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Tetralogy of Fallot (TOF) is the first described and most common cyanotic congenital heart anomaly that generated the first successful surgical palliation procedure and definitive intracardiac repair. Classical TOF comprises the four typical features of right ventricular outflow tract obstruction (RVOTO), right ventricular hypertrophy (RVH), ventricular septal defect (VSD) and aortic dextroposition. Complex forms of the condition include TOF associated with absent pulmonary valve and TOF with pulmonary atresia (TOF-PA) with or without major aortopulmonary collateral arteries (MAPCAs). The pathophysiological understanding that the tetrad is basically the sequel of a singular abnormality of infundibular malformation, with anterior deviation of the infundibular septum, had major surgical consequences that improved survival. Diagnostic and functional imaging play a key role in the clinical and surgical management of patients with TOF. We revisit the role of traditionally employed imaging modalities (echocardiography and cardiac catheterisation) only briefly as these remain within the domain of cardiology practice. The emphasis of the present review is to outline the role of cardiac computed tomographic angiography (CTA) in the evaluation of TOF, with special reference to the technical considerations and best practice recommendations. Cardiovascular magnetic resonance imaging (CMR) is addressed only in passing, as this service is not currently available at our institution.

The finding of substantial IRR amongst radiologists and urologists utilizing the AAST system supports continued use of the broad parameters of the AAST system, with some modification in specific categories with lower agreement.

IntroductionPrimary IgG4-related disease (IgG4-RD) of the temporal bone is a rare condition. Unlike typical petrous apicitis or Gradenigo syndrome, our patient presented exclusively with unilateral cranial nerve VI palsy and symptoms of diplopia. Skull base imaging demonstrated a destructive bony lesion in the petrous apex. Imaging and systemic investigations were insufficient to support a diagnosis. The diagnosis was achieved histologically after acquiring the specimen by middle cranial fossa craniotomy and temporal bone biopsy. This case report is thought to be the first published description of a diagnosis of IgG4-RD proven with the middle cranial fossa approach.Case ReportWe describe a 29-year-old female with primary IgG4-RD of the petrous apex of the temporal bone. This patient presented with a few-month history of left-sided headache and recent-onset diplopia due to paralysis of cranial nerve VI. Imaging demonstrated a petrous apex lesion, and comprehensive systemic investigations could not reach a diagnosis. A middle cranial fossa craniotomy and a biopsy of the temporal bone lesion were undertaken to establish the diagnosis. Histological confirmation of IgG4-RD was proven. Following treatment with corticosteroids, the patient experienced complete recovery and resolution of her symptoms.ConclusionThis study describes a case of primary IgG4-RD of the petrous apex of the temporal bone that presented with diplopia and was diagnosed by middle fossa craniotomy and temporal bone biopsy. To the best of our knowledge, this is the first case description where primary diagnosis was made based on middle cranial fossa craniotomy and temporal bone biopsy.