Bernard-Soulier syndrome: A challenge for anesthetist in an emergency surgery

Haq, Muhammad Inam Ul; Sohaib, Muhammad; Khan, Sidra; Nazir, Mohsin

doi:10.4103/0970-9185.161709

Cited by 6 publications

(5 citation statements)

References 4 publications

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“…Pregnancy in BSS is associated with a variable course. Outcome varies among different patients, and even for the same patient in different pregnancies[ 3 5 ] it may be complicated by maternal and fetal morbidity of various severity. [ 2 ] Although the optimum mode of delivery is not clear, vaginal delivery is recommended unless obstetrically 3 indicated CS.…”

Section: Discussionmentioning

confidence: 99%

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Bernard–Soulier syndrome (BSS) with uncontrollable menorrhagia

Bhadra

Chakraborty

2020

Asian J Transfus Sci

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Menorrhagia is a common problem in women of reproductive age group and 5% of women between 30 and 49 years of age group consult their general practitioner for this problem. Menorrhagia occurring in adolescent age group and perimenopausal age group is chiefly due to anovulatory cycles. In perimenopausal age group, local pathology like leiomyomas can also contribute to menorrhagia. Recently, bleeding disorders like von Willebrand Disease, single coagulation factor deficiencies particularly factor XI, VIII, Factor V and platelet function disorders have been found to be prevalent in patients presenting with menorrhagia. Bernerd soulier syndrome is extremely rare haemostatic disorder due deficiency in GP IB/IX/V receptor complex present on platelet membrane which is necessary for platelet aggregation. As a result of the defect patients have prolonged bleeding time, which might vary in symptoms ranging from spontaneous self- limiting epistaxis to life threatening haemorrhage. Here we report a case of 31-year-old woman known case of Bernard soulier syndrome with severe menorrhagia treated with mirena insertion and course of complication and its management.

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Section: Discussionmentioning

confidence: 99%

“…Prevalence has been estimated at <1/1,000,000. [ 3 ] In India, the reported cases are 27 till date. [ 4 ] The mode of inheritance is usually autosomal recessive with autosomal dominant pattern seen in isolated case.…”

Section: Introductionmentioning

confidence: 99%

Bernard–Soulier syndrome (BSS) with uncontrollable menorrhagia

Bhadra

Chakraborty

2020

Asian J Transfus Sci

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“…Thirty-three of those were assessed for eligibility. One report was excluded because it was related to second-trimester abortion and the remaining 32 reports were included in this systematic review 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 . Fig.…”

Section: Methodsmentioning

confidence: 99%

“…When they resumed oral TXA therapy, the bleeding stopped. Similarly, Ul Haq et al administered 2 g intravenous TXA prophylaxis just before the operation followed by 500 mg intravenous TXA every 6 hours for 48 hours and then continued with oral therapy and experienced no PPH [35]. In another case, 1 g TXA and 7 mg recombinant factor VIIa infusion were administered together at delivery, and 1 g TXA intravenous infusion was continued postpartum for 8 hours [22].…”

Section: Maternal Outcomementioning

confidence: 99%

Bernard-Soulier Syndrome from the Perspective of the Obstetrician: A Case Report with a Review of the Literature

Saridogan

Onat

Düz

et al. 2023

Z Geburtshilfe Neonatol

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Objective Bernard-Soulier syndrome (BSS) is one of the rare inherited platelet disorders that is characterized by macrothrombocytopenia and adhesion abnormality due to the absence or malfunctioning of the membrane GPIb-IX-V complex. There is no high-quality evidence on obstetric management of BSS owing to its rarity. Here we report an uncomplicated delivery of an adolescent with BSS and review the literature on the topic of BSS and pregnancy. Methods PUBMED, EMBASE, COCHRANE, and Google Scholar databases were searched up to April 2022 without language and year restriction using the terms “Bernard Soulier” and “Pregnancy”. The primary objectives were to evaluate maternal and fetal outcomes. The secondary objectives were to analyze pregnancy complications, gestational age at delivery, mode of delivery, administered prophylaxis, treatment approaches, duration of postpartum hospitalization, and the postpartum requirement of blood and blood product. Results The patient was a 19-year-old and 39-week pregnant woman who was diagnosed with BSS at the age of 10 by flow cytometry and genetic analysis. Single donor platelet transfusions and oral tranexamic acid were administered as prophylaxis at the peripartum period. She was delivered by cesarean section due to failure of labor. The postpartum period was uneventful for both mother and neonate. In the literature review, postpartum hemorrhage (PPH) was found in 52.9% (27/51) of deliveries. Late PPH occurred more frequently than early PPH (35.3 and 31.4%, respectively). 49% (25/51) of pregnancies had severe thrombocytopenia, and antepartum hemorrhage was observed in 11.8% (6/51) of those. The platelet count was in close relation to antenatal complications. 64.7% (33/51) of the patients were delivered via cesarean section. PPH and late PPH were found to be more common in those who delivered vaginally compared to those who delivered by caesarean section. It was observed that PPH was less common in women who were given prophylaxis in the peripartum period. Conclusion BSS is an inherited macro-thrombocytopathy that may cause adverse maternal and neonatal outcomes. The optimal mode and timing of delivery remain unclear. A multidisciplinary approach with prophylaxis at the peripartum period should be applied.

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“…The literature on perioperative and postoperative care for BSS patients remains scarce, primarily focusing on adult patients under 40 years old (Table 1) [11][12][13][14][15][16][17][18][19][20]. We have presented a successful management case of a frail patient diagnosed with BSS undergoing a major surgical procedure.…”

Section: Cryoprecipitatementioning

confidence: 99%

Perioperative Management of a Frail Patient With Bernard-Soulier Syndrome

Goncalves,

Fortunato

2024

Cureus

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Bernard-Soulier syndrome (BSS) is an autosomal recessive inherited bleeding disorder characterized by prolonged bleeding time, thrombocytopenia, and giant platelets. Patients with BSS are at an increased risk of bleeding, especially during traumatic injury and surgical procedures. The literature on the anesthetic management of patients with BSS is limited. In this report, we detail the successful management of a frail patient with BSS who underwent a major surgical procedure. Despite comprehensive clinical monitoring and an extended pharmacological strategy, a hemorrhagic complication occurred in the later postoperative phase, emphasizing the necessity for continued support and vigilant clinical monitoring due to the ongoing bleeding risk associated with these patients. In this case, a combined strategy involving antifibrinolytics, recombinant factor VII, and platelet transfusions proved effective.

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Bernard-Soulier syndrome: A challenge for anesthetist in an emergency surgery

Cited by 6 publications

References 4 publications

Bernard–Soulier syndrome (BSS) with uncontrollable menorrhagia

Bernard–Soulier syndrome (BSS) with uncontrollable menorrhagia

Bernard-Soulier Syndrome from the Perspective of the Obstetrician: A Case Report with a Review of the Literature

Perioperative Management of a Frail Patient With Bernard-Soulier Syndrome

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