Perioperative management of a patient with Bernard-Soulier syndrome for third molar surgery

Michael J Hartman1, John F Caccamese, Stewart A Bergman

  • 1University of Maryland Medical Center, R. Adams Cowley Shock Trauma Unit, Baltimore College of Dental Surgery, Baltimore, MD 21201, USA. mhart002@umaryland.edu

Bernard-Soulier Syndrome (BSS) is a disease characterized by prolonged bleeding time, thrombocytopenia, and extremely large platelets and has a prevalence of less than 1 in 1,000,000. Patients with disorders of coagulation and bleeding can be among the most difficult surgical patients to manage. Perioperative hemorrhage can contribute to life-threatening complications in even the most routine surgical procedures. Because of the rarity of BSS, there are no well-defined protocols for the management of perioperative bleeding associated with this condition. Treatment with preoperative and intraoperative systemic aminocaproic acid, HLA-matched platelets, and topical gelfoam and thrombin resulted in sustained hemostasis and a durable healing response. For those rare few afflicted with this disease, we present a combined systemic and topical approach that may be helpful in the control and prevention of perioperative hemorrhage in this and other similar platelet disorders.

Related Concept Videos

Aneurysm IV: Nursing Management01:22

Aneurysm IV: Nursing Management

Vigilant monitoring for aneurysm rupture is essential for patients undergoing aortic surgery.Preoperative Nursing ManagementContinuously monitor the patient for manifestations of aneurysm rupture, such as pallor, weakness, tachycardia, hypotension, abdominal, back, groin, or periumbilical pain, changes in consciousness, and a pulsating abdominal mass. Regularly assess the patient's peripheral pulses.Instruct the patient to consume a clear liquid diet the day before surgery and administer...
Cardiomyopathy VII: Pre and Post Operative Nursing Management01:28

Cardiomyopathy VII: Pre and Post Operative Nursing Management

Patients with hypertrophic cardiomyopathy (HCM) and left ventricular outflow tract (LVOT) obstruction who remain symptomatic despite optimal medical therapy may undergo a septal myectomy (Morrow procedure). This procedure involves excising a portion of the hypertrophied septum below the aortic valve using a heart-lung machine to improve blood flow through the LVOT. Effective preoperative and postoperative nursing management ensures successful patient outcomes, minimizes complications, and...
Peripheral Artery Disease V: Postoperative Nursing Management01:23

Peripheral Artery Disease V: Postoperative Nursing Management

During the postoperative period, it is crucial to focus on maintaining circulation, identifying and managing potential complications, and planning for discharge.Nursing AssessmentVital signs monitoring: Regularly monitor vital signs, including blood pressure, heart rate, respiratory rate, and temperature, to detect early signs of complications such as bleeding and infection.Circulation assessment: Monitor pulses, perform Doppler assessments, and check capillary refill, color, temperature, and...
Endoscopic Procedures IV: Sigmoidoscopy and Laproscopy01:26

Endoscopic Procedures IV: Sigmoidoscopy and Laproscopy

Sigmoidoscopy and laparoscopy are distinct medical procedures that enable physicians to internally inspect different parts of the GI tract. Although they serve different purposes, each is essential for diagnosing and, in some cases, treating various medical conditions.
Sigmoidoscopy
Sigmoidoscopy is a diagnostic procedure that uses a flexible sigmoidoscope equipped with a light source and camera to examine the rectum and sigmoid colon. The procedure involves inserting the tube through the anus...
Tonsillitis II: Management01:26

Tonsillitis II: Management

This lesson will focus on the different treatment options for managing tonsillitis, which typically depend on the cause and severity.
Mitral Stenosis III: Medical Management01:26

Mitral Stenosis III: Medical Management

Mitral stenosis, a condition marked by the narrowing of the mitral valve, necessitates an integrated approach for effective management. This approach includes preventative measures, medical therapy, and surgical interventions to reduce symptoms and prevent complications.PreventionPrevention of mitral stenosis primarily focuses on reducing the incidence of bacterial infections, particularly streptococcal infections, which can lead to rheumatic fever and subsequent valvular damage. Timely...