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Introduction: Conjoined twins are identical twins with fused bodies, joined in utero. They
are rare complications of monochorionic twinning. The purpose of this study is to describe
the various types of conjoined twins, the role of imaging and recent advances aiding in
their management. Material and methods: This was a twin institutional study invo...
Context in source publication
Context 1
... first was a case of cephalopagus twins (Fig. 2) of 28 weeks gestational age diagnosed in a 29-year-old primi- gravida. Ultrasound (Fig. 3) revealed conjoined twins fused from the top of the head down to the umbilicus with a single large face with hypotelorism, two unfused thoracic spines, fused thorax with two hearts, eight limbs, and two separated ...
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Conjoined twin is a rare complication seen in 1% of monochorionic twins and associated with severe morbidity and mortality. It occurs due to a division event at the primitive streak stage of the human embryonic development at about 13-14 days after fertilisation, in monochorionic monoamniotic gestations. Early prenatal diagnosis of conjoined twin p...
Citations
... Another modality that can be used is magnetic resonance imaging (MRI), which offers enhanced precision in providing anatomically detailed radiological images compared to ultrasound. [12][13][14] In the present case, a prenatal ultrasound assessment is performed during the 17th to 18th weeks of pregnancy. The result showed that the cranium and the thalamus are joining, affecting the brain's structure. ...
... 18 Once applicable, contemporary techniques such as 3D printing may facilitate surgical pre-planning and subsequent separation. 13 A viable pregnancy is easier to terminate vaginally if the diagnosis is made before that time, which may lessen the risk of trauma. 19 As the diagnosis progresses, the probability of achieving termination via cesarean section augments. ...
HIGHLIGHTS Conjoined twins have captivated mankind for centuries due to the rarity of this birth type; nonetheless, physicians have constantly encountered difficulties in dealing with conjoined twins. Early diagnosis of conjoined twins during prenatal examination is critical for ascertaining the prognosis of the fetus, guiding parental counseling over appropriate courses of action, and potentially enabling the termination of the pregnancy. First-trimester ultrasonography and MRI are complementary modalities in determining the diagnosis and prognosis of conjoined twins in early pregnancy. ABSTRACT Objectives: To discuss the crucial early diagnosis of conjoined twins to determine the type and prognosis. Case Report: A 27-year-old woman was referred to the type A referral hospital with suspicion of congenital abnormalities at 17 weeks of pregnancy. Ultrasound results showed intrauterine monochorionic monoamniotic twins with babies fused on their heads to the urogenital part. The MRI showed a craniopagus, suspected meningomyelocele, and severe bilateral hydronephrosis in the second baby. Due to non-separable cases and a bad prognosis for the fetus, the pregnancy was terminated using misoprostol induction and inserting a balloon catheter. The fetus is born weighing 400 g and 20 cm in length. Conjoined twins obtained the rostral type: a fused head with two faces, a fused thorax until the lower abdomen with one umbilicus, and two pairs of hands and feet. The diagnosis of conjoint twins becomes a problem in early pregnancy, mostly in developing countries. Early diagnosis of conjoined twins during prenatal examination is critical for ascertaining the prognosis of the fetus, guiding parental counseling over appropriate courses of action, and potentially enabling the termination of the pregnancy to prevent maternal stress and complications. Conclusion: Conjoined twins should be identified as soon as feasible to establish the best course of management for both mother and fetus. Ultrasonography and MRI are modalities for determining the diagnosis and prognosis of conjoined twins.
... There seems to be a higher proportion within the female population, with ratio of 3: 1 [2]. Based on the anatomical fusion site, conjoined twins can be categorized into thoracopagus (chest), omphalopagus (abdomen), thoraco-omphalopagus (chest and abdomen), pygopagus (sacrum), ischiopagus (pelvis), craniopagus (cranium), parapagus (one side), cephalopagus (head), and rachipagus (spine) [1,3]. The ischiopagus type is an uncommon variety of conjoined twins, with joint site at the pelvis, and accounts for only 6% to 11% of all conjoined twins [4]. ...
... The diagnostic examinations that aim for thorough preoperative evaluation involve plain radiography, ultrasonography, contrast imaging of the gastrointestinal and urinary systems, endoscopy, echocardiography, CT angiography, and magnetic resonance imaging [7]. Three-dimensional (3D) imaging can be helpful in preoperative planning since it can provide anatomical details and vascularization of the fused organs [3]. ...
Patient: Male, 3-year-old
Final Diagnosis: Ischiopagus tripus conjoined twins after surgical correction
Symptoms: Fused body in pelvis reion
Clinical Procedure: Amputation • osteotomy
Specialty: Orthopedics and Traumatology
Objective
Congenital defects/diseases
Background
The rarity of ischiopagus tripus conjoined twins complicates the surgical separation, owing to the lack of cases and high complexity. We aim to report our experience in performing orthopedic correction for ischiopagus tripus twins.
Case Report
A pair of 3-year-old conjoined boys presented with a fused body at the pelvis region and only 1 umbilicus. There were 2 legs separated by shared genitalia and an anus at the midline, and 1 fused leg, which could be felt and moved by both of the patients. The twins also shared internal organs of the bladder, intestine, and rectum, as visualized through angiography computerized tomography scan. After several team discussions with the institutional review board, the hospital ethics committee, and both parents, it was agreed to perform disarticulation of the fused third limb, followed by correction of the trunk alignment by pelvic closed wedge osteotomy and internal fixation. We successfully reconstructed the pelvis using locking plates and additional 3.5-mm cortical screws and 1.2-mm stainless steel wire.
Conclusions
This report describes the presentation and surgical management of a case of ischiopagus tripus conjoined twins. It highlights the challenges involved in surgery and the importance of investigating these infants for other congenital abnormalities. Although surgical approaches for different sets of twins should be individually tailored, interventions aimed to provide optimal outcomes should consider ethical issues and parental/patient expectations. Even in situations in which the twins are inseparable, there is still room for surgical correction to be performed.
... Conjoined twins are identical twins joined in utero and are a rare phenomenon, 1 in 50,000-100,000 births. 40-60% are stillborn, and 35% of live births die within 24 hours [1]. Females have a slightly higher predominance, 2 : 1 to 3 : 1 [2]. ...
Conjoined twins are identical twins joined in utero and are a rare phenomenon. This report discusses a case of female thoraco-omphalo-ischiopagus tripus conjoined twins. The twins were separated at age two, and once medically stable, spent one month in inpatient rehabilitation to improve their sitting balance and gross motor skills. This was followed by outpatient physical therapy. The twins initially had customized ZipZac seats, which they were able to wheel independently. After six months of therapy, the girls began walking with posterior walkers and prostheses. The hemipelvectomy prosthesis included a customized thoracolumbosacral orthosis component and was directly attached to a non-articulated pylon. A manual-locking hip joint was added to accommodate sitting. An articulated ankle-foot orthosis was used for the intact leg. Care of formerly conjoined twins requires comprehensive care from a multidisciplinary team involving, but not limited to, a physiatrist, orthopaedic surgeon, physical therapist, and orthotist/prosthetist. Complex congenital limb deficiencies are often a major undertaking for the rehabilitation team as continuous treatment and management are needed throughout the patient’s lifetime due to growth, development, and evolving physical demands. Anatomic variations must be examined on a case-by-case basis but often include limb deficiencies, orthopedic abnormalities, and organ comorbidities.
... Due to the increased risk of neonatal morbidity and mortality, conjoined twins should be monitored prenatally by ultrasonography in the first trimester. Ultrasound scanning and magnetic resonance imaging can be utilized to confirm and clarify the diagnosis in the second trimester by establishing the precise anatomy of the two fetuses and the parts fused in detail [5,6]. Ultrasound features that encourage the identification of conjoined twins include inseparable body and skin contours, fetuses facing one another with hyperflexed cervical spines, fewer limbs than expected, organ sharing, and a single umbilical cord with more than three veins [7,8]. ...
Identical twins joined in utero are called conjoined twins. They are also called “Siamese twins.” It is one of the uncommon variants of twin pregnancy. Our case report aims to demonstrate the significance of prenatal diagnosis and evaluation of conjoined twins due to the increased risk of perinatal morbidity and death. Early prenatal diagnosis and assessment of the degree of joining provide an opportunity for parents to decide whether to continue the pregnancy.
... However, postnatal echocardiography and cranial and abdominal US are required for screening of all conjoined twins irrespective of their type. Abdominal US can demonstrate anomalies in the urinary system and detect hydronephrosis or renal fusion [15]. Chest and abdominopelvic radiography is also required for an overall evaluation. ...
Medical imaging plays a crucial role in the management of conjoined twins. The role of imaging is to explore the anatomy, outline the shared organs and determine whether surgery is feasible. It also serves as a roadmap for successful separation. Additionally, imaging helps with counseling parents about prognosis. This review aims to illustrate recent advances in different imaging modalities and their role in the management of the various types of conjoined twins with an emphasis on relevant tips for optimal imaging.
Graphical abstract
... Delivery is always preferred in a tertiary care centre for want of immediate paediatric and paediatric surgical support. Surgical separation is not done in cephalopagus twins as the success rate is negligible [5][6][7][8]. Parents need to be counselled for further pregnancies. ...
Background
Conjoined twin pregnancy is a very rare variety of twin pregnancy which results from the failure of complete separation of a zygote after 13 days, ‘cephalopagus’ being the rarest variety of such twin pregnancies. Two forms of cephalopagus conjoined twins have been described in the literature: Janiceps (two faces are on the either side of the head) and non-janiceps (with one head and a single face); non-janiceps type being even rarer. The features that may hint towards the diagnosis on ultrasound include inseparable fetal bodies, unvarying relative positioning of the two fetuses, both heads persistently at the same level to each other, bi-breech or bicephalic presentations and a single umbilical cord with more than three vessels. 3-D ultrasound and Fetal MRI may help in confirmation of this rare entity.
Case presentation
A 19-year-old primigravida, presented at 12 weeks of gestation for antenatal ultrasound. Ultrasonography showed evidence of a conjoined twin with a single head and single face, fused thoracic cavities with single heart, two lungs, fused upper abdomen with shared stomach and liver, separate pelvic cavities with two different urinary bladders and two pairs of upper and lower limbs. Fetal MRI corroborated these findings. The pregnancy was terminated vaginally, and cephalopagus fetus was delivered. The parents were appropriately counselled for future pregnancies.
Conclusions
A very rare case of non-Janiceps variety of cephalopagus conjoined twin gestation which was detected at 12 weeks of gestation by antenatal ultrasound and confirmed on fetal MRI. Early antenatal diagnosis is extremely important for appropriate and timely management as the prognosis is very poor in such twins. Antenatal ultrasound and fetal MRI prove very beneficial in this aspect as well as for diagnosis of other associated fetal abnormalities.
... In the second trimester, ultrasound assessment and magnetic resonance imaging can further improve and confirm the diagnosis by viewing the detailed anatomy and shared parts of the two fetuses. The prenatal ultrasound diagnosis characteristics of conjoined twins include a single placenta without septum; fetuses in the same constant position with head and body parts at the same level; inseparable body and skin contours; fetuses facing each other with cervical spine hyperflexion, sharing organs and a single umbilical cord with over three blood vessels; fewer limbs than normal twins; and abnormal flexion of the spine (Mathew et al., 2017). ...
Research question: Are there any differences between conjoined twin fetuses at the molecular level?
Design: Skin tissues were collected from thoracopagus conjoined twins at 15+4 weeks of gestation. The skin tissues were
collected from the thigh side of conjoined twins after the abortion procedure. All specimens were obtained after written
informed patient consent and were fully anonymized. All relevant ethical regulations were followed. Every specimen underwent
multiomics sequencing analysis to determine associations among the DNA methylome, transcriptome and mutations in the exon
regions in the conjoined twins.
Results: The global methylation pattern was similar in the two fetuses of conjoined twins, while significant differences were seen in
local regions such as CpG islands (P = 0.026), enhancers (P <0.001) and various repetitive elements (P < 0.05), which showed
significant differences. The conjoined twins also differed in genes related to growth and development, cellular component
morphogenesis and cellular stress, both in terms of DNA methylation levels and gene expression levels. Exon data analysis revealed
that the common mutations in conjoined twins mainly occurred in neural development, lipid metabolism and microtubule
morphogenesis. Specific mutations were associated with cellular component biosynthesis, behaviour and germ cell development.
Conclusion: Conjoined twins were similar to each other globally, but there were significant differences related to growth and
development, cellular component morphogenesis and cellular stress. The current study reveals the molecular features of
conjoined twins for the first time, laying the foundation for future exploration of the mechanism of conjoined twins.
... Modern techniques like 3D printing may assist with surgical pre-planning and subsequent separation, if necessary. 11 The approach to manages conjoined twins is complex. Conjoined twins can be separated into the following groups with regard to prognosis: those who do not survive in utero, those who survive pregnancy but do not survive past infancy, those who survive infancy, but cannot be separated and those who survive infancy and can be surgically separated. ...
Unlabelled:
Conjoined twins (Siamese twins) represent the rarest form of twin pregnancy. Reported here are two rare cases of conjoined term twins presented to the department of Obstetrics and Gynaecology within 3 months. The first case, 32 years of gravida 6 parity 5 referred from periphery after full trial of labour following multi-organ dysfunction and term intrauterine dead twins. Intraoperatively it was dead conjoined thoraco-omphalopagus females. The patient died after 3 days following multiorgan dysfunction syndrome and disseminated intravascular coagulation. The second case, 22 years gravida 2 parity 1 also referred from periphery in second stage of labour with diagnosis of 39 weeks intrauterine dead twins with obstructed labour, delivered by caesarean with intraoperative conjoined dead females of thoracophagus type. Twins are high-risk pregnancy. This rare diagnosis with complications could have been prevented by regular antenatal checkups, ultrasonography performed by radiologists and early referral antenatally in labour along with multidisciplinary approach.
Keywords:
conjoined; monozygotic twinning; siamese twins; twins.
... Anomalies that have been described in conjoined twins have been elaborated [11] The surgical management of the conjoined twins recognizes three categories [8,9]: ...
To report a case of cephalo-thoracophagus janicep monosymmetros. A 30 year old female of child bearing age came to the department of radiology for routine obstetric ultrasonography with gestational age of 30 weeks. Patient had never conceived previously. In the current pregnancy patient had complaints in relation with the pregnancy. Routine ultrasonography was performed to obtain diagnostic output. Radiological evaluation revealed conjoined monochorionic monoamniotic twin pregnancy. Detailed screening revealed fusion of head (cephalophagus) with two sided face of which single eye on one ventral side (cyclops) and two eyes on the dorsal side (Janicep) along with fusion of the thorax (thoracophagus). 'Janicep' is derived from the word Janus, the two faced roman God. The presence of this prototype makes the case report of a rare variety. Cephalothoracophagus janicep monosymmetros has a prevalence 1:50,000 to 1:200,000 with a male:female ratio of 1:3. However post caesarean delivery of the conjoined twin revealed female sex making the case report more rare.
... Postnatal magnetic resonance imaging should be guided by prenatal imaging [27]. If necessary, surgical pre-planning and subsequent separation may benefit from modern techniques like 3D printing [28]. ...
... For surgical planning and prognosis, an imaging method that precisely characterizes anatomic fusion, vascular anomalies, and related abnormalities is essential. Accurate preoperative imaging facilitates conjoined twin separation [28]. ...
Conjoined twins are described as having been physically fused during pregnancy and delivery. They were first mentioned a long time ago when there was not much known about this. They share some organs that are vital for survival, like the heart; these twins are almost impossible to save, but there are some cases wherein there is evidence of their survival. The article aims to present a unique discussion on conjoined twins. This article talks about the formation of conjoint twins, their types and nomenclatures, embryological concepts, past history/traditional tales, case studies, and the medical enhancements happening in this area. Both fission and fusion are thought to contribute to the disease. A monozygotic twin pregnancy cleaves when it occurs more than thirteen days after fertilization. There is just one placenta and one womb for conjoined twins (one amniotic sac). The twins that are born and stay alive after delivery usually stay alive for a few days or weeks. It's pretty rare for them to live a long prosperous life, but this article shows the otherwise, too, like the Siamese twins, which is a unique example of conjoint twins who lived for a long time. This kind of pregnancy is a complex procedure that needs to be managed by a team of professionals.
