Preoperative preparation and surgical separation of conjoined pygopagus twins

• DOI: 10.3760/cma.j.issn.0366-6999.2010.13.035
• Publication Date: 2024-01-16T16:22:27Z
• Authors: Tian-sheng SUN; Shao-guang LI; Zhi-cheng ZHANG; Fang LI; Kai GUAN
• Keywords: Conjoined twins

Conjoined twins are very rare with an estimated incidence of about 1 in 200 000 births a male-female ratio 1:3.1 The separation conjoined presents challenge to surgeons and also test for comprehensive efficiency hospital. Recently, pair pygopagus were admitted our hospital successful surgical was carried out. CASE REPORT male (Figure 1) delivered by cesarean section at 37th week gestation, transferred 2 months after birth. They had combined weight 8 kg. joined back the lumbar, sacral buttock regions. width conjunction bridge 6.0 cm on sagittal plane, whilst 7.7 frontal plane. length 7.9 circle 23 cm. One side appeared broader thicker. Each twin independent extremities, anus penis. cryptorchidism. All four extremities each functioned normally. Stimulation extremity one did not trigger response other.Figure 1: . Clinical photograph showing marks cross V-shaped skin flaps.Lateral view full-length spines showed neither anomalies nor spinal bony structural connection plain radiograph examination 2). CT three-dimensional reconstruction canal opened under level third lumbar. There no site junction 3). separate rectum. According original reformation model data spine skin, solid layered, produced industrial plastic materials through melt extrusion manufacturing (MEM) process, using Aurora, rapid prototyping system software 4). Magnetic resonance imaging (MRI) that from second lumbar vertebra, subcutaneous tissue there lamella, presenting low signal T1 T2 weighted spin-echo sequence, suggesting cartilage connecting twins. Lying deeper, near sacrococcygeal pelvic regions, epidural fat varying thickness. Below level, common dural sac. end cord situated cords well separated, innervation cauda equina seen. bladder rectum 5). Using AutoCAD software, perimeter area sac measured compared sum separated cephalic part order assess if could be directly closed separation.2 results quantitative measurements parts 4.6 4.9 respectively while 11.2 1.8 2.1cm2 respectively, 5.1 cm2. So it expected dual sutured separation. Somato-sensory evoked potential (SSEP) test: When stimulation electrode receiver crosswise placed both twins, amplitude wave recorded, indicating shared neural structure between Echocardiography patent ductus arteriosus normal heart function. Assessment liver, gall, pancreas kidneys revealed abnormalities. Ultrasound middle echo-free, nerves cross-dispositioned.Figure 2: Plain examination: lateral connection.Figure 3: junctionFigure 4: (A). (B).Figure 5: MR vertebra. seen.The operation difficult we much experienced. So, more departments got involved, limited time. A work team assembled administration as leading body, included orthopedics, neurosurgery, anesthesia, surgery, pediatric intensive care unit (PICU) other related departments. Team meetings held many times discuss ensure all together closely complete successfully. In main operating room where performed, anesthetic equipment duplicated. set operative prepared next-door room. Surgeons divided into two groups. Two microscopes prepared. synthetic Gore-Tex patches close defects sacs. Under general anethesis, decubitus position padded pressure areas. broad pedicle region upper. Skin axillae toes. started marking out flap over waist its based midline body. would eventually cover raw once separated. flaps equilateral triangles, being 10 tips pointing unbilicus 1). bedside marked methylene blue injected key points. upper incised raised deep fascia. Attention paid avoiding over-stretching hemostasis secured. base dissected sufficient 6A).Figure 6: successfully (B).The felt been raised. caudal first chosen cut carefully because MRI farther away dura due thick layer fat. exposed completely. Then cephalad protection elevator, off laminectomy rongeur. trimminged smooth. protected carefully. patients turned Trendelenburg position. open central No. 15 blade. It found hypertrophied fibers located ventral No adherence or 6B). opening extended toward ends completely last. left behind cotton wisps. then gradually down soft tissues. fascia lying above muscles. fasia superficial help planned incision line blue. complete. moderate size sacs allowed them without compromising their caliber. prone duras over-and-over whip 5–0 terylene braid threads. regions covered albumin gel bilateral sacrospinalis muscle flaps. primary suture. high tension detected. wound drained continuous negative sterilized dressing 7). total lasted 6 hours, anesthesia induction completion.Figure 7: separated.Postoperatively, briefly ventilated neonatal laid supporting sandbags back. Movements lower normal. extubated coming around. On postoperative day, drainage tubes withdrawn. At time, blood circulation began take food. fourth colour tip observed getting dark. twelfth 1.5 cm×2.5 necrosised formed scabs clear edges. Additional operations applied resect necrotic local rotated wounds healed dermal sutures made weeks later. leakage cerebrospinal fluid infection, any sign nerve damage. follow-up later, children developed movements bowel functions 8).Figure 8: clinical elasticity similar surrounded skin.DISCUSSION symmetrical forms asymmetrical forms. Symmetrical further classified according most prominent into: thorax (thoracopagus), abdomen (omphalopagus), (pygopagus), pelvis (ischiopagus), skull (craniopagus). Pygopagus is type. review literature3 identified organs region. share 100 percent, gastrointestinal (GI) tract 25% genitourinary (with single urethra) 15%, but equinae usually remain separate. case, fifth GI urinary shared. Caudae crossed confirmed SSEP. sharing important tissues probable. CAD techniques played crucial role success operation. Martinez et al4 reviewed sets traditional image examinations can used guidance planning sophisticated methods offer detailed information reconstruction. hand, provides real-life simulation directions. determining shape rehearsals before operation, especially sterile environment during complicated steps such preparing, draping, changing separating bed. repair defect. artificial defect ways rebuild sac, procedure relatively complicated. Moreover, foreign body fluid. AutoCAD, calculated perimeters areas proximal We former larger than latter, predicted artifical dura. circumstances matched software. complex bed avoided simplified prevented well. coverage when large. Ways commonly solve problem include grafting, insertion expanders, transferring so on.5 grafting good controlling infection preventing leakage. Insertion expanders easily lead infection.6 suitable better option. designed three-dimentional model. arised uniovular pregnancy failed undergo separation, cause rejection. triangles insure adequate supply tips. With V-Y method closure, graft needed. After survived except became necrotic, infection. secondarily rotational Our experience shows depends whether accurate investigation, preoperative preparation, delicate techniques, rigorous management well-coordinated multi-disciplinary approach, success.