1/352. Diprosopus (partially duplicated head) associated with anencephaly: a case report. Craniofacial duplication (diprosopus) is a rare form of conjoined twin. A 16 year old mother with a twin pregnancy delivered one normally formed baby boy and one diprosopus male. The malformed baby was 33 weeks of gestation with a single trunk, normal limbs and various degrees of facial duplication. Of the following structures there were two of each: noses, eyes, ears (and one dimple), mouths, tongues and, with bilateral central cleft lips and cleft palates. This was associated with holoprosencephaly and craniorachischisis. Internal organs showed no duplication. There were multiple congenital anomalies including diaphragmatic hernia, small lungs, two lobes of the right lung, ventricular septal defect, small adrenal gland and small left kidney with short ureter. The body also had a short neck, small chest cavities and kyphosis. X-ray revealed duplication of the vertebral column. The case presented here represents a type II of diprosopia of Rating (1933) and is the least common type reported. We also reviewed 22 recently reported cases of diprosopus. In addition to facial duplication, anencephaly, neural tube defect and cardiac malformations represent the more common congenital abnormalities associated with diprosopus. The pathogenesis of diprosopus is not well understood. Factors that play a role in diprosopus are probably similar to those factors (genetic, environmental and abnormal placental circulation) which affect monozoygotic twins as observed in this case report. Early ultrasonography diagnosis of diprosopus permits one to consider a vaginal therapeutic abortion. ( info) |
2/352. Delivery of Siamese twins by Caesarean section. The diagnosis of a pair of Siamese twins and their delivery by Caesarean section are described. A major cardiac anomaly precluded surgical separation. ( info) |
3/352. intelligence test scores from infancy to adulthood for a craniopagus twin pair neurosurgically separated at 4 months of age. Long-term effects in a neurosurgically separated twin pair were illuminated by standard psychological test scores obtained over a period from 2 to 38 years of age. Interdigitation of the gyri of their right frontal lobes had necessitated separation in two stages at 4 months of age. One twin clearly suffered some brain injury and showed some impairment during the testing at 5 years of age. The scores of both twins rose at the adult testing. The brighter twin has an IQ comparable to that of the mother. The unique data set is a kind of model for long-term assessment of early brain surgery, particularly with craniopagus twins. ( info) |
4/352. Scintigraphic evaluation of craniopagus twins. Craniopagus twinning is a rare congenital abnormality, occurring at a frequency of 4-6 per 10 million births. A case is reported in which separation was successful for both twins. The importance of pre-operative radionuclide assessment of crucial organ function (liver, kidneys, heart, brain) and crossed circulation is stressed. The scintigraphic results were in keeping with radiographic, operative, and clinical findings. The routine use of radionuclide studies in the investigation of conjoined twins is recommended in order to delineate individual organ function, degree of fusion and measurement of cross-circulation. ( info) |
5/352. Separation of complex pygopagus conjoined twins. Pygopagus twins were born with a unique spectrum of anomalies including a conjoined distal spinal cord, single kidney (in twin A), single rectum (in twin A), single vagina (in twin B), and severe central nervous system anomalies in twin B that precluded her independent survival. Separation at 10 weeks of age was tailored toward Twin A's survival. This report discusses the surgical modifications necessary in view of the unique anatomy, including salvaging the distal spinal cord and vagina for twin A. ( info) |
6/352. Morphological and cytogenetic studies on conjoined twins. Two cases of monoamniotic conjoined male twins, born at term after normal pregnancies, are reported. The first case, a bicephalus, shows hypoplastic and malformed left-side organs, absence of the left umbilical artery, and two communicating hearts, the left one with three cameras. The second case, a pygothoracopagus, consists in a twin "parasite", with no head but with two upper and two lower limbs, slightly less developed than those of the formed twin. The left eye of the formed twin is double than the right one and contains two eye apples -- one well-formed and the other rudimentary. There is a rudiment of a second mouth on the left cheek. The umbilical cord contains five blood vessels |
one umbilical vein and four umbilical arteries. The cytogenetic study of the pygothoracopagus reveals aneuploidy, more pronounced in the "parasite" than in the formed twin. ( info) |
7/352. A case of conjoined twins. Among 56,249 maternities in the Collaborative Perinatal Project, 615 were twin maternities. One of these resulted in stillborn thoracoomphalopagus MZ female twins with multiple cardiovascular, alimentary and other malformations. The case is of further interest in that the mother had a surgically removed brain tumor in childhood and exhibited neurological symptoms and bizarre behavior before and during pregnancy. Drugs and treatments which she received during pregnancy are not known to be teratogenic. ( info) |
8/352. Surgical ligation of a persistent arterial duct in one of conjoined thoracopagus twins prior to surgical separation. One of conjoined thoracopagus twins, with separate hearts and a common pericardial sack, presented with respiratory distress because of a persistent arterial duct causing congestive heart failure in the neonatal period. Surgical ligation of the duct was performed prior to subsequent separation, with an excellent outcome. ( info) |
9/352. Anesthetic and surgical experience in a case of total vertical craniopagus. BACKGROUND: A case of craniopagus twins is presented. The twins were attached to each other at the parietal vertex with an interaxis angle of 180 degrees and an interface angle of 90 degrees. To assess the bony, vascular, and nervous system interconnections, the twins underwent computerized tomographic scanning and cerebral angiography under inhalational general anesthesia. CASE DESCRIPTION: To provide adequate skin, tissue expanders were implanted under the scalp at 3 and 7 months of age. Separation became necessary at 1 year of age on an emergency basis, because of respiratory complications in the small twin. Profuse bleeding and hypovolemia led to the death of the healthy big twin. While one would expect that at least one of the twins could have been saved, the small twin also succumbed to air emboli and hypotension hours later. Postmortem examination revealed that the brains were joined at both medial and lateral hemispheric surfaces and shared a common circumferential sinus. CONCLUSION: This case was quite different as regards cerebral anatomy compared to those already reported in the literature. ( info) |
| BACKGROUND/PURPOSE: Conjoined twins often have different body composition and growth rate before separation. This may be because of differences in energy metabolism. The aim of this study was to investigate the energy expenditure, body composition, and calorie intake of thoracopagus conjoined twins with shared hepatic circulation but separate gastrointestinal tracts. methods: The twins were studied at two periods: (1) before separation (age, 73 days) and (2) after separation (age, 97 days). Calorie intake over the study periods was carefully documented. Respiratory gas exchange was measured by computerized indirect calorimetry. The postseparation weight ratio of twin A to twin B was used to approximate the preseparation weights. body composition (total body fat) was calculated from skinfold thickness and anthropometric measurements. RESULTS: The body composition of the twins was different: body weight and total body fat were higher in twin B. Resting energy expenditure and calorie intake were markedly different between the conjoined twins before separation. In both twins, the energy expenditure increased after separation. After separation, the resting energy expenditure of the twins was similar. CONCLUSIONS: This study illustrates the difference in energy metabolism in a set of thoracopagus conjoined twins. The authors speculate that twin A was supplying nutrients to twin B resulting in increased energy expenditure before separation. This would explain the lower calorie intake and higher fat mass of twin B. ( info) |