Prevalence of hereditary hemorrhagic telangiectasia in patients operated for cerebral abscess: a retrospective cohort analysis

Abstract

It is well described that patients with pulmonary arteriovenous malformations (PAVMs) and Hereditary Hemorrhagic Telangiectasia (HHT) have an increased risk of cerebral abscess (CA). However, as both CA and HHT are rare, the proportion of patients with CA who are diagnosed with HHT has not been previously described. A retrospective study was carried out of all patients treated surgically for CA between January 1995 and September 2014 at the Department of Neurosurgery, Odense University Hospital. The cases were then cross-referenced with the Danish HHT database. Eighty patients aged 5-79 years were included. The incidence of CA was 0.33/100,000/year. Two patients (2.5%) were registered as having HHT. Bacterial pathogens were identified in 70% of all cases, most frequently streptococci species (46.3%). The most common predisposing condition was odontogenic infection (20%), followed by post-operative infection (13.8%) and post-trauma (6.3%). Patients undergoing a full diagnostic program to determine predisposing conditions causing CA increased over the 20-year period from 11.8% to 65.2%. The 3-month and 1-year mortality rates were 7.5% and 11.25%, respectively. There is an overrepresentation of HHT patients in a cohort of patients with CA, and HHT should be investigated as the cause of the CA if no other apparent cause can be identified.

Positive predictive value of ICD-10 diagnosis codes for brain abscess in the Danish National Patient Registry

“…Among these, 80 were confirmed to have brain abscess after review of medical records, yielding a PPV of 89% (95% CI: 80–94), similar to our PPV of 84% using the previously described criteria. 15 However, in our nationwide population-based study, we observed that only 54% of patients with brain abscess would have been identified by retrieval of ICD-10 diagnosis and procedure codes for brain abscess at departments of neurosurgery. If these results also apply to other countries, this suggests that previous estimates of incidence and descriptive studies of brain abscess may be incomplete.…”

Life-Threatening Intraventricular Rupture of Brain Abscess in a Patient With Undiagnosed Hereditary Hemorrhagic Telangiectasia

“…However, compared to BAs in the general population, Staphylococcus is rarely isolated from BAs in HHT in which BAs are more likely to be polymicrobial [ 3 , 12 , 13 ]. Approximately 20%-30% of BAs in HHT patients will have sterile cultures for unclear reasons, as was the case in our patient [ 3 , 11 ]. One possible explanation for the sterile cultures could be the use of antibiotics prior to the LP.…”

“…BAs represent a serious complication of HHT and generally present with systemic signs of infection, focal neurological deficits, or signs of increased intracranial pressure. Although BAs present similarly in HHT as they do in the general population, the risk of BAs in patients with HHT is evidently 1,000 times greater than the general population [ 3 , 11 ]. Anaerobic and facultative anaerobic bacteria are frequently implicated in both populations, with Streptococcus species being the most common in both [ 3 , 11 , 12 ].…”

“…Although BAs present similarly in HHT as they do in the general population, the risk of BAs in patients with HHT is evidently 1,000 times greater than the general population [ 3 , 11 ]. Anaerobic and facultative anaerobic bacteria are frequently implicated in both populations, with Streptococcus species being the most common in both [ 3 , 11 , 12 ]. However, compared to BAs in the general population, Staphylococcus is rarely isolated from BAs in HHT in which BAs are more likely to be polymicrobial [ 3 , 12 , 13 ].…”

Brain abscess of odontogenic origin in children: a systematic review of the literature with emphasis on therapeutic aspects and a new case presentation

“…Inherited bleeding telangiectasia (HHT), or Rendu-Osler-Weber syndrome, is an autosomal dominant disorder that is a predisposing factor for the onset of BAs. Recurrent episodes of spontaneous epistaxis, skin telangiectasia and mucous and visceral AVMs are its major clinical features and may involve the pulmonary circulatory system, liver, brain, spinal and other systems 23 . It is estimated that BAs occur in approximately 5 to 10% of patients with HHT; bacteraemia, especially of dental origin, plays a decisive role in the pathogenesis of BAs in patients with HHT.…”