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.

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.

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.

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)

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.

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.

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.

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.

Morphological study of cephalothoracopagus deradelphus type conjoined twins. A case report.

Cephalopagus is a rare variety of conjoined twins. They are fused with their heads, thoracic and upper abdominal cavities. The exact mechanism for development of conjoined twins cannot be clearly explained. It appears that there is an alteration in the normal developmental process of monozygotic twins, which fail to separate from each other. We present the morphology of a cephalothoracopagus, revealed through anatomical dissection, emphasizing the arrangement of the viscera in the thoracic and abdominal cavities. They are fused with their heads, thoracic and upper abdominal cavities. The lower abdomen and pelvic cavities are free. Each twin has two upper and lower limbs, normally shaped. Each twin has a heart and two lungs. There is a single pharynx, esophagus and stomach, but normal lower abdominal systems. The genital and urinary systems are apparently normal. Due to the fusion of the heads and abnormal arrangement of the superior central nervous system, surgery is not attempted in these cases, the prognosis being very poor.

Parasitic twin--a supernumerary limb associated with spinal malformations. A case report.

We describe a case of rachipagus parasitic twin with spinal cord malformations (lipomyelomeningocele and tethered cord) in a 7-month-old Ethiopian infant. The parasitic mass had a well-formed foot, ankle and lower leg and a small sinus that resembled an anus. Magnetic resonance imaging scans revealed spinal malformations including a distal syringohydromyelia. The mass was successfully resected and the dural attachment was closed. Histopathological examination confirmed the diagnosis. Postoperatively, the child had unchanged, intact neurological function in both lower limbs. Almost all rachipagus parasitic twins are associated with spinal malformations. They should, therefore, be operated on by surgeons experienced in myelomeningocele surgery.

Conjoined twins: Radiological experience.

Imaging plays a key role in the management of conjoined twins. Pre-operative multi-modality studies are vital to assess operability and to aid surgical planning. Technical advances in imaging such as high-resolution isovolumetric magnetic resonance imaging (MRI) techniques and three-dimensional modeling now result in extremely accurate anatomical information. Varied information from a comprehensive radiological work-up enables the surgeons to plan the safest possible operative procedure, helps the anesthetic team before and during surgery, and guides the intensive care team in the post-operative phase. This article will review the radiological techniques used in our institution, highlighting potential pitfalls with the various imaging modalities.

The management of conjoined twins: Cardiology assessment.

Structural cardiac defects occur in at least 1 twin in about 75% of conjoined twins with thoracic level fusion. Outcomes after surgical separation of thoracic level conjoined twins have been favorable when the hearts have been separate. However, even in this situation, the outlook is poor for an individual twin with an important cardiac defect. Arterial anastomosis between twin circulations is an important additional consideration, with poor outcomes for perfusion recipient twins. Surgical separation is contraindicated when ventricular level cardiac fusion exists. Cardiac assessment is a key component of prenatal counseling.

Planning the operation.

The management of conjoined twins falls into three distinct groups-non-operative, emergency separation and elective separation. Planning meetings involving all the personnel who will be required during the operation are held. The radiological findings are presented and the anaesthetic, nursing and intensive care requirements are highlighted.

The separation procedure.

The various stages of the separation are carefully planned but despite this, variations which will change the schedule of the procedure may exist. In general the operation commences on the opposite side from the main procedure and then the twins are turned for the remainder of the operation. Each type of conjoined twin is different but basically thoracopagus involves the hearts, omphalopagus involves the liver and small intestine and ischiopagus involves the large intestine and genito-urinary system. Our results are presented together with interesting cases from which lessons have been learned.

Urological management.

The pediatric urologists role is confined to ischio-, para-, and pygopagus conjoined twins. The aim is to preserve renal function, to address problems with urinary continence, and to optimize genital issues with particular reference to sexual and reproductive function. Full urological imaging is essential prior to separation for planning and for the operation itself.

Staged separation of craniopagus twins.

Craniopagus twins are rare and account for up to 6% of all conjoined twins. No hospital will encounter many such twins and the opportunity to develop expertise is limited. We have dealt with 2 such sets and illustrate our approach by reference to our most recent set. We believe that detailed imaging allows precise delineation of the anatomy and facilitates detailed planning of the surgery. When venous drainage from the 2 brains is connected, we believe that staged separation is preferable as gradual alteration of hemodynamics may be safer than a single-stage procedure.

Orthopedic aspects of separation.

In the management of conjoined twins the orthopedic surgeon may be involved directly, as part of the surgical team at operation or indirectly, in dealing with associated anomalies or the sequelae of surgery. The overwhelming indication for orthopedic involvement is ischiopagus, either tripus or tetrapus. The main role of the orthopedic surgeon is to facilitate midline closure through the use of pelvic osteotomies, which allow the symphysis pubis to be approximated.

Cardiothoracic surgery.

Conjoined twins often have complex cardiac anomalies associated with other congenital defects. The correct cardiac diagnosis delineates the degree of cardiac fusion and the feasibility of separation. The outcome in twins with fused hearts remains poor.