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

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

Patient 1: Khasan Diagnosis: Idiopatic dilatation cardiomyopathy, IV stage cardiac insufficiency. Background: Congenital development malformation - s Patient 2: Khusan Diagnosis: Acute respiratory infection, rhinosinusit. Background: Congenital development malformation - s Patient 1: Khasan Diagnosis: Idiopatic dilatation cardiomyopathy, IV stage cardiac insufficiency. Background: Congenital development malformation - siamese twins with 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: Khusan Diagnosis: Acute respiratory infection, rhinosinusit. Background: Congenital development malformation - siamese twins with 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.

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

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

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

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

The twins position on the operation table

Argon – plasma scalpel “Bloodless” cautery and separation

The liver separation The liver separation

Children separation

Anterior abdominal cavity plasty

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

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 ). 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.

INTENSIVE CARE DEPARTMENT Khusan

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

Postoperative period course – Operation – Stable course, subfebrile condition, anemia – pneumonia, reintubation, SpO 2 – 84-87%, sepsis, Klebsiella in blood, thrombocytopenia, leukocytosis, infected wound, respiratory distress syndome – abdominal cavity bleeding, DIVC – Peritonitis chronic course, sepsis treatment – Postoperative surgical treatment, alloplasty of anterior abdominal cavity defects Remission Transition to enteral feeding

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: periventricular leukomalacia, hypoxic-ischemic encephalopathy

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

Enteral feeding (40 day)

The skin defect alloplasty (50 postoperative day)

TWO MONTHS AFTER THE OPERATION

SIX MONTHS AFTER THE OPERATION

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?