• Users Online: 364
  • Home
  • Print this page
  • Email this page
Home About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Login 
  Navigate here 
 Resource links
 »  Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 »  Article in PDF (873 KB)
 »  Citation Manager
 »  Access Statistics
 »  Reader Comments
 »  Email Alert *
 »  Add to My List *
* Registration required (free)  

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded222    
    Comments [Add]    

Recommend this journal


  Table of Contents 
Year : 2020  |  Volume : 21  |  Issue : 1  |  Page : 66-69

An adolescent with large extradural hematoma and undiagnosed congenital cyanotic heart disease at wee hours: An anesthetist's nightmare

1 Department of Anaesthesiology and Critical Care, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
2 Department of Paediatrics, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

Date of Submission09-Jul-2019
Date of Decision24-Aug-2019
Date of Acceptance25-Aug-2019
Date of Web Publication13-Feb-2020

Correspondence Address:
Dr. Amiya Kumar Barik
Department of Anaesthesiology and Critical Care, All India Institute of Medical Sciences, Rishikesh . 249 203, Uttarakhand
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/TheIAForum.TheIAForum_49_19

Rights and Permissions


Extradural hematoma (EDH) is a life-threatening emergency, which needs emergent evacuation to prevent mortality and morbidity. We report a case of an adolescent, who had large fronto-temporo-parietal EDH, with significant midline shift, and was incidentally diagnosed to have congenital cyanotic heart disease with deranged coagulation profile in the preoperative area using point-of-care ultrasound.

Keywords: Adolescent, congenital cyanotic heart disease, extradural hematoma, point of care ultrasound, tetralogy of fallot

How to cite this article:
Gupta P, Rahul J S, Barik AK, Gupta G. An adolescent with large extradural hematoma and undiagnosed congenital cyanotic heart disease at wee hours: An anesthetist's nightmare. Indian Anaesth Forum 2020;21:66-9

How to cite this URL:
Gupta P, Rahul J S, Barik AK, Gupta G. An adolescent with large extradural hematoma and undiagnosed congenital cyanotic heart disease at wee hours: An anesthetist's nightmare. Indian Anaesth Forum [serial online] 2020 [cited 2021 Sep 25];21:66-9. Available from: http://www.theiaforum.org/text.asp?2020/21/1/66/278182

  Introduction Top

Extradural hematoma (EDH) accounts for 1%–4% of all head injuries and about 15% of all fatal head trauma,[1] with mortality varying from 10% to 40%.[2] This rare case report describes the anesthetic challenges and successful management of EDH with associated uncorrected tetralogy of fallot (TOF) and the importance of point-of-care ultrasound (POCUS) in perioperative anesthetic evaluation.

  Case Report Top

A 14-year-old female patient (weight – 35 kg and height – 132 cm) posted for emergency evacuation of a right-sided fronto-temporo-parietal EDH following traumatic head injury under the American Society of Anesthesiologists (ASA) class VE [Figure 1]. Her Glasgow Coma Scale (GCS) was-E2V2M5 with dilated sluggishly reactive right pupil. She had central cyanosis and clubbing in fingers and toes. Her mother gave the history of multiple instances of bluish discoloration while crying or playing, and she was also diagnosed to have “some heart problem” for which surgery was advised. However, any notes/reports pertaining to this history were unavailable. Her baseline SPO2 was 65%, which was refractory to supplemental oxygen. On auscultation, S1 and S2 were normally heard with a systolic murmur in the parasternal area. Cardiology consultation could not be made due to the emergent nature of the surgery; rather a preliminary echocardiography was performed by the anesthetists on duty using POCUS, which revealed an ejection fraction of ~60% with a 10–12 mm ventricular septal defect (VSD) with apparent right to left shunting in the apical 4-chamber view along with significantly narrowed right ventricular chamber and right ventricular outflow tract [Figure 2]. The right ventricular wall was significantly hypertrophied and visibly thicker than the left ventricle wall. The findings were consistent with TOF. Baseline blood investigations were Hb – 24 g/dl, platelet – 2.4 lakhs/mm3, and INR – 1.7. ABG on room air revealed a PH of 7.383, PaCO2 of 32.0 mm Hg, and PaO2 of 35.9 mm Hg.
Figure 1: Noncontrast computed tomography scan head showing large extradural hematoma in the right fronto-temporo-parietal region

Click here to view
Figure 2: Echocardiography showing ventricular septal defect with right to left shunting in the apical 4-chamber view with narrowed right ventricular chamber and right ventricular outflow tract obstruction

Click here to view

After shifting to the operating room, standard ASA monitors including electrocardiographic (ECG), NIBP, SPO2, and temperature probe were attached, a 20 G intravenous (IV) cannula was secured, and 0.9% normal saline was infused for preloading @ 4 ml/kg/h. Ceftriaxone 50 mg/kg was given as prophylaxis. Baseline hemodynamic parameters were heart rate – 95 beats/min, NIBP – 115/64 mm Hg, and SPO2 of 64% despite oxygen supplementation. Arterial cannulation was performed in nondominant hand using local anesthesia under strict asepsis. The patient was preoxygenated with 100% oxygen for 3 min and induced with IV fentanyl 3 μg/kg, ketofol 0.3 ml/kg at 1:4 ratios (10 mg ketamine with 40 mg propofol) and 0.1 mg/kg of vecuronium. Lignocaine 1.5 mg/kg was given to blunt the sympathetic response and trachea was intubated with cuffed endotracheal tube 6.5 mm. Two units of fresh frozen plasma were transfused for optimization of INR following induction. All IV lines were kept free of air bubbles. Anesthesia was maintained with sevoflurane, 60:40 O2: Air (MAC 0.7–1.0). A slow bolus of 6 mg morphine was administered. Ultrasound-guided central venous cannulation performed in the right internal jugular vein under strict asepsis. Phenylephrine infusion at 20–50 μg/kg/h was started to decrease the right to left shunting and which in turn would promote the right ventricular outflow and lung perfusion. As the lung perfusion was severely poor and end-tidal carbon dioxide could not be used as a yardstick to guide ventilation, patient was ventilated purely based on weight (VT - 8 ml/kg, RR - 16/min) to maintain PaCO2 between 30 and 35 mm Hg. At no point of the surgery did the surgeon complain of any brain bulge needing intervention. A midline craniotomy was performed, and about 150 ml of blood clot was evacuated with total blood loss around 400–600 ml. One unit of packed red blood cell was transfused after achieving hemostasis. The patient was hemodynamically stable throughout the procedure. After successful surgery, the patient was shifted to the intensive care unit (ICU) for elective ventilation and further management. Overnight phenylephrine (10–20 μg/kg/h) and fentanyl (1 μg/kg/h) infusion was continued. Hypovolemia, hypoxemia, hypo/hypercapnia, hypo/hyperthermia, hypo/hyperglycemia, and other metabolic derangements were avoided throughout the intra- and post-operative period. Multimodal analgesia (paracetamol 500 mg 6 hourly and tramadol 50 mg 8 hourly) was given throughout the intra- and post-operative period for adequate pain control. The trachea was extubated on postoperative day 1 after satisfactory neurological recovery. The patient was shifted from ICU on day 2 of surgery with GCS-E3V4M6. Later, on cardiology consultation, the diagnosis of TOF with right to left shunt was confirmed. Parents were counseled for the corrective surgery.

  Discussion Top

TOF accounts for 7%–10% of all congenital cyanotic heart disease (CCHD) worldwide, affecting 3–5 of every 10,000 live births with equal distribution in male and female population.[3] However, in India, TOF accounts for 16% of all CCHD.[4] The basic morphological anomaly in TOF is anterior-cephalic deviation of the muscular portion of the ventricular septum, leading to four classical components: VSD, right ventricular outflow tract obstruction (RVOTO), concentric right ventricular hypertrophy, and an overriding of aorta. The degree of RVOTO determines the severity of TOF.

The management of EDH with TOF becomes more challenging due to the involvement of two major organ systems. EDH can affect the cardiovascular system by causing catecholamine surge and Cushing's reflex characterized by hypertension, bradycardia, and irregular respiration. On the other hand, uncorrected TOF itself can cause ischemic brain injury and brain abscess. The anesthetic goals for TOF is to maintain or increase the systemic vascular resistance (SVR), minimize pulmonary vascular resistance (PVR), and prevent hyper cyanotic spells intraoperatively.[5] The patient was induced with IV fentanyl (3 μg/kg) and ketofol for better hemodynamic stability.[6] Although no anesthetic agent is absolutely contraindicated in CCHD patient, keeping risk-benefit ratio in mind, IV ketamine is the preferred inducing agent for TOF as it causes minimal myocardial depression and maintains the SVR.[7] However in patients with hypoxia and hypercarbia there is a theoretical risk of infundibular spasm and increase in right to left shunt with ketamine. However, this effect is insignificant in adequately oxygenated patient.[8] Ketamine is an N-methyl-D-aspartate receptor antagonist and so may provide neuroprotection, but inducing dose of ketamine can cause acute rise in intracranial pressure which can be detrimental in this patient with EDH with mass effect. Hence, propofol was added to ketamine to neutralize its adverse effects and better hemodynamic stability. Other agents preferred for induction are etomidate, ketomidate, and inhalational agents. However, etomidate was unavailable with us, and it was decided not to waste valuable time in arranging the drug.

In a patient with TOF tachycardia, dehydration and sympathetic stimulation must be avoided, as it can cause spasm of hypertrophied pulmonary infundibulum, leading to the development of hypercyanotic or tet spell, characterized by a rapidly falling SPO2 in response to stressful stimulation with hypotension and ischemic ECG changes. The exact pathophysiology of hypercyanotic spell is unknown, but the possible mechanism could be due to pulmonary infundibular spasm leading to worsening RVOTO and increased right to left shunt. The management of hypercyanotic spell under general anaesthesia includes, reconfirming the position of endotracheal tube, increasing fraction of inspired oxygen to 100 %, deepening the plane of anaesthesia, analgesia (fentanyl 1 mcg/kg), giving additional bolus of fluid (10-20 ml/kg), administering phenylephrine (1 mcg/kg bolus followed by 5-10 mcg/kg infusion) and esmolol (25 mcg/kg/min). Thus, phenylephrine infusion was continued throughout intra- and postoperative period in this patient to prevent hypercyanotic spell by increasing SVR.[9] Normothermia should be maintained in CCHD patients with polycythemia because hypothermia can lead to metabolic acidosis, red blood cell sludging, organ infarct, leftward shift of the oxyhemoglobin dissociation curve with decreased oxygen delivery to tissue, platelet dysfunction leading to excess surgical bleeding and prolonged duration of action of neuromuscular blocking agents.[8] Adequate multimodal analgesia should be provided, as pain can cause catecholamine release which can lead to hemodynamic instability and hypercyanotic spell.

To the best of our knowledge, this is the first reported case of an adolescent patient presenting with acute EDH with co-existing CCHD in the form of TOF. This report also emphasizes on incorporating the basic level of training regarding POCUS for the anesthesiology trainees. Thus, a real-time hemodynamics, immediate diagnostic capability, and decision-making may be ensured, particularly in an emergent and life-threatening situation.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Kumar CS, Prasad KS, Rajasekhar B, Raman BV. A study on various clinical presentations of extradural hemorrhage, factors affecting treatment and early outcome. Int J Res Med Sci 2017;5:1288-93.  Back to cited text no. 1
Babu MI, Bhasin SK, Kumar A. Extradural hematoma – An experience of 300 cases. JK Sci 2005;7:205-7.  Back to cited text no. 2
Diaz-Frias J, Guillaume M. Tetralogy of Fallot. StatPearls. Treasure Island (FL): StatPearls Publishing; 2019.  Back to cited text no. 3
Bhardwaj R, Rai SK, Yadav AK, Lakhotia S, Agrawal D, Kumar A, et al. Epidemiology of congenital heart disease in India. Congenit Heart Dis 2015;10:437-46.  Back to cited text no. 4
Menghraj SJ. Anaesthetic considerations in children with congenital heart disease undergoing non-cardiac surgery. Indian J Anaesth 2012;56:491-5.  Back to cited text no. 5
Morse Z, Sano K, Kanri T. Effects of a propofol – Ketamine admixture in human volunteers. Pac Health Dialog 2003;10:51-4.  Back to cited text no. 6
Walker A, Stokes M, Moriarty A. Anesthesia for major general surgery in neonates with complex cardiac defects. Paediatr Anaesth 2009;19:119-25.  Back to cited text no. 7
Mohindra R, Beebe DS, Belani KG. Anaesthetic management of patients with congenital heart disease presenting for non-cardiac surgery. Ann Card Anaesth 2002;5:15-24.  Back to cited text no. 8
[PUBMED]  [Full text]  
Dilesh K, Cheran K, Gopi Kumar G, Chandy IP. Anaesthetic management of tetralogy of fallot coming for non-cardiac surgery: A case report. J Evol Med Dent Sci 2015;4:4029-32.  Back to cited text no. 9


  [Figure 1], [Figure 2]


Print this article  Email this article