The Republican Specialized Center of Surgery named after acad. V. Vakhidov

1. The Republican Specialized Center of Surgery named after acad. V. Vakhidov The first experience of separation the “siamese twins” in the Republic of Uzbekistan. Anesthesiological support and postoperative management

2. The Komilzhonov boys had xyloomphalophagia. Khasan (bellow in picture) and Khusan were born on 05.07.2006 in Koshkupir district, Khoresm province

3. Patient 1: Khasan Diagnosis: Idiopatic dilatation cardiomyopathy, IV stage cardiac insufficiency.Background: Congenital development malformation - siamese twinswith adhesion of the chest lower part and the anterior abdominal wall.Associated diseases: II stage hypotrophy (weight deficiency 20%). I stage infectious-alimentary anemia. Polyenzyme deficiency. Intestinal dysbacteriosis. Klebsiella carrier.Patient 2: KhusanDiagnosis: Acute respiratory infection, rhinosinusit.Background: Congenital development malformation - siamese twinswith adhesion of the chest lower part and the anterior abdominal wall.Associated diseases: III stage hypotrophy (weight deficiency 35%). I stage infectious-alimentary anemia. Polyenzyme deficiency. Intestinal dysbacteriosis. Klebsiella carrier.

4. Anatomic features • Mirror position • The chests are slightly deformed, cubic from adhesed beginning from the middle 1/3 of the sternum, but the sternums in the both twins were separate • The liver and pericardium adhesive • Presence of common vessels in the adhesive places • The diaphragm deformations, anterior sinuses absence • Dislocation of Khusan”s small intestine loops into Khasan”s abdominal cavity

5. Twins computed tomography (CT) Review CTangiography Review CT Review CT. The cardiac caul Review CTangiography. The liver adhesive

6. Anesthesiologial problems • Difficulties with monitoring • Difficulties with trachea intubation • Difficulties with the central veins catheterization • Single circulation drugs • Difficulties with dose regimen • Threat of intraoperative hypothermia

7. Preoperative estimation of children”s objective status • IV ASA class • Defined the problematic respiratory way for Khasan – IV stage Malumpate, for Khusan – III stage • Planning anesthesia – general balanced anesthesia with isoflurane in minimal flow conditions

8. The twins position on the operation table

9. Argon – plasma scalpel “Bloodless” cautery and separation

10. The liver separation

11. Children separation

12. Anterior abdominal cavity plasty

13. Operation time: In Khasan – 7 hours 55 minutes In Khusan – 9hours 50minutes Anesthesia time: In Khasan – 14hours In Khusan – 16hours Intraoperative infusion - 12 ml/kg/h Crystalloids : colloids – 1:1 (Hydroxyethyl starch: chilled plasma, albumin, clean erythrocytes 1:3:1:1) Perspiration 5ml/kg/h Diuresis 4ml/kg/h Minimal hemorrhage (50 ml) Balance + 150 ml

14. Khasan The child died on 7th daily POSTMORTAL DIAGNOSIS: Main: Xyloomphalophagia. Idiopatic dilatation cardiomyopathy. Twins state after their detachment with siamese twins state after operation fortheir detachment with synchrodal costodiaphragmatic joint and II andthe hepatic tissue II and IV segments adhesion separation (from 12.11.2006). Complication: Post hypoxemia encephalopathy. Brain edema. Acute respiratory and cardiovascular insufficiency. Background:Congenital development malformation. Siamese twins with adhesion of the chest low part and the anterior abdominal wall. Associated diagnosis: II stage hypotrophy (weight deficiency - 20%). Infectious-alimentary anemia. Polyfermental deficiency. Intestinal dysbacteriosis.

15. INTENSIVE CARE DEPARTMENTKhusan

16. Postoperative management problems • Curative – protective regimen • Respiratory therapy • Adequate antibacterial therapy • Provision with optimum nourishment • Careful control after fluid and body weight • Joined work of surgeons and intensivists

17. Postoperative period course • 11.11.06 – Operation • 11.11.06 – 21.11.06 Stable course, subfebrile condition, anemia • 21.11.06 – pneumonia, reintubation, SpO2 – 84-87%, sepsis, Klebsiella in blood, thrombocytopenia, leukocytosis, infected wound, respiratory distress syndome • 26.11.06 – abdominal cavity bleeding, DIVC • 30.11.06 – Peritonitis chronic course, sepsis treatment • 14.12.06 – Postoperative surgical treatment, alloplasty of anterior abdominal cavity defects • Remission • Transition to enteral feeding

18. Khusan”s diagnosis in the postoperative period • Main: xyloomphalopagia, II-nd from twins. Postoperative period course in Siamese twins after their detachment with synchrodal costodiaphragmatic joint and the hepatic tissue II and IV segments adhesion separation • Complications: respiratory distress syndrome, DIVC-syndrome, sepsis (Klebsiella), bilateral pneumonia, mediastinitis(?) • Associated diseases: periventricularleukomalacia, hypoxic-ischemic encephalopathy

19. Adaptation to self-dependant breathing(non-invasive CPAP,30 day after the operation)

20. Enteral feeding (40 day)

21. The skin defect alloplasty (50 postoperative day)

22. TWO MONTHS AFTER THE OPERATION

23. SIX MONTHS AFTER THE OPERATION

24. SIX MONTHS AFTER THE OPERATION

25. Questions for Discussion:1. Did we choise right the isoflurane for basis anesthesia?2. Did we choise right the solutions for infusion?3. Did the first child have chance for survise?