Coagulation changes in a siamese twin in SIRS after death of the other twin : a case study

(1)

Coagulation changes in a siamese twin in SIRS after death of the other twin: A case study

Marcin Owczarek¹ABCDEFG, Roman Kaźmirczuk¹ABDG, Andrzej Prokurat²AF, Małgorzata Chrupek²AF, Kamila Sadaj-Owczarek³ABEFG, Krzysztof Kusza¹ACDEG

1

Chair and Department of Anesthesiology and Intensive Care, Nicolaus Copernicus University, Collegium Medicum in Bydgoszcz, Bydgoszcz, Poland

2

Chair and Department of Pediatric Surgery, Nicolaus Copernicus University, Collegium Medicum in Bydgoszcz, Bydgoszcz, Poland

3

Department of Emergency Medicine, Nicolaus Copernicus University, Collegium Medicum in Bydgoszcz, Bydgoszcz, Poland

Source of support: Departmental sources

Summary

Background:

Conjoined twins represent a rare case of embryonic failure. Siamese twins’ final outcome is usually associated with poor prognosis due to complications, among which the inflammatory and sep- tic disturbances are often present.

Case Report:

The article describes Siamese twins of craniopagus type. One of the twins died of heart and aortic failure after 48 hours of life. The decision regarding surgical separation then became an emergency. At the same time laboratory results for the surviving twin I indicated steadily worsening coagulation functions and processes, which we believed were caused by the death of twin II, as well as by systemic inflammatory response syndrome (SIRS).

Conclusions:

Due to vascular conjunction between the twins’ brains’ circulatory systems the sequenced progress of coagulopathy was noticed in the surviving twin.

key words: Siamese Twins • systemic inflammatory response syndrome (SIRS) • coagulopathy

Full-text PDF: http://www.medscimonit.com/abstract/index/idArt/878297 Word count: 1491

Tables: 5 Figures: 2 References: 16

Author’s address: Marcin Owczarek, Chair and Department of Anesthesiology and Intensive Care, Nicolaus Copernicus University in Toruń, Ludwik Rydygier Collegium Medicum, M. Skłodowskiej-Curie 9 Str., 85-095 Bydgoszcz, Poland, e-mail: owczarekmarcin@o2.pl

Authors’ Contribution:

A Study Design B Data Collection C Statistical Analysis D Data Interpretation E Manuscript Preparation F Literature Search G Funds Collection Received: 2009.04.17 Accepted: 2009.06.15 Published: 2010.01.01

Case Study

PMID: 20037495

CS

(2)

B

ackground

Conjoined twins represent a rare case of incomplete embryonic separation [1]. The incidence of conjoined twins is reported as being from 1 in 50,000 to 1 in 100,000 births.

[2] The anatomical features of Siamese twin conjunctions are various and complex. Eight types are generally recognized [3,4]. Of these, 5% are comprised of the craniopagus type [5], occurring in 0.6 cases per one million births [6].

The anatomical conjunctions between Siamese twins are often accompanied by various congenital abnormalities in other organs, not only at the place of junction. Many of these abnormalities do not give hope for a successful separation.

The enormous deformations present in many conjoined twins also provide justification for the parents to forbid even the attempt to separate the twins. In Al Rabeeah’s meta-analysis such twins are designated “the nonoperated cases” [2].

In operative cases the survival rate depends on the com- plexity of junction and the extent of coexisting congenital failures in other organs. Votteler and Lipsky (2005) described 10 pairs of conjoined twins successfully separated, with 14 surviving, but craniopagi did not occur among these pairs. Among these 10 pairs, 6 emergency separa- tions were performed because of the death or impending death of 1 of the twins [7]. Winston et al. (1987), on the other hand, discovered 30 cases of craniopagi with 31 surviving twins [8].

Several factors affect the survival outcome of Siamese twins:

proper preoperative evaluation (including clinical typing and three-dimensional imaging), and most importantly, the achieved maturity and age of the Siamese twins [6,9].

c

ase

d

escription

Male Siamese twins, craniopagus type, were born premature- ly by C-section at the 33rd week of fetal life. Weight of twin I was 1700 g, and of twin II 1650 g. In the first minute after birth the twins had an Apgar score of 7. Twin I was conjoined with twin II at the top of the head, while twin II was conjoined with twin I at the forehead and top of the head, his facial skeleton compressed. The conjunction area was approximately 10 cm². Both twins were ventilated support- ively with n-CPAP.

In the 30^th hour of life Twin II’s condition worsened. Because of respiratory dysfunction the patient was intubated and connected to a respirator.

Echocardiographic examination revealed a pathologic aortal arch. Coarctation or interruption of the aortal arch was suspected; therefore the patient was given intravenous infusion of alprostadil, which resulted in transient clinical stabilization.

At the 45^th hour of life twin II’s condition dramatically worsened. After an hour and a half of resuscitation, he died.

Postmortem examination revealed that death was caused by severe heart failure and aortic coarctation.

The surviving twin was admitted to our pediatric intensive care unit soon after twin II’s death, at the 49^th hour after

birth, when immediate separation was of great urgency. He was still conjoined with deceased twin II. His skin and mu- cous membranes were pale, and the anterior fontanel was slightly depressed. The patient needed infusion of dobuta- mine at doseage 10 mcg/kg/min.

Figure 1 shows twin I conjoined with the deceased twin II after admission to the pediatric intensive care unit and Figure 2 shows CT preoperative imaging of the relation of the twin’s heads; specifically, connections between the vascular systems of both twins’ brains in the area of sagittal sinus with separate nervous systems of the twins on the level of sagittal sinus, while the brains were separated with meninx.

Tables 1, 2, and 3 show the first twin’s hemodynamic and biochemical parameters upon admission to our center and later.

After admission to the ward, the surviving twin was re-intubated, and biphasic mechanical ventilation was applied.

Figure 1. The twins after admission to our Center.

Figure 2. Two-dimensional contrast CT imaging at the place of

conjunction before separation.

(3)

Although the patient had mechanical ventilation, no clinical or radiologic lung damage indicators were present. The patient was treated with empirical wide–spectrum antibiotic therapy (meropenem, vancomycin, fluconazol).

Blood, pharyngeal and anal swabs were sent for microbiological examination. They did not reveal the presence of any pathogens in the samples.

Echocardiographic examination of the heart-vascular system of twin I did not reveal any abnormalities.

Coagulation plasma tests taken 2 times before separation, at the 2^nd and 5^th hours after twin II’s death. They showed significant deviations from normal condition in the coagulation system. Tables 4 and 5 show the dynamics of these changes.

The separation surgery took place at the 5^th hour after the death of the twin II.

Prior to surgery, circulating blood volume and coagulation conditions were leveled by the application of crystalloid

At admission and 5

hours before surgery 2 hours

after surgery 24 hours

after admission 2 days after admission

SBP (systolic blood pressure) [mmHg] 68 82 73 74

DBP (diastolic blood pressure) [mmHg] 55 52 47 52

HR (heart rate) [1/min.] 202 179 152 141

External body Temperature [˚C] 36.4 36.3 36.4 36.7

CVS (Central venous pressure) [mmHg] 8 7 3

SpO2 (Pulsoxymetry) [%] 94% 100% 98% 98%

Table 1. Hemodynamic and clinical parameters measured in first twin at admission to our ward and after surgery.

On admission 1 day after admission and surgery 2 days after admission and surgery

Glucose 404 mg/dl 124 mg/dl

Creatynine 0.9 mg/dl 0.85 0.91 mg/dl

BUN 23.2 mg/dl 18.3 mg/dl 16 mg/dl

Total Bilurbin 7.24 mg/dl 7.16 mg/dl 7.73 mg/dl

Direct Bilurbin 0.45 mg/dl 0.38 mg/dl 0.36 mg/dl

LDH 612 U/l 266 U/l 254 U/l

C-reactive protein <3.19 mg/l <5.0 mg/l

Table 2. Selected biochemical parameters in first twin’s plasma measured in order of sequenced days after admission and separation surgery in our hospital.

At admission 4 hours after admission

and 1 hour before surgery 1 hour

after surgery 20 hours

after surgery

pH 7.22 7.384 7.348 7.485

pCO2 53.3 mmHg 23.7 mmHg 33.9 mmHg 20.3 mmHg

pO2 143.7 mmHg 53.3 mmHg 86.7 mmHg 63.7 mmHg

HCO3 17.0 mmol/l 13.8 mmol/l 19.3 mmol/l 22.7 mmol/l

BE –12.6 mmol/l –9.7 mmol/l –6.4 mmol/l –3.1 mmol/l

AnGap 26.2 mmol/l 19.2 mmol/l 24.2 mmol/l 17.7 mmol/l

Lactate 6.00 mmol/l 7.03 mmol/l 7.90 mmol/l 2.54 mmol/l

Table 3. Capillary blood gas samples.

CS

(4)

fluids, 5% albumin, fresh frozen plasma, erythrocyte concentrate and platelets concentrate.

In addition, massive intraoperative blood loss occurred during surgery. Again, administration of an intraoperative trans- fusion of crystalloid, erythrocytes concentrate, cryoprecip- itate, fresh frozen plasma, albumins, and antithrombin III was given: all in an amount approximately 4-fold that of circulating blood volume.

Finally, during the operation incidental circulatory arrest occurred due to hypovolemia. This complication was con- trolled and hemodynamic circulatory function was restored.

After surgery the patient was supported with substitute res- piration and catecholamines and sent to the pediatric intensive care unit.

Subsequently the patient’s condition systematically improved.

On the 5^th day catecholamines were withdrawn, and on the 11^th day the patient was extubated.

d

iscussion

Few sepsis cases in conjoined twins have been reported in the literature. Kato et al. (2006) reported thoracopagi that died of sepsis [10]. In their historical review of various types of conjoints, Winder and Law (2006) includ- ed a report on 2 pyopagus conjoints. The first involved the original “Siamese twins” (the twins to whom the term

“Siamese twins” was first applied), the brothers Chang and

Eng Bunker (1811–1874). Chang died of pneumonia and within a few hours Eng died.

In the second case, involving the well-known Blazinek Siamese sisters (born in 1878) of Bohemia, Josepha died at the age of 49 from cholecystitis with concomitant bron- chopneumonia and her twin, Rosa, died approximately 12 minutes later [1].

Sung et al. (1979) reported the case of thoracopagi who de- veloped systemic candidiasis, which was treated successfully with miconazole [11]. Thus no exact data are available on how sepsis influences the mortality of Siamese twins;

nevertheless, sepsis is recognized as a significant morbidi- ty and mortality factor in hospitalized premature neonates and infants. [12]

Like the cases cited earlier, surgical separation of twins in this report arose as a serious emergency because of the death of twin II. The emergency became apparent in the 5th hour after the death of twin II. We did not prove the presence of infectious pathogens in the biological samples taken from the surviving twin.

Directly after admission to our ward and before separation surgery began, we noticed the steady decline of clinical state and deterioration of coagulation parameters in the living brother. Despite the therapy given to the patient, the coagulation system was destabilized. Coagulation findings re- sembled disseminated intravascular coagulation in the 4^th hour after the death of twin II. It is thus highly probable

Time lapse since

Twin II’s death 2 hours 4 hours 8 hours 14 hours 24 hours

WBC [10³/ml] 3.63 3.95 2.14 2.08 3.41

RBC [1000000/ml] 4.14 2.32 3.26 4.15 4.58

Hgb [g/dl] 15.2 8.3 10.0 11.6 13.0

Hct [%] 44.0 25.5 27.8 33.4 36.7

PLT [10³/ml] 21 51 35 179 216

Table 4. Changes in platelet count and blood morphology in the plasma of the surviving twin, observed after twin II’s death.

Time lapse since

Twin II’s death 2 hours 5 hours 14 hours 24 hours 48 hours

Prothrombin Time [sec] 18.0 29.9 14.2 12.9 14.0

Prothrobine index [%] 66% 40% 84% 92% 85%

INR 1.49 2.43 1.19 1.08 1.17

APTT [sec.] 79.5 166.5 46.9 42.0 34.3

Fibrinogen [g/L] 0.77 0.49 1.81 2.42 2.55

Anti-Thrombin III concentration [%] 26.85 23.46 52.61 54.94 41.88

D-dimers [mcg/l] 768 4300 142 222 429

Table 5. Changes in the coagulation parameters in the plasma of the surviving twin, observed after Twin II’s death.

(5)

that coagulation changes in the living twin were caused by an inflammatory cascade triggered by the deceased twin.

Inflammation, coagulation and impaired fibrynolysis, pre- viously described to co-occur as a sepsis triad, constitute an important mechanism in the pathophysiology of sepsis [12].

Due to conjunction with the deceased twin, twin I devel- oped 2 major criteria significant for SIRS: leucopenia and tachycardia. This meets the criteria of SIRS [12]. The expanding inflammatory process affected circulatory, respiratory and coagulology systems.

Low plasma concentration of C-reactive protein in the surviving twin suggests a non-infective origin of the inflammatory process. C-reactive protein is usually a good differenti- ation marker for true bacteriemia [15].

Microbiological cultures of blood or other specimens are frequently used to distinguish infection from non-infectious conditions of expanding process [16].

Changes in coagulation are important criteria in the diag- nosis of sepsis. They also comprise a diagnostic element in the general inflammatory reaction of the patient and in multiorgan dysfunction syndrome.

Neonates and premature infants are naturally predisposed to hypercoagulability [14].

The coagulation parameters of the patient, measured before surgery, were consistent with the coagulative criteria of systematic inflammatory response syndrome and disseminated intravascular coagulation.

Moreover, Liu and Zhonghua (2004) demonstrated a link between systematic inflammatory response syndrome and coagulopathy [13].

Current findings suggest that although neonates and infants are immature, the pathophisiology of the coagulative dysfunction in SIRS and in sepsis is similar to that in adults.

Thus, despite physiological differences in the maturity of immunologic and hematologic systems, the inflammatory, coagulative and impaired fibrynolytic processes are similar in adults and infants [12].

It is noteworthy that the coagulation parameters began to ap- proach abnormal values immediately after the death of twin II, while the extensiveness of the changes was due to the anatomic conjunction between the twins’ circulatory systems.

Because of vascular junctions at sagital sinus – revealed in the tomography of the twins’ heads – necrotic processes in the deceased twin provoked massive inflammatory response in the living twin.

c

onclusions

1. The craniopagi twins present a rare case of incomplete embryonic separation.

2. The death of one of conjoined twins may result in severe inflammatory response accompanied by impending coagulopathy.

r

eferences

:

1. Winder M, Law A: Separation of pyopagus conjoined twins: A New Zealand neurosurgical experience. J Clin Neurosci, 2006; 13(9): 968–75 2. Al Rabeeah A. Conjoined twins – past, present, future. J Pediatr Surg,

2006; 41: 1000–4

3. Spencer R: Anatomic description of conjoined twins: A plea for stan- dardized terminology. J Pediatr Surg, 1996; 31: 941–44

4. Spencer R: Theoretical and analytical embryology of conjoined twins.

Part I. Embryogenesis. Clin Anat, 2000; 13: 36–53 5. Spitz L, Kiely E: Conjoined twins. JAMA, 2003; 289: 1307–10 6. Khan Zahid H, Seyed A, Saberi H: Anesthetic and surgical experience

in a case of total vertical craniopagus. Surg Neurol, 1995; 5: 62–67 7. Votteler TP, Lipsky K: Long-term results of 10 conjoined twin separa-

tions. J Pediatr Surg, 2005; 40: 618–29

8. Winston KR, Rockoff MA, Mulliken JB et al: Surgical division of craniopagi. Neurosurgery. 1987; 21(6): 782–91

9. Campbell S: Separation of craniopagus twins: The Brisbane experience.

Childs Nerv Syst, 2004; 20(8–9): 601–6

10. Kato T, Yoshino H, Hebiguchi T, Koyama K: Experience with treatment of three pairs of conjoined twins. J Clin Neurosci, 2006; 13: 968–75 11. Sung JP, Rajani K, Chopra DR et al: Miconazole therapy for systemic

candidiasis in a conjoined (Siamese) twin and a premature newborn.

Am J Surg, 1979; 138(5): 688–91

12. Mary A: Linking the sepsis triad of inflammation, coagulation, and su- pressed fibrynolysis to infants. Advances in Neonatal Care, 2004; 4(5):

258–73

13. Liu YG, Li CS: Coagulation dysfunctions in patients with systemic inflammatory response syndrome. [Article in Chinese] Zhonghua Nei Ke Za Zhi [Chinese Journal of Internal Medicine], 2004; 43(2): 94–97 14. Goldstein B: International pediatric sepsis consensus conference:

Definitions for sepsis and organ dysfunction in pediatrics. International Consensus Conference on Pediatric Sepsis. Pediatr Crit Care Med., 2005;

6(1): 2–8

15. Shaoul R, Lahad A, Tamir A et al: C Reactive Protein (CRP) as a pre- dictor for true bacteremia in children. Med Sci Monit, 2008; 14(5):

CR255–61

16. Schultz MJ, Determann RM: PCT and sTREM-1: The markers of infection in critically ill patients? Med Sci Monit, 2008; 14(12): RA241–47