The first experience of separation the The first experience of separation the ““siamese siamese

twinstwins” in the Republic of Uzbekistan. ” in the Republic of Uzbekistan.

Anesthesiological support and postoperative Anesthesiological support and postoperative

managementmanagement

The first experience of separation the The first experience of separation the ““siamese siamese

twinstwins” in the Republic of Uzbekistan. ” in the Republic of Uzbekistan.

Anesthesiological support and postoperative Anesthesiological support and postoperative

managementmanagement

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

The Komilzhonov boys The Komilzhonov boys hadhad xyloomphalophagia. Khasan xyloomphalophagia. Khasan ((bellow in picturebellow in picture) and Khusan were born on 05.07.2006 in ) and Khusan were born on 05.07.2006 in

Koshkupir district, Khoresm provinceKoshkupir district, Khoresm province

Patient 1: Khasan Patient 1: Khasan DiagnosisDiagnosis: Idiopatic dilatation cardiomyopathy, IV stage cardiac : Idiopatic dilatation cardiomyopathy, IV stage cardiac insufficiency.insufficiency.Background:Background: Congenital development malformation Congenital development malformation - - ssiamese 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: KhusanPatient 2: Khusan DiagnosisDiagnosis: Acute respiratory infection, rhinosinusit: Acute respiratory infection, rhinosinusit.. Background:Background: Congenital development malformation Congenital development malformation - - ssiamese 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 featuresAnatomic features

Mirror positionMirror positionThe chests are slightly deformed, The chests are slightly deformed, cubic from cubic from adhesed beginning from the middle 1adhesed beginning from the middle 1/3 of the /3 of the sternum, but the sternums in the both twins were sternum, but the sternums in the both twins were separateseparateThe liver and pericardium adhesiveThe liver and pericardium adhesivePresence of common vessels in the adhesive placesPresence of common vessels in the adhesive placesThe diaphragm deformations, anterior sinuses The diaphragm deformations, anterior sinuses absenceabsenceDislocation of Khusan”s small intestine loops into Dislocation of Khusan”s small intestine loops into Khasan”s abdominal cavityKhasan”s abdominal cavity

Twins computed tomography (CT)Twins computed tomography (CT)

Review CT Review CT angiography

Review CT angiography. The liver adhesive

Review CT. The cardiac caul

Anesthesiologial problemsAnesthesiologial problems

Difficulties with monitoringDifficulties with monitoring

Difficulties with trachea intubationDifficulties with trachea intubation

Difficulties with the central veins Difficulties with the central veins catheterizationcatheterization

Single circulation drugsSingle circulation drugs

Difficulties with dose regimenDifficulties with dose regimen

Threat of intraoperative hypothermiaThreat of intraoperative hypothermia

Preoperative estimation of Preoperative estimation of children”s objective statuschildren”s objective status

IV ASA class IV ASA class

Defined the problematic respiratory way Defined the problematic respiratory way for Khasan – IV stage Malumpate, for for Khasan – IV stage Malumpate, for Khusan – III stageKhusan – III stage

Planning anesthesia – general balanced Planning anesthesia – general balanced anesthesia with isoflurane in minianesthesia with isoflurane in minimal flow mal flow conditionsconditions

The twins position on the operation table

Argon – plasma scalpel Argon – plasma scalpel “Bloodless” cautery and “Bloodless” cautery and

separationseparation

The liver separationThe liver separation

Children separationChildren separation

Anterior abdominal cavity plastyAnterior abdominal cavity plasty

Operation time:Operation time:In KhasanIn Khasan – 7 – 7 hourshours 55 55 minutesminutesIn KhusanIn Khusan – – 99 hourshours 5 500 minutesminutes Anesthesia time:Anesthesia time:In KhasanIn Khasan – – 1414 hourshoursIn KhusanIn Khusan – – 1616 hourshours Intraoperative infusion - Intraoperative infusion - 12 12 mlml//kgkg//hh Crystalloids Crystalloids : : colloidscolloids – 1:1 – 1:1((Hydroxyethyl starchHydroxyethyl starch: : chilled plasma, albumin, clean chilled plasma, albumin, clean

erythrocyteserythrocytes 1:3:1:1) 1:3:1:1)Perspiration 5Perspiration 5 mlml//kgkg//h h Diuresis 4Diuresis 4 mlml//kgkg//h h Minimal hemorrhageMinimal hemorrhage (50 (50 mlml) ) BalanceBalance + 150 + 150 mlml

KhasanKhasanThe child died on 7th dailyThe child died on 7th dailyPOSTMORTAL DIAGNOSISPOSTMORTAL DIAGNOSIS: : MainMain: : Xyloomphalophagia. Idiopatic dilatation Xyloomphalophagia. Idiopatic dilatation

cardiomyopathycardiomyopathy. . Twins state after their detachment with Twins state after their detachment with siamese twins state after operation fortheir detachment siamese twins state after operation fortheir detachment with synchrodal costodiaphragmatic joint and II andthe with synchrodal costodiaphragmatic joint and II andthe hepatic tissue II and IV segments adhesion separation hepatic tissue II and IV segments adhesion separation ((fromfrom 12.11.2006). 12.11.2006).

ComplicationComplication: : Post hypoxemia encephalopathy. Brain edema. Post hypoxemia encephalopathy. Brain edema. Acute respiratory and cardiovascular insufficiencyAcute respiratory and cardiovascular insufficiency. .

Background:Background: Congenital development malformation. Siamese Congenital development malformation. Siamese twins with adhesion of the chest low part and the anterior twins with adhesion of the chest low part and the anterior abdominal wallabdominal wall..

Associated diagnosisAssociated diagnosis: : II stage hypotrophyII stage hypotrophy ( (weight deficiency weight deficiency - - 20%). 20%). Infectious-alimentary anemia. Polyfermental Infectious-alimentary anemia. Polyfermental deficiency. Intestinal dysbacteriosisdeficiency. Intestinal dysbacteriosis..

INTENSIVE CARE INTENSIVE CARE DEPARTMENTDEPARTMENT

KhusanKhusan

Postoperative management Postoperative management problemsproblems

Curative – protective regimenCurative – protective regimen

Respiratory therapyRespiratory therapy

Adequate antibacterial therapyAdequate antibacterial therapy

Provision with optimum nourishmentProvision with optimum nourishment

Careful control after fluid and body weightCareful control after fluid and body weight

Joined work of surgeons and intensivistsJoined work of surgeons and intensivists

Postoperative period coursePostoperative period course

11.11.06 – 11.11.06 – OperationOperation1111.11..11.0606 – – 21.11.06 Stable course, subfebrile 21.11.06 Stable course, subfebrile condition, anemiacondition, anemia 21.11.06 – 21.11.06 – pneumonia, reintubation, pneumonia, reintubation, SpOSpO22 – 84-87% – 84-87%, , sepsis, Klebsiella in blood, thrombocytopenia, sepsis, Klebsiella in blood, thrombocytopenia, leukocytosis, infected woundleukocytosis, infected wound, , respiratory distress respiratory distress syndomesyndome26.11.06 – 26.11.06 – abdominal cavity bleeding, DIVCabdominal cavity bleeding, DIVC 30.11.06 – 30.11.06 – Peritonitis chronic course, sepsis treatmentPeritonitis chronic course, sepsis treatment14.12.06 – 14.12.06 – Postoperative surgical treatment, alloplasty Postoperative surgical treatment, alloplasty of anterior abdominal cavity defectsof anterior abdominal cavity defectsRemissionRemissionTransition to enteral feedingTransition to enteral feeding

Khusan”s diagnosis in the Khusan”s diagnosis in the postoperative periodpostoperative period

Main: xyloomphalopagia, II-nd from twins. Main: xyloomphalopagia, II-nd from twins. Postoperative period course in Siamese twins Postoperative period course in Siamese twins after their detachment with synchrodal after their detachment with synchrodal costodiaphragmatic joint and the hepatic tissue II costodiaphragmatic joint and the hepatic tissue II and IV segments adhesion separationand IV segments adhesion separationComplicationsComplications: : respiratory distress syndromerespiratory distress syndrome, , DIVC-syndrome, sepsis (Klebsiella), bilateral DIVC-syndrome, sepsis (Klebsiella), bilateral pneumonia, mediastinitispneumonia, mediastinitis(?)(?)Associated diseasesAssociated diseases: : periventricularperiventricular leukomalacia, hypoxic-ischemic encephalopathyleukomalacia, hypoxic-ischemic encephalopathy

Adaptation to self-dependant Adaptation to self-dependant breathingbreathing ((non-invasive non-invasive CPAPCPAP,30 ,30 day day

after the operationafter the operation))

Enteral feeding (Enteral feeding (4040 day) day)

The skin defect alloplasty The skin defect alloplasty (50(50 postoperative day postoperative day))

TWO MONTHS AFTER THE TWO MONTHS AFTER THE OPERATIONOPERATION

SIX MONTHS AFTER THE SIX MONTHS AFTER THE OPERATIONOPERATION

SIX MONTHS AFTER THE SIX MONTHS AFTER THE OPERATIONOPERATION

Questions for Discussion:Questions for Discussion:1. Did we choise right the 1. Did we choise right the

isoflurane for basis isoflurane for basis anesthesia?anesthesia?

2. Did we choise right the 2. Did we choise right the solutions for infusion?solutions for infusion?

3. Did the first child have 3. Did the first child have chance for survise?chance for survise?