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Surgical interventions in childhood rare factor deficiencies: a single-center experience from Turkey
Zafer Salcioglu1, Deniz Tugcu, Arzu Akcay
1aKanuni Sultan Süleyman Education and Research Hospital, Pediatric Hematology and Oncology Clinic bKanuni Sultan Süleyman Education and Research Hospital, Pediatric Surgery Clinic cIstanbul University, Cerrahpasa Medical School, Internal Medicine Department, Istanbul, Turkey.
Insights
Surgical interventions in rare factor deficiencies are feasible with careful pre-surgical planning and appropriate factor replacement therapy. This study highlights successful management strategies for patients with rare bleeding disorders undergoing surgery.
Area of Science:
- Hematology
- Surgical Management
- Rare Bleeding Disorders
Background:
- Congenital rare factor deficiencies present diagnostic and management challenges, often with limited pre-surgical guidelines.
- These deficiencies, more prevalent in consanguineous populations, necessitate tailored approaches for surgical interventions.
- Existing data on surgical management are primarily based on case reports, lacking comprehensive directories.
Purpose of the Study:
- To evaluate the pre-surgical preparation, factor replacement strategies, and post-operative follow-up for patients with rare coagulation factor deficiencies.
- To analyze the safety and efficacy of various replacement therapies during surgical procedures in this patient cohort.
Main Methods:
- Retrospective analysis of 171 patients with rare factor deficiencies, focusing on 61 patients who underwent 88 surgical interventions between 1990 and 2012.
- Data retrieval from patient records and a data-processing environment, including details on factor deficiencies, pre-surgical activities, replacement therapies, and outcomes.
- Categorization of interventions into major, minor, and circumcision, with analysis of replacement strategies including fresh frozen plasma, recombinant factor VIIa, and antifibrinolytic agents.
Main Results:
- Factor VII deficiency was the most common (45 patients), followed by factors V, X, XI, XIII, and fibrinogen deficiencies.
- Surgical interventions included 24 major, 24 minor, and 40 circumcisions, with varying factor replacement strategies employed.
- Twenty-seven patients underwent surgery without replacement therapy; no thrombotic events or antibody development were observed post-intervention.
Conclusions:
- Surgical interventions in patients with rare factor deficiencies can be safely performed with meticulous pre-operative planning and appropriate factor replacement.
- The study demonstrates the feasibility of managing these complex cases, emphasizing the importance of individualized therapeutic strategies.
- Absence of thrombotic events and antibody formation suggests a favorable safety profile for the applied management protocols.
Abstract:
Congenital rare factor deficiencies may present in infancy by life-threatening bleedings or may not show any symptoms until adulthood. It is reported more commonly in countries having consanguineous marriages. Data regarding surgical interventions of rare congenital factor deficiencies are based on case reports and records of guidelines. There are no well documented and separately prepared directories related to pre-surgical and prophylactic approaches of surgical interventions of these deficiencies. Our retrospective study consisted of 171 rare factor deficiencies that were followed up in our clinic, and of whom 61 had 88 surgical interventions between 1990 and 2012. Of these patients, 45 were having factor VII deficiency, and factor V, X, XI, XIII and fibrinogen deficiencies were present in five, four, three, two and two patients, respectively. In 23 patients, factor coagulant activities were under 5% (37.7%), in 15 it was between 5 and 30% (24.6%), and in 23 between 30 and 50% (37.7%). Twenty-eight were symptomatic and 33 were asymptomatic. Information of 51 (83.6%) male and 10 (16.4%) female patients with an age range of 5-25 years (13 ± 5.27), whose age at presentation ranged between 3 weeks and 18 years (7 ± 4.66), were retrieved from patient records and from the records contained in the data-processing environment introduced in 2005. The rate of familial consanguinity was 49.2%. Of the surgical interventions, 24 (27.3%) were major, 24 (27.3%) were minor and 40 (45.4%) were circumcision. We used fresh frozen plasma in 32, recombinant factor (rF)VIIa in 20, prothrombin complex concentrate in five and fibrinogen in three patients during surgical interventions. In 18 patients, antifibrinolytic agents were also used. In 27 patients, surgical interventions were applied without any replacement therapy. No additional doses were required after surgical prophylaxis doses. Thrombotic events were not observed. Antibody occurrence was not detected in these patients. In our study, we evaluated preparation for surgical procedures, factor replacement therapy before surgical intervention and postoperative follow-up in patients with rare coagulation factor deficiency.
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