Introduction
Spontaneous haemorrhage arising from the tonsils is a rare condition. Nearly all reported cases have been related to infection of the tonsils. In the pre-antibiotic era, bleeding from the major vessels was most commonly caused by peri-tonsillar or deep neck abscess.Reference Salinger and Pearlman 1 Since the advent of antibiotics, acute tonsillitis has become the commonest reported cause of spontaneous tonsillar haemorrhage.Reference Skinner and Chui 2 –Reference Kumra, Vastola, Keiserman and Lucente 7 Spontaneous tonsillar bleeding due to infectious mononucleosis, and idiopathic tonsillar haemorrhage, have also been reported.Reference Griffies, Wotowic and Wildes 4 , Reference Wolfe and Rowe 8 , Reference Vaughan and Parker 9
We present a case of spontaneous tonsillar haemorrhage secondary to von Willebrand's disease, which presented without symptoms or signs of infection.
Case report
An otherwise healthy, nine-year-old boy was admitted after ‘spitting out’ fresh blood for one day. There was a history of this phenomenon occurring a few times each year for the previous two years, with spontaneous recovery on every occasion. The child was apyrexial, without any recent history of sore throat or trauma to the oral cavity. In the past, he had undergone an uneventful herniotomy at the age of seven years, without any haemorrhagic complication, and he had no family history of bleeding tendencies.
On examination, blood could be seen oozing from the upper and middle parts of the right tonsil. The tonsil appeared otherwise normal and uninflamed. The patient was haemodynamically stable. A complete blood analysis, erythrocyte sedimentation rate (ESR) assessment and coagulation tests were done (Table I). The patient's activated partial thromboplastin time (APTT) was mildly elevated, at 46.2 seconds; on repeated analysis, the result was 38.6 seconds (normal range 27–37 seconds).
Table I Laboratory investigations on admission
*Range = 10–12; †range = 27–37. Hb = haemoglobin; WCC = white blood cell count; ESR = erythrocyte sedimentation rate; PT = prothrombin time; INR = international normalised ratio; APTT = activated partial thromboplastin time; sec = seconds
The patient's tonsillar haemorrhage persisted in spite of conservative treatment, which included hydrogen peroxide oral rinse and empirical intravenous antibiotics.
Therefore, surgical examination under general anaesthesia, with a right tonsillectomy, was undertaken. Intra-operative haemostasis using standard ties and diathermy techniques was challenging; a tonsillar tamponade pack was thus secured in situ. This was successfully removed three days later.
After the original explorative surgery, a bleeding time test was performed, with a prolonged result of 15 minutes (normal range <10 minutes). Coagulation factor assays were undertaken, and von Willebrand factor activity was found to be significantly reduced, at 19 per cent (normal range 50–200 per cent). Assay screens for other coagulation factors were within normal limits. A diagnosis of von Willebrand's disease was made.
With this diagnosis established, a more detailed clinical history was taken. It appeared that the patient's mother had actually experienced significant post-operative haemorrhage after a hysterectomy.
Family screening for von Willebrand's disease was undertaken, which revealed that the patient's mother and maternal aunt were also affected.
A further, secondary post-tonsillectomy haemorrhage occurred during the second post-operative week. This was effectively controlled by cryoprecipitate infusion and intravenous antibiotics.
Further recovery was uneventful and the patient was referred for specialist haematology management on discharge.
Discussion
Spontaneous tonsillar haemorrhage is uncommon. Almost all previous reports have identified infection as the cause.
In the pre-antibiotic era, Salinger and Pearlman reported 227 cases of spontaneous tonsillar haemorrhage caused by fatal erosion of the major vessels due to peri-tonsillar abscesses or deep neck abscesses.Reference Salinger and Pearlman 1
Since the widespread availability of antibiotics, there has been only a single case report of this complication.Reference Blum and McCaffrey 10 Griffies et al. found that only 1 per cent of patients with acute tonsillitis presented with spontaneous tonsillar haemorrhage.Reference Griffies, Wotowic and Wildes 4 However, acute tonsillitis has been the commonest reported aetiology of spontaneous tonsillar haemorrhage in recent decades (Table II).Reference Skinner and Chui 2 –Reference Kumra, Vastola, Keiserman and Lucente 7 All cases complained of a sore throat with inflamed tonsils on presentation. Evidence of tonsillitis may be apparent, such as pyrexia, cervical lymphadenopathy and an elevated white cell count, together with a histological picture of neutrophil infiltration, microabscess formation and superficial ulcerations in the surgical tonsillar specimen. In cases of acute tonsillitis, the pathogenesis of spontaneous tonsillar bleeding is believed to be erosion of superficial tonsillar vessels.Reference Griffies, Wotowic and Wildes 4 Furthermore, xenon-133 clearance studies have demonstrated an increase in tonsillar blood flow during tonsillitis, which may also contribute to the risk of haemorrhage.Reference Ozdemir, Ercan and Kaya 11
Table II Reports of spontaneous tonsillar haemorrhage due to acute tonsillitis
*Three tonsillectomies were performed after haemorrhage and infection had been controlled. Pts = patients; y = years; CL = cervical lymphadenopathy; WCC = white blood cell count; M = male; F = female; ↑= elevated; N = normal; strep = group A β-haemolytic streptococcus; Xmas = Christmas; vWB = von Willebrand's
Vaughan and Parker reported the only described case of idiopathic spontaneous tonsillar bleeding: a patient who was asymptomatic for infection and had normal clotting times, platelet counts and bleeding times, and for whom histopathological examination showed no features of inflammation or abnormality.Reference Vaughan and Parker 9
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• In the pre-antibiotic era, spontaneous tonsillar bleeding was usually related to major vessel bleeding, due to peri-tonsillar or deep neck abscess
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• In the antibiotics era, spontaneous tonsillar bleeding is usually related to acute tonsillitis
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• Spontaneous tonsillar bleeding without evidence of infection may be related to bleeding disorders
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• A normal or near-normal clotting profile cannot completely exclude von Willebrand's disease, and further investigations should be undertaken if necessary
Two cases of acute tonsillitis with underlying bleeding disorders have been reported. One patient had a known history of Christmas disease (i.e. factor XII deficiency), whilst the other was confirmed subsequently to have von Willebrand's disease.Reference Levy, Brodsky and Stanievich 5 , Reference Kumra, Vastola, Keiserman and Lucente 7
Our patient suffered spontaneous tonsillar haemorrhage secondary to von Willebrand's disease, without any objective evidence of established infection. He was asymptomatic, with noninflamed tonsils, and tonsillar histological examination did not demonstrate neutrophil infiltration, microabscesses or lymphoid hyperplasia. The patient's ESR was 14 mm/hour and his white blood cell count was 16 × 109/l.
Von Willebrand's disease is the commonest inheritable coagulation disorder. It is an autosomal dominant condition with variable penetrance. Phenotypic variability is the hallmark of this disease, with a variety of clinical presentations. The prothrombin time is normal, while the APTT can be normal or slightly elevated. A high index of clinical suspicion is necessary, and bleeding time should be tested if indicated. Diagnosis is confirmed by assay of coagulation factor VIII. In cases of undiagnosed von Willebrand's disease, surgeons may experience difficulty achieving haemostasis during elective tonsillectomy, and post-operative haemorrhage may be encountered.Reference Gumprecht and Cichon 12
Conclusion
Spontaneous tonsillar haemorrhage is uncommon. It is usually related to infection of the tonsils, the peri-tonsillar space or the deep neck space. Further investigations to exclude underlying bleeding disorders may be necessary, especially when bleeding is significantly prolonged or if there are no symptoms or signs of infection. Normal or slightly abnormal clotting times cannot completely exclude bleeding disorders. The bleeding time should be assessed and coagulation factor assays undertaken.
