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Conjoined twins
- 1. Anesthesia For Conjoined Twins By Remon Nasr MD.
- 2. Interesting Facts • The first recorded conjoined twins were in England in 1100 A.D. • Conjoined twins have lived from a few minutes to 63 years • 70% of conjoined twins are female • 60% are still born • Up until 1990, surgical separation of conjoined twins has been attempted 167 times • Out of approximately every 400,000 births, four are conjoined. Of the four, 3 will die. Of the remaining set of twins, 70% of them will either die (one or both) or they will live out their lives handicapped. The babies that survive are truly miracle babies!
- 3. Conjoined Twins • History • Types • Anesthetic Management – Preoperative Preparation – Anesthetic Technique – Postoperative Period
- 4. History • Biddenden Maids Mary & Elisa Chulkhurst – Born in 1100, lived for 34 years in England – They were the first reported case of conjoined twins in history "As we came together, we will go together"
- 5. History • Siamese Twins Chang and Eng Bunker – Born in Siam (Thailand) in 1811 – Lived for 63 years. They married two sisters and between them they had 22 children
- 6. History • The Two-Headed Lady: Millie and Christine McCoy – Born in USA in 1851 – Died in 1912 seventeen hours apart
- 7. History • Recent Conjoined Twins Mohamed and Ahmed Ibrahim
- 8. History • Recent Conjoined Twins Laleh and Ladan Bijani
- 9. Conjoined Twins • History • Types • Anesthetic Management – Preoperative Preparation – Anesthetic Technique – Postoperative Period
- 10. Types This suffix is preceded by the anatomical site of conjunction Pagos = that which is joined or fixed
- 11. Types • Thoracopagus • Omphalopagus • Pygopagus • Ischiopagus • Craniopagus
- 12. Types • Thoracopagus – 40-45% of cases – Usually lie face to face with an exaggerated lordosis
- 13. Types • Thoracopagus Degree of fusion – Common sternum, diaphragm and upper abdominal wall from xiphisternum to umbilicus – Hearts are conjoined in 75% – Pericardial fusion in 90% – Fused intestinal tract in 50% – Fused biliary tract in 25% – Almost always a degree of hepatic fusion
- 14. Types • Omphalopagus – 33% of cases – Usually lie face to face
- 15. Types • Omphalopagus Degree of fusion – Joined from xiphisternum to umbilicus – May vary from extremely complex conjunction (thoraco-omphalo- ischiopagus) to a simple abdominal connection – Omphalocele is often present and may rupture during delivery – Bridge of hepatic tissue – Upper intestine is usually separate, but may have a common terminal ileum and colon
- 16. Types • Pygopagus – 19% of cases – Degree of fusion • Joined at the sacrum, buttocks, and / or perineum • Spinal cords usually separate • Distal bowel may be shared • Varieties of conjunction of the urogenital systems
- 17. Types • Ischiopagus – 6% of cases – May have two, three or four limbs – Depending on the number of limbs they are referred to as bipus, tripus or quadripus
- 18. Types • Ischiopagus Degree of fusion – Joined at the pelvis or from the umbilicus to the pelvis – Intestinal tract joined at the terminal ileum and colon – Four kidneys and two bladders are often present but crossover occurs – Male twins fusion of genitourinary tract distal to the bladder – Female twins four ovaries, two uteri and two sets of external genitalia
- 19. Types • Craniopagus – 2% of cases – Conjunction may occur any where on the skull but more commonly on the vertex, occiput or lateral parietal areas – High mortality and morbidity in this group – Degree of conjunction may be incompatible with separate existence or it may be merely a simple bony fusion
- 20. Types • Rare Types – Dicephalus
- 21. Types • Rare Types – Dicephalus – Xiphopagus
- 22. Types • Rare Types – Dicephalus – Xiphopagus – Cephalopagus
- 23. Types • Rare Types – Dicephalus – Xiphopagus – Cephalopagus – Parasitic Twin
- 24. Conjoined Twins • History • Types • Anesthetic Management – Preoperative Preparation – Anesthetic Technique – Postoperative Period
- 25. Anesthetic Management • Guidelines – All the basic principles of pediatric anesthesia apply – Anesthetic plans should be placed so as to treat each child as a separate individual with his or her own anesthetic team and equipment – The requirements of each baby may differ significantly at any stage of the operation – Surgery for separation may be elective or emergency
- 26. Anesthetic Management • Elective separation – One operation – Staged procedure • Emergency separation – When there is damage to a connecting bridge (omphalopagus) – When the condition of one twin threatens the survival of the other (complex congenital heart disease, cardiomyopathy, sepsis) – Deterioration of both twins due to hemodynamic and respiratory compromise (typically in thoracopagus twins) – Where the condition of one twin is in compatible with life (still born, anencephalic, acardiac) and the other has a good chance of survival
- 27. Anesthetic Management • Preoperative Preparation – Preoperative assessment – Preparation of the operating theatre • Anesthetic Technique • Postoperative Period
- 28. Preoperative Assessment • Classification of the twins – Identify the site of conjunction – Complexity of the conjunction – Anatomy of the organs involved and their vascular supply – Presence of other congenital anomalies – Degree of cross-circulation between the two babies
- 29. Preoperative Assessment • Size and age of the babies – Operative survival • 50% in neonatal period • 90% in infants > 4 months – Optimum time for separation is between 4 and 11 months – Earlier separation: concern about organ maturity and accuracy of investigations – Later separation: concern about the psychological status of the twins – Unless there is significant discrepancy in their sizes the weight of each baby is assessed as being half of their combined weight – Length and width should be measured to assess if they will fit onto the operating table
- 30. Preoperative Assessment • Airway anatomy – Assess • Ease of intubation • Tube placement and fixation – According to this assessment an induction and intubation plan should be made – Especially in craniopagus and thoracopagus twins: intubation has to be performed on one baby at a time.
- 31. Preoperative Assessment • Venous Access – Peripheral venous access • After prolonged hospitalization sites of venous access are often limited • Drips should not lie in the surgical field of any of the surgical specialties involved – Central venous access • The anatomy of conventional vessels used for central venous cannulation may be considerably altered • Perioperative Doppler Ultrasound may be of value
- 32. Preoperative Assessment • Cross-circulation – The degree of cross-circulation between the twins is often difficult to measure – Isotope and contrast studies may be required to identify the extent to which blood will cross from one to the other and how rapidly this will occur – Usually there is more cross- circulation in thoracopagus and craniopagus twins – Very few sets of twins have no cross-circulation
- 33. Preoperative Assessment • Blood picture – A preoperative Hb and coagulation profile is useful – Blood cross-matched should be appropriate to the anticipated blood loss for each infant – Component therapy (FFP, platelets, cryoprecipitate) should be available if necessary
- 34. Anesthetic Management • Preoperative Preparation – Preoperative assessment – Preparation of the operating theatre • Anesthetic Technique • Postoperative Period
- 35. Preparation Of Theatre • Equipment and Monitoring – All equipment need to be duplicated e.g. anesthetic machines, all monitors including invasive BP monitoring, CVP monitors, temperature probes and urinary catheters – Everything should be color-coded to match the babies including anesthetic circuits, lines and monitors
- 36. Preparation Of Theatre • Equipment and Monitoring – All facilities normally used to preserve warmth and provide heat to children in the OR should be used e.g. blood warming and warm air convection devices – Diathermy pads need to be placed on each baby but only one machine is needed until after separation – The need for cardiopulmonary bypass must be considered • In any set of thoracopagus twins • In craniopagus twins where massive blood loss is anticipated
- 37. Preparation Of Theatre • Theatre layout – Positions of the anesthetic machines relative to the operating table will be determined by the type of twins • Heads of thoracopagus twins will be at the same end of the operating table • Heads of ischiopagus twins will be usually at opposite ends – Positioning of the babies in all types of twins requires careful thought and planning in the preoperative period – Changes in positions require planning, gentle handling and appropriately placed monitors and vascular lines
- 38. Preparation Of Theatre • Plan of action – A detailed operative plan including: • The order in which surgeons will operate • Who is involved from each speciality • What position is required – Simple diagrams of the anticipated anatomy and planned surgery facilitate anesthetic planning – Control of traffic in the OR is very essential
- 39. Anesthetic Management • Preoperative Preparation – Preoperative assessment – Preparation of the operating theatre • Anesthetic Technique • Postoperative Period
- 40. Anesthetic Technique • Induction – It is preferable to have IV access at induction but this is not always feasible – Any induction technique used in children may be appropriate – It is advisable to induce anesthesia and intubate one child after the other as inducing both together may create a crowded and chaotic condition – Rapid sequence induction is not advised unless airway maintenance in both babies is possible
- 41. Anesthetic Technique • Maintenance and Monitoring – Anesthesia can be maintained in a variety of ways depending on personal preference and suitability for the type of conjunction – Regional anesthesia (e.g. caudal epidural anesthesia) is always an attractive option but may be difficult due to abnormal spinal anatomy – Monitoring: all routine monitors especially • Invasive BP monitoring: essential for all except the simplest conjunction • Temperature monitoring: loss of skin cover may be considerable
- 42. Anesthetic Technique • Bleeding – Degree of bleeding depends on • Type of conjunction • Complexity of organ anatomy • Extent of surgery performed
- 43. Anesthetic Technique • Bleeding – Degree of bleeding depends on – Aprotonin may be considered when massive blood loss is anticipated • When hearts or livers are joined • For craniopagus twins • For cardiopulmonary bypass
- 44. Anesthetic Technique • Bleeding – Degree of bleeding depends on – Aprotonin may be considered when massive blood loss is anticipated – You should assume replacement of at least 1- 2 times the estimated blood volume of each baby – Chemistry and hematology should be monitored regularly and any abnormalities corrected as necessary
- 45. Anesthetic Technique • Changing positions Requires particular attention to: – Stabilizing the tracheal tube and IV lines – Support and protect the limbs – Reestablishing color-coded lines and monitors appropriately – Freeing the diaphragm for adequate ventilation
- 46. Anesthetic Technique • Recovery – For simple procedures postoperative ventilation may not be necessary – If there is any question about adequate ventilation or stable hemodynamics it is advisable to ventilate – Factors contributing to successful recovery • Adequate bony chest wall and skin cover • Good diaphragmatic function • Minimal abdominal distension • Absence of gastro-esophageal reflux
- 47. Anesthetic Management • Preoperative Preparation – Preoperative assessment – Preparation of the operating theatre • Anesthetic Technique • Postoperative Period
- 48. Postoperative Period Problems in the immediate postoperative period • Sequelae of massive blood transfusion • Consequences of tight closure – Abnormal renal function – Respiratory compromise – Diaphragmatic dysfunction – Atelectasis and pneumonia • Temperature derangements • Neurological sequelae for patients with spinal or cerebral involvement in their conjunction
- 49. Conjoined Twins
- 50. Conjoined Twins
- 51. Conjoined Twins
- 52. Thank You