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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 3  |  Issue : 1  |  Page : 20-23

When bleeding wins clotting: The surgical dilemma in life-threatening hemothorax in hemophilia


1 Department of General Surgery, St. John's Medical College Hospital, Bengaluru, Karnataka, India
2 Department of Cardiothoracic and Vascular Surgery, St. John's Medical College Hospital, Bengaluru, Karnataka, India
3 Department of Surgical Oncology, St. John's Medical College Hospital, Bengaluru, Karnataka, India

Date of Web Publication20-Dec-2018

Correspondence Address:
Joseph Sushil Rao
Department of Surgical Oncology, St. John's Medical College Hospital, Bengaluru - 560 034, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jctt.jctt_2_18

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  Abstract 


Due to the lack of awareness and poor access to laboratory diagnosis, hemophilia may not be diagnosed preoperatively leading to therapeutic misadventure during surgery. Hence, this congenital bleeding disorder due to Factor VIII deficiency reduces surgical management. We report a 39-year-old gentleman, diagnosed of Factor VIII deficiency who presented to emergency with acute spontaneous left hemothorax and underwent a successful thoracotomy and decortication which saved his life. He is positive for human immunodeficiency virus as well as hepatitis B for which he is on treatment. The management guidelines for thoracic surgery are not addressed to in the World Federation of Hemophilia guidelines, making the management challenging in this scenario. We report this case due to its rarity and emphasize that early recognition with immediate surgical intervention supported with Factor VIII transfusion played an important role in saving life.

Keywords: Factor VIII supplementation, hemophilia, hemothorax, surgical management


How to cite this article:
Jain NB, Balasundaram S, Rao JS. When bleeding wins clotting: The surgical dilemma in life-threatening hemothorax in hemophilia. J Cardiothorac Trauma 2018;3:20-3

How to cite this URL:
Jain NB, Balasundaram S, Rao JS. When bleeding wins clotting: The surgical dilemma in life-threatening hemothorax in hemophilia. J Cardiothorac Trauma [serial online] 2018 [cited 2019 Dec 12];3:20-3. Available from: http://www.jctt.org/text.asp?2018/3/1/20/248100




  Introduction Top


Hemophilia A is an X-linked autosomal recessive disorder of coagulation presenting with deficiency of Factor VIII. Hemophilia patients are prone to life-threatening bleeding[1] and are often not suitable for surgery unless adequate therapeutic material with laboratory facilities for factor assays and inhibitor assays is available. Surgery in hemophilia patients continues to be challenge to surgeons and hematologists in the modern era of innovations/technology.

This is an unusual case of hemophilia A presenting with spontaneous left hemothorax requiring surgery and intensive care with high ventilator supports.


  Case Report Top


A 39-year-old gentleman diagnosed of hemophilia A on episodic Factor VIII supports since childhood alongside treatment for human immunodeficiency virus and hepatitis B presented to emergency with acute onset of breathlessness of 24 h with no history of trivial trauma to the chest or abdomen. Clinical examinations revealed tachypnea and tachycardia with saturation of 82% on room air. On further blood investigations relevant to his condition, he was found to have hypoxia on arterial blood gas (pH: 7.4; pCO2: 33; p02: 54.2; and HCO3: 18.5) and low Factor VIII inhibitor titers requiring admission for further evaluation and management.

On admission, he was anemic with a hemoglobin of 8.1 with Factor VIII assay of <1% and Factor VIII inhibitor in Bethesda assay being one. The CD4 count was 825.

Imaging studies showed haziness of the left lung on posteroanterior view of chest X-ray and massive hematoma in the left hemithorax with concordant left pleural effusion causing a mediastinal shift to the right on computed tomography [Figure 1] and [Figure 2].
Figure 1: Computed tomography chest sagittal section showing left massive hemothorax with left pleural effusion

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Figure 2: Computed tomography chest – axial section showing left massive hematoma with left pleural effusion causing mediastinal shift to the right

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The patient was intubated and administered 3000 IU of Factor VIII every 6 h under regular activated partial thromboplastin time monitoring on day 1. He underwent under high risk an emergency left thoracotomy. At the time of inducing anesthesia, injection antihemophilic factor 3000 IU was administered with a repeat dose of 3000 IU 1 h into the surgery.

Left lateral thoracotomy was performed and parietal pleura entered in the left fifth intercostal space. Approximately, 1600 ml of clotted blood/hematoma was evacuated [Figure 3] and [Figure 4]. Densely adherent visceral pleural peel was noted [Figure 5]. As the lung expansion was not satisfactory even after the evacuation of clots, decortication was performed on the left lower and part of the upper lobe [Figure 6]. Postdecortication, saturation improved from 82% to 95% and lung expansion was satisfactory. Basal and apical intercostal drains were placed. Three units of packed cells and two units of crystalloids were transfused intraoperatively.
Figure 3: Evacuated blood clots following left thoracotomy

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Figure 4: Clotted blood/hematoma approximately measuring 1.6 L

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Figure 5: Adherent visceral pleura noted after evacuation of clots

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Figure 6: A 0.5-cm thick densely adherent visceral pleura noted, decortication done

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Following successful decortication, lung expansion was adequate, and the patient was gradually weaned off the ventilator on the 6th postoperative day and managed on noninvasive ventilation for the next few days. Factor VIII infusion therapy continued with dynamic monitoring of Factor VIII levels [Figure 7].
Figure 7: Dynamic monitoring of Factor VIII levels and Anti-Hemophillic Factor administered during the course of hospitalization

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Postoperative thoracic drainage reduced gradually, and the basal chest tube was removed on postoperative day 6, and the patient showed no evidence of discomfort. On postoperative day 8, the apical chest tube was also removed. X-ray revealed postoperative changes in lung compliance, with good lung re-expansion.


  Discussion Top


Surgery in hemophilic A patient is challenging involving a multidisciplinary approach. Surgical management of hemophilia in the developing world is difficult in the setting of limited resources; the major constraint being limited availability of factor concentrates and laboratory supports.

There have only been studies describing minor surgical procedures in hemophilia patients.[2],[3],[4] Thus, extensive preoperative preparations and postoperative care are of utmost importance for these patients. Through the course of the diagnosis and treatment in the present case, the maintenance of Factor VIII levels was found to be critical, since the biological half-life of Factor VIII is between 6 and 14 h. Regular monitoring of clotting factor levels during treatment, adjustment of infusion doses, and intervals is crucial.

The management guidelines for thoracic surgery in hemophilia are not addressed in the World Federation of Hemophilia guidelines making the management even more challenging in our case. These recommendations were not established based on the large clinical trials and fail to define the safe lower limit of factor concentrate prophylaxis for surgery. We report this case in which we successfully managed the surgical intervention with low levels of Factor VIII. The World Federation of Hemophilia guidelines established for neurovascular bleeds suggested desired factor levels of 80–100 IU/dl for initial 1–7 days and maintenance of 50 IU/dl for 8–21 days,[5] where there is no significant resource constraint. However, recommend levels of 50–80 IU/dl for 1–3 days and maintenance of 30–50 IU/dl for 4–7 days followed by 20–40 IU/dl up to 2 weeks in resource-constrained situations.[5] However, we successfully managed the surgical intervention at low levels of Factor VIII during hospitalization with the chest tube drainage gradually reducing and removed at the time of discharge emphasizing that low-dose protocols are effective and are not associated with a significantly increased risk of delayed hemorrhage.

Acknowledgment

We would like to thank the Department of Cardiothoracic and Vascular Surgery, St. John's Medical College Hospital, Bangalore - 560034, India.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Chen HZ. Practical Internal Medicine. 10th ed. Beijing: People's Medical Publishing House; 1998.  Back to cited text no. 1
    
2.
Washino S, Hirai M, Kobayashi Y, Saito K, Miyagawa T. Heavy hematuria requiring cystectomy in a patient with hemophilia A: A case report and literature review. BMC Urol 2015;15:84.  Back to cited text no. 2
    
3.
Ingram GI. Haemophilia and the forbidden abdomen. Haemophilia 2000;6:719-22.  Back to cited text no. 3
    
4.
Lavery HJ, Senaratne P, Gainsburg DM, Samadi DB. Robotic prostatectomy in a patient with hemophilia. JSLS 2010;14:439-41.  Back to cited text no. 4
    
5.
Srivastava A, Brewer AK, Mauser-Bunschoten EP, Key NS, Kitchen S, Llinas A, et al. Guidelines for the management of hemophilia. Haemophilia 2013;19:e1-47.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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