Twin reversed arterial perfusion syndrome in a monochorionic monoamniotic twin pregnancy.

OBJECTIVE: Twin-Reversed Arterial Perfusion (TRAP) sequence is a rare complication of monochorionic multiple gestation. Conservative management should be considered if there is no poor prognostic factor. CASE REPORT: This is a 35 year-old female with twin pregnancy with acardiac monster. Under the request of the patient, there was no intervention during the whole pregnancy. We keep regular and close sonography weekly follow up. There was no maternal complication and there was also no heart failure sign or polyhydramnios of the donor twin. Minimal blood flow was noted at the anastomotic vessels under the sonography at late gestational age. Due to breech presentation, cesarean section was performed at gestational age 37 + 1/7 weeks. She delivers a healthy baby smoothly. CONCLUSION: Antenatal sonography is an important tool to evaluate the fetus status. Under special condition, term pregnancy is still possible without any treatment. CASE REPORT: Twin reversed arterial perfusion syndrome in a monochorionic monoamniotic twin pregnancy.

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.

[Morphologic and tomodensitometric comparative analysis of two cyclotocephalic newborns]. / Analyse morphologique et tomodensitométrique comparée de deux nouveau-nés affectés d'une cyclotocéphalie.

Two specimens of cyclotocephalic newborns, coming from museum collections, have been the subject of a comparative morphologic analysis. This one comprised an external examination and a CT scan exploration. If the craniofacial features were similar, one had a quasi-normal brain, while the second specimen displayed alobar holoprosencephaly. This observation relatively surprising is discussed in the light of current knowledge on the developmental genetics of this family of malformations.

Monoamniotic twins discordant for body stalk anomaly.

Body stalk anomaly is a rare malformation. This anomaly in monozygotic twins is extremely unusual. We describe a case of monoamniotic pregnancy discordant for body stalk anomaly diagnosed at 11 weeks. Ultrasound showed a fetus with a large anterior abdominal wall defect, anomaly of the spine and no evidence of lower extremities and other with a normal morphology. As far as our concern, only three monoamniotic pregnancies discordant for this malformation were reported. Our case represents the fourth reported monoamniotic pregnancy discordant for body stalk anomaly with diagnosis made by ultrasound and the second diagnosed in the first trimester.

Dichorionic triamniotic triplet pregnancy complicated by acardius acormus.

This report describes an acardiac fetus of the acormus phenotype in a triplet pregnancy. The diagnosis was confirmed at 15 weeks. In the absence of signs of heart failure in the co-fetus the pregnancy was managed conservatively. The pregnancy was complicated by preterm labour and the fetuses were delivered at 26+5 weeks. The prenatal diagnosis of the acormus phenotype with a well-developed cephalic pole is extremely rare and has never been described antenatally in a higher order multiple pregnancy. We suggest that this rare acardiac fetus phenotype may have a different pathophysiology than those of other phenotypes. The report also summarizes the perinatal outcomes of triplet pregnancies complicated by an acardiac fetus, where the median gestational age at delivery is 26-27 weeks, and discusses the possible therapeutic interventions.

Antenatal diagnosis of twin-reversed arterial perfusion sequence (acardiac amorphous) in a triplet pregnancy: case report.

Acardiac twinning is the most extreme form of twin-twin transfusion syndrome occurring in monzygotic twin pregnancies with monochorionic placentation. A case of acardiac amorphous foetus, occurring in association with spontaneously conceived triplet pregnancy, diagnosed on ultrasound in early second trimester is described and its antenatal management and brief review of literature discussed.

Epignathus: always a simple teratoma? Report of an exceptional case with two additional fetiforme bodies.

We report on a case of a fetal epignathus combined with two fetus-like structures resembling acardius acranius. The anomaly was detected at 23 weeks of gestation and led to termination of pregnancy at 24 weeks. This is the first description of epignathus with parasitic fetuses detected prenatally. It shows that the boundary between fetal teratoma and multiple pregnancy in special cases may be difficult to define.

Computed tomography of a cyclotocephalic neonate.

Cyclotocephaly is a very rare malformative lethal condition which associates otocephaly (extreme hypoplasia of the mandibular arch with agnathia) and cyclopy with proboscis. The head of a cyclotocephalic neonate from our Museum of Anatomy and Embryology was examined using computed tomography (CT). Cutaneous and osseous three-dimensional reformations were performed. Severe bony malformations were observed. A single orbital cavity was surrounded by a cartilaginous proboscis and a median fusion of maxillae, temporal and zygomatic bones. The single orbital cavity contained both paramedial eyeballs (synophthalmia). The external auditory meati and the ear pinnae were also parasagittal. No oral cavity and mandible were observed. Despite the poor conservation state of the brain, lobar holoprosencephaly was suspected. The mesencephalon and pituitary gland were absent. This exercise could lead to optimizing ultrasonographic prenatal diagnosis.

Prenatal sonographic diagnosis of a twinning epigastric heteropagus.

Epigastric heteropagus is a rare type of conjoined twinning which results from an ischemic atrophy of one fetus at an early stage of gestation. We present what we believe to be the first case diagnosed antenatally at 22 weeks' gestation. The pelvis and lower limbs of the ischemic fetus (the parasite) were attached to the epigastrium of the well-developed fetus (the autosite), which had a small omphalocele. Antenatal sonography provided an accurate diagnosis, enabling unnecessary abortion to be avoided.

[Acardius acephalus. Description of a case]. / Acardius acephalus. Descripción de un caso.

We report a case of twin gestation (monochorionic-diamniotic) in which one of the twins was acardiac and acephalic. His co-twin suffered infectious, hematological and neurological complications. This condition belongs to the broad spectrum of twin-to-twin transfusion syndrome (TTTS) and is observed in monozygous pregnancies in which the vascular systems of the fetuses are connected. The severity of this syndrome depends on the type of anastomoses and the timing of their establishment. Circulation is accomplished by the heart of the more perfect twin ("pump twin") who is at risk for heart failure. The recipient twin may display severe and sometimes lethal anomalies, such as acardia or acephalus. In some cases a prior situation such as a single umbilical artery or chromosomal anomaly in the acardiac twin may be found. Early diagnosis is essential to formulate a plan of management focused on the normal twin.

Treatment of acardiac twinning.

BACKGROUND: The twin reversed-arterial-perfusion sequence is a complication of monochorionic twin pregnancies characterized by the hemodynamic dependence of a "recipient" twin from a "pump" twin. The recipient twin exhibits lethal abnormalities including acardia and acephaly. The pump twin has a mortality rate of 50% as a result of high-output heart failure. CASE: The blood supply to an acardiac-acephalus twin was interrupted at 24 weeks' gestation using endoscopic laser coagulation. The co-twin was delivered at 35 weeks and had an uneventful neonatal course following correction of a persistent patent ductus arteriosus. Review of the literature reveals 22 cases of acardiac twinning treated with invasive procedures, seven of them using endoscopic laser coagulation. Pump twin mortality with fetal surgery was 13.6% in comparison with 50% mortality with expectant management (P < .001). CONCLUSION: Fetal surgery is the best available treatment for acardiac twinning. Endoscopic laser coagulation at or before 24 weeks and endoscopic or sonographic guided umbilical cord ligation after this gestational age seem to be the best treatments for this condition.

Severe brain and limb defects with possible autosomal recessive inheritance: a series of six cases and review of the literature.

Six fetuses with normal chromosomes were found to have severe craniofacial, limb, and visceral malformations during the second trimester of pregnancy. Two of these fetuses were monozygotic twins while a third one had a healthy dizygotic twin brother. A case with familial recurrence was also observed. Autopsy and skeletal radiographs suggested several diagnoses such as neural tube defect with limb defects or XK aprosencephaly. The development of these severe conditions in monozygotic twins and familial recurrence emphasize the difficulties of genetic counseling in such situations. These cases may suggest autosomal recessive inheritance.

Skeletal deformities of acardius anceps: the gross and imaging features.

A morphology based imaging review is presented of the characteristic skeletal deformities associated with acardius anceps in three acardiac twins. These fetuses demonstrated poorly developed skulls, limb reduction defects, and phocomelia of the upper limbs, as well as narrow thoracic cages with or without the complete development of ribs, clavicles, scapulae, and cervical, thoracic, or lumbar vertebrae. However, their lower limbs and pelvic girdles were almost normal. The authors conclude that skeletal development is likely to be jeopardized in the area adjacent to the heart and in the cephalic portion of the body in fetuses with acardius anceps, and suggest vascular deficiency and hypoperfusion as pathogenetic mechanisms in this type of skeletal deformity.

[Early prenatal diagnosis of asymmetric cephalothoracopagus twins]. / Diagnostic anténatal précoce de jumeaux céphalo-thoracopages asymétriques.

The authors report on one case of cephalothoracopagus Janiceps conjoined twins discovered by echography at 18 weeks' gestation. Conjoined twins have been said to occur with a frequency of 1/50,000 to 1/100,000 deliveries. The incidence of the cephalothoracopagus variety is one in three million births or one in 58 conjoined twins. Three different mechanisms that may have played a role in the abnormal development of these twins are discussed. The risk that the condition recurs in a subsequent pregnancy are to be considered negligible.