ischiopagus twins. CT was very accurate in the description of bone components. MRI allowed better visualization of the visceral organs than CT. Only autopsy could disclose the aspect of the two gastrointestinal tracts and the external genitalia. CONCLUSIONS: Prenatal ultrasound represents the standard diagnostic exam for conjoined twins. CT-MRI virtual autopsy (virtopsy) may be an option if the couple refuses to authorize necropsy or may be useful to plan a minimally invasive autopsy preserving the external phenotype.

Fetus in Fetu: Two Cases and Literature Review.

Fetus in fetu is a rare cause of abdominal mass in infants wherein a parasitic twin grows inside a host. The true etiology is unclear. Various theories have been postulated. We report two cases of retroperitoneal fetus in fetu in female infants.

Diprosopus: Systematic review and report of two cases.

BACKGROUND: Diprosopus is a subtype of symmetric conjoined twins with one head, facial duplication and a single trunk. Diprosopus is a very rare congenital anomaly. METHODS: This is a systematic review of published cases and the presentation of two new cases born in Argentina. We estimated the prevalence of conjoined twins and diprosopus using data from the National Network of Congenital Anomalies of Argentina (RENAC). RESULTS: The prevalence of conjoined twins in RENAC was 19 per 1,000,000 births (95% confidence interval, 12-29). Diprosopus prevalence was 2 per 1,000,000 births (95% confidence interval, 0.2-6.8). In the systematic review, we identified 31 diprosopus cases. The facial structures more frequently duplicated were nose and eyes. Most frequent associated anomalies were: anencephaly, duplication of cerebral hemispheres, craniorachischisis, oral clefts, spinal abnormalities, congenital heart defects, diaphragmatic hernia, thoracic and/or abdominal visceral laterality anomalies. One of the RENAC cases and three cases from the literature had another discordant nonmalformed twin. CONCLUSION: The conjoined twins prevalence was similar to other studies. The prevalence of diprosopus was higher. The etiology is still unknown. The presence of visceral laterality anomalies may indicate the link between diprosopus and the alteration or duplication of the primitive node in the perigastrulation period (12-15 days postfertilization). Pregnancies of more than two embryos may be a risk factor for diprosopus. Given the low prevalence of this defect, it would be useful to perform studies involving several surveillance systems and international consortiums. Birth Defects Research (Part A), 2016. © 2016 Wiley Periodicals, Inc. Birth Defects Research (Part A) 106:993-1007, 2016. © 2016 Wiley Periodicals, Inc.

Separation of Thoraco-Omphalo-Ischiopagus Conjoined Twins: Surgical Planning, Management, and Outcomes.

BACKGROUND: Conjoined twins are a rare medical phenomenon that offers a unique challenge for medical professionals. The complex anatomy of conjoined twins dictates their survival and amenability to separation, making each case different in terms of medical management, surgical planning, and patient outcomes. Thoraco-omphalo-ischiopagus twins, joined from the thorax to the pelvis, are one of the rarest orientations recorded in the medical literature, and successful separation of this subset of conjoined twins has not been documented. This report presents a novel case of thoraco-omphalo-ischiopagus tetrapus twins who were successfully separated at 10 months of age. The preoperative planning, operative details, and postoperative course are discussed as they relate to the reconstructive effort. METHODS: Three-dimensional medical modeling was pursued early in the planning process and was used to estimate the soft-tissue requirements for reconstruction and to design custom tissue expanders. RESULTS: The reconstructive effort required postponement until respiratory status was optimized. Even with elaborate preoperative planning, primary closure of the abdomen was limited because of tissue edema and other less predictable patient factors. Delayed closure of the abdominal wall was made possible with negative-pressure wound therapy and secondary flap advancements. CONCLUSION: Preoperative coordination with necessary vendors, a multidisciplinary surgical effort, and optimal timing of the surgical intervention all contribute to the successful separation and long-term survival of thoraco-omphalo-ischiopagus conjoined twins. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.