Journal of Pediatric Neurosciences
: 2020  |  Volume : 15  |  Issue : 3  |  Page : 322--324

Anesthetic management of an adolescent with hypofibrinogenemia posted for cervical deformity correction!

Mayank Tyagi, Indu Kapoor, Girija P Rath 
 Department of Neuroanaesthesiology and Critical Care, Neuroscience Centre, All India Institute of Medical Sciences (AIIMS), New Delhi, India

Correspondence Address:
Dr. Indu Kapoor
Department of Neuroanaesthesiology and Critical Care, Neuroscience Centre, All India Institute of Medical Sciences (AIIMS), New Delhi.


A 15-year-old boy diagnosed as case of cervical kyphosis, atlantoaxial dislocation, and hypofibrinogenemia was posted for corrective surgery under general anesthesia. His preoperative plasma fibrinogen levels and other coagulation profile were deranged. He was diagnosed with type 1 congenital fibrinogen abnormality, that is, hypofibrinogenemia. Preoperative blood products including cryoprecipitates were transfused to correct fibrinogen levels and coagulation profile. Cryoprecipitate was transfused at the dose of 1 bag per 10kg body weight per day. During intraoperative period, blood products were transfused following blood loss of 1100mL. Postoperatively the repeat laboratory investigations after 20 h revealed normal PF levels (211 mg/dL) with normal coagulation profile. He was discharged five days later with the uncomplicated postoperative course.

How to cite this article:
Tyagi M, Kapoor I, Rath GP. Anesthetic management of an adolescent with hypofibrinogenemia posted for cervical deformity correction!.J Pediatr Neurosci 2020;15:322-324

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Tyagi M, Kapoor I, Rath GP. Anesthetic management of an adolescent with hypofibrinogenemia posted for cervical deformity correction!. J Pediatr Neurosci [serial online] 2020 [cited 2021 Jan 28 ];15:322-324
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Hypofibrinogenemia (HF) is a rare autosomal recessive coagulopathy. It has an estimated incidence of 1:1–2,000,000 with male-to-female ratio of 1:1.[1],[2] HF attributes to myriad of presentations which include epistaxis, central nervous system bleeding, gastrointestinal bleeding, menorrhagia in females, and wound dehiscence after surgery. The prolonged bleeding from the umbilical cord is usually the first manifestation in 85% of cases.[1]

We report a case of 15-year-old boy posted for distraction, compression, extension, and retraction (DCER) procedure with deformity correction for atlantoaxial dislocation and cervical kyphosis under general anesthesia. During preoperative assessment, his laboratory investigations revealed plasma fibrinogen (PF) concentration was <50 mg/dL, prothrombin time (PT) >60s, activated partial thromboplastin time (aPTT) >180s and thrombin time (TT) >60s. Rest all investigations were within normal limits. He was diagnosed with type 1 congenital fibrogen abnormality, that is, HF. A day before surgery, six units of cryoprecipitate and three units of fresh frozen plasma were transfused. Repeat investigations after transfusion showed PF of 196 mg/dL with normal coagulation profile. Standard protocol was used to induce anesthesia. In view of anticipated difficult airway, tracheal intubation was done using fiberoptic bronchoscope. Anesthesia was maintained with sevoflurane 0.8% concentration with oxygen:air (40%:60%), fentanyl (1 µ/kg/min), and rocuronium (0.2 mg/kg/h). During intraoperative period two units of packed red cells, two units of fresh frozen plasma and four units of cryoprecipitate was administered following total blood loss of 1100mL. Surgery lasted for 8h and the hemodynamics remained stable throughout the procedure. He was shifted with endotracheal tube in situ to intensive care unit for further management. Next morning, his trachea was extubated. The repeat laboratory investigations after 20 h were suggestive of normal PF levels (211 mg/dL) with normal coagulation profile. He was discharged five days later with the uncomplicated postoperative course.

Cervical kyphosis deformity (CKD) is an uncommon but potentially debilitating condition.[3] Treatment of CKD in terms of ideal mode of surgical correction is still controversial. Management of a patient of CKD with HF undergoing corrective surgery is an extremely challenging situation for an anesthesiologist. Airway difficulties are anticipated when the kyphosis involves the upper thoracic or cervical spine. Also devices like halo traction may interfere with securing the airway. There might be difficulty in maintaining oxygenation in these patients as distortion of the tracheobronchial tree is a common accompaniment.[4] Apart from an anticipated difficult airway, significant blood loss is another main concern in this patient diagnosed with congenital fibrinogen abnormality undergoing major corrective spine surgery.

HF is defined by a decreased level of normal fibrinogen below 50 mg/dL.[1] Fibrinogen supplementation can be provided by transfusion of fresh-frozen plasma (FFP), cryoprecipitate, and fibrinogen concentrate.[5],[6] FFP has several limitations including a low fibrinogen content, which means that large volumes must be given, and the risk of transfusion-related complications (transfusion-related acute lung injury (TRALI) and viral transmission) is very high. Although cryoprecipitate contains a higher concentration of fibrinogen than FFP, usually around 15g/L, it shares many of the disadvantages of FFP as its fibrinogen concentration is not standardized and blood group matching is needed prior to transfusion. Time is also required to thaw cryoprecipitate, and this aspect represents a clear disadvantage in the setting of massive hemorrhage. Fibrinogen replacement therapy is currently indicated as prophylaxis and therapy of hemorrhage in congenital and acquired fibrinogen deficiency, this latter being associated with liver failure, disseminated intravascular coagulation, massive transfusion and cardiac surgery.[7],[8] Prophylactic treatment is recommended to raise PF levels to 100–150 mg/dL during surgery.[2] Replacement therapy with plasma-derived fibrinogen concentrate is the treatment of choice for symptomatic bleeding patients. Though cryoprecipitate has been withdrawn from many European countries because of safety concerns, it remains available for haemostatic therapy in the USA and Canada where it still lacks Level 1 evidence to support its efficacy. The American Society of Anesthesiologists (ASA) recommends the perioperative administration of cryoprecipitate only during three circumstances: (a) for prophylaxis in nonbleeding perioperative patients with congenital fibrinogen deficiencies, (b) for bleeding patients with von Willebrand disease, and (c) in massively transfused patients with fibrinogen concentrations less than 80–100 mg/dL or when fibrinogen concentrations cannot be measured in a timely manner.[9] In our case, indication for cryoprecipitate transfusion was congenital fibrinogen deficiency. Cryoprecipitate was transfused at the dose of 1 bag per 10kg body weight per day. After transfusion of six units of cryoprecipitate, the bleeding parameters improved before surgery. In immediate postoperative period, repeat coagulation profile was normal, but PF level was still <50 mg/dL. It is based on the fact that the fibrinogen concentrate in blood takes time of 18–36h to reach at its peak level after transfusion.[2] Hence the report of sample taken after 20 h showed the normal fibrinogen levels (211 mg/dL). For intraoperative fibrinolysis monitoring, use of viscoelastic essays, that is, TEG and ROTEM would help in guarded transfusion of blood products. However, studies have revealed equivocal findings for blood loss and transfusion requirements when these essays were compared with no fibrinolysis monitoring.[10],[11] Our case report affirm the underline importance of preoperative comprehensive clinical examination, elaborative laboratory workup and prophylactic treatment to raise fibrinogen level 100–150 mg/dL before surgery.

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