Korean J Hematol.  2012 Dec;47(4):281-285. 10.5045/kjh.2012.47.4.281.

Surgery in patients with congenital factor VII deficiency: A single center experience

• Kim SH
• Park YS
• Kwon KH
• Lee JH
• Kim KC
• Yoo MC

• KMID: 1832107
• DOI: http://doi.org/10.5045/kjh.2012.47.4.281

Abstract

BACKGROUND
Congenital factor VII (FVII) deficiency is a rare hemorrhagic disorder that can cause excessive bleeding during and after surgery in affected patients. The recombinant form of activated factor VII (rFVIIa, NovoSeven(R) from Novo Nordisk, Bagsvaerd, Denmark), which was developed as a second-generation bypassing agent, has recently been used in the management of bleeding for patients with congenital FVII deficiency.
METHODS
We reviewed the results of 8 surgical procedures in 5 patients with congenital FVII deficiency at the Kyung Hee University Hospital, Gangdong, Seoul, Korea, between January 2008 and June 2010. We administrated rFVIIa preoperatively in six patients and postoperatively in five patients.
RESULTS
Between January 2008 and June 2010 at our center, 8 operations were performed successfully and no complications were observed in the 5 patients with congenital FVII deficiency. The median level of FVII activity was 2% (range, 0.6-7%). Four orthopedic procedures, 1 tonsillectomy, and 3 dental extractions were performed. The median duration of hospitalization was 8.5 days (range, 0-15 days). rFVIIa was administered at all procedures, except the dental extraction that was performed using only antifibrinolytic agents without any replacement. No bleeding or thrombogenic complications were observed in any case.
CONCLUSION
Patients with congenital FVII deficiency who require surgery can be treated efficiently and safely with rFVIIa or antifibrinolytic agents. rFVIIa was well tolerated and maintained effective hemostasis and showed good clinical outcome after the major surgery.

Keyword

Congenital FVII deficiency; Surgery; rFVIIa; Antifibrinolytic agent

MeSH Terms

Antifibrinolytic Agents
Factor VII
Factor VIIa
Hemorrhage
Hemorrhagic Disorders
Hemostasis
Hospitalization
Humans
Korea
Orthopedic Procedures
Recombinant Proteins
Tonsillectomy
Antifibrinolytic Agents
Factor VII
Factor VIIa
Recombinant Proteins

Figure

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  Fig. 1 (A) and (B) Three-dimensional computed tomography scan of the pelvis and hip showing diffuse and symmetrical narrowing in both hip joint spaces and erosive changes in the posteroinferior portions of both femoral heads (case 2).

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  Fig. 2 Radiograph of the elbow showing severe joint-space narrowing and deformities of the radiohumeral, humeroulnar, and proximal radioulnar joints (A). Radiograph of the right ankle showing joint-space narrowing, slanting of the tibiotalar joint, and irregularities with deformities of the distal tibia, fibula, and talus (B, case 5).

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  Fig. 3 Levels of factor VII (FVII, %) in 3 patients who underwent surgery. We checked baseline activity levels of factor VII following the administration of rFVIIa before surgery and 30 min after the administration of rFVIIa.

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