Hemophilia A and B are rare X-chromosome-linked recessive bleeding disorders caused by mutations in the genes causing abnormalities of blood clotting factors VIII and IX, respectively. Surgery in these patients will require additional planning and interaction among the surgeon, anesthetist, and a hematologist because they inevitably result in bleeding, excessive blood loss, and other life-threatening complications. The authors present a case 62-year-old male with haemophilia B and progressive neurological claudication. On plain radiographs and MRI the patient had grade 1 spondylolisthesis with lumbar canal stenosis at L4-L5 with a VAS score of 8 and ODI score of 45 and was operated with MIS-TLIF with 22 mm diameter tubular retractor (METRx, Medtronics) and an operating microscope. Pre-operatively, the hematologist opinion was taken and the patient was optimised by maintaining the plasma factor peak level activity according to the WFH guidelines. The patient had uneventful peri-operative period. The total hospital stay is 16 days and a VAS score of 3 and ODI score of 12 after one-year follow-up and without any notable complications. Minimally invasive surgical techniques are a better option in hemophilia patients as these techniques provide the surgeon with an excellent magnification of the operative field, which enables the use of a smaller incision, better hemostasis, and facilitates less traumatic procedures.
Spine surgery in the hemophilia patient is not a well documented entity in the literature. As per the author’s knowledge, there is no literature related to minimally invasive spine surgery in hemophilia. We report our experience with a patient of hemophilia B treated with minimally invasive transforaminal lumbar interbody fusion (MIS-TLIF).
After taking patient consent for purpose of the study with due care to maintain his privacy, the authors present a known case of 62-year-old male with haemophilia B and progressive neurological claudication. On plain radiographs, the patient had grade 1 spondylolisthesis according to mayerdings classification at L4-L5 with a visual analogue scale (VAS) score of 8 and Oswestry disability index (ODI) score of 45 and no symptomatic improvement with conservative management. On MRI, the patient had L4-L5 lumbar canal stenosis and the patient was operated on with MIS-TLIF (
Under anesthesia, the patient was prone positioned on a radiolucent operating table. Under fluoroscopic guidance, the level was confirmed and a 3 cm long paraspinal incision 3–5 cm away from midline was given on the more symptomatic side for decompression utilizing the same for pedicle screw insertion on that side. Sequential dilatation was done and a tubular retractor with 22 mm diameter (METRx, Medtronics) was docked over the facet and spino-laminar line. Ipsilateral facetectomy and laminotomy, along with the removal of ligamentum flavum were performed under a microscope to accomplish adequate neural decompression. Utmost care is taken at every step to achieve meticulous hemostasis. Following discectomy and preparation of endplates, an appropriate size interbody cage filled with autologous bone was inserted. The pedicles were cannulated with a cook’s needle under fluoroscopic guidance and guidewires were inserted on both sides. The serial dilators were used to dilate over the guidewires and the pedicles were tapped using a cannulated tap. Screws were placed with corresponding screw extenders and the rod was introduced with a device through a proximal stab incision. After placement of locking-cap screws through the screw extenders and application of compression, the screws were torqued and the screw extenders were removed. Closure in layers was performed following wound hemostasis and unlike routine protocol, negative suction drain was kept for one day.
Hemophilia A and B are rare X-chromosome-linked recessive bleeding disorders caused by mutations in the genes causing qualitative and quantitative abnormalities of blood clotting factors VIII and IX, respectively [
It has been described in the descent of Queen Victoria of England and is often called “the disease of the kings” [
Surgery in these patients will require additional planning and interaction among the surgeon, anesthetist, and a hematologist because they inevitably result in bleeding, excessive blood loss, and other life-threatening complications. There are some previous studies showing that hemophilia patients can be operated with good results [
Although we had good results in this case report, further studies with a large study group are required. However, this case helps in understanding the protocols of peri-operative management in a hemophilia patient.
Surgeries are safe in hemophilia patients with good planning and a holistic team approach including surgeon, hematologist and anaesthetist with dose adjustment of the coagulation factor to maintain a desirable factor level and having a smooth postoperative recovery. Minimally invasive surgical techniques will further help in these patients, as these techniques provide the surgeon with an excellent magnification of the operative field, which enables the use of a smaller incision, less muscle trauma and better hemostasis.
No potential conflict of interest relevant to this article.
Index case. (A) Pre-operative plain radiographs. (B) Axial MR images. (C) Sagittal MR images. (D) Post-operative plain radiographs.
Summary of the patient
Age | 62 years |
Procedure | L4-5 MIS-TLIF |
Operative time | 139 minutes |
Blood loss | 130 mL |
Drain output | 20 mL |
Hospital stay | 16 days |
Pre-op factors (IU/DL) | 60–80 |
Post-operative factors (IU/DL) | |
1–3 | 40–60 |
4–6 | 30–50 |
7–14 | 20–40 |
Blood transfusion | Not done |
Complications | None |
MIS-TLIF: minimally invasive transforaminal lumbar interbody fusion.