Parasitic Rachipagus Conjoined Twins With Spina Bifida, Diplomyelia, Scoliosis, Tethered Cord Syndrome, and Ventricular Septal Defect -Case Report-

Abstract

A 17-year-old girl presented with a rare case of parasitic rachipagus conjoined twins associated with spina bifida, diplomyelia, scoliosis, tethered cord syndrome, and ventricular septal defect. Physical examination found a well developed breast and an apophysis on the back of the patient, and neuroimaging demonstrated scoliosis, spina bifida from T8 to L5, butterfly-shaped vertebra of T6, abnormal bone behind T7, diplomyelia, and tethered cord. Successful surgical excision of the parasitic mass was performed. Histological examination discovered uterine tube, Muller's duct, bone, cartilage, and nerve tissue besides the mammary gland. Rachipagus conjoined twins are extremely rare, but should be considered if well developed abnormal tissue is found in the dorsal midline of the spine. However, the possibility of malformations in other organs in the autosite should be considered. Careful preoperative examination and refined microsurgery may provide good outcome for the patient.

Parasitic rachipagus twins; report of two cases

“…Due to this embryologic basis, most reported cases of rachipagus including this report are associated with some neural tube defects like spina bifida, lipomyelomeningocele, and diastematomyelia [4,10].…”

“…At the third or fourth gestational weeks, the neural folds of the two different embryos can merge if the skin covering the neural tube gets damaged. If the two embryos develop fair and equally, two complete but conjoined fetuses arise [10]. But, in most cases, one of the twins dies and some parts of its body can remain attached to the vertebral column of the other fetus [10,3].…”

“…However, conjoined twinning is very rare, and the rachipagus is even more rare [1,2]. An ectopic or accessory limb attached to the back is an extremely rare and strange condition, and there are only a few documented cases in the worldwide literature [1][2][3][4][7][8][9][10]. So the aim of this study is to present two patients of parasitic rachipagus with different presenting features.…”

A Rare Form of Heteropagus Twinning: Three-Armed Infant with Spinal Dysraphism

“…At the third or fourth gestational weeks, the neural folds of the two different embryos can merge if the skin covering the neural tube gets damaged. If the two embryos develop fair and equally, two complete but conjoined fetuses arise [7]. But, in most cases, one of the twins dies and some parts of its body can remain attached to the vertebral column of the other fetus [1, 7].…”

“…If the two embryos develop fair and equally, two complete but conjoined fetuses arise [7]. But, in most cases, one of the twins dies and some parts of its body can remain attached to the vertebral column of the other fetus [1, 7]. This attached part composed of primitive embryonic tissue prevents closure of the neural tube during later development, resulting in spina bifida, or other neural tube defects [4, 8].…”