Pulmonary atresia with abnormal ventriculoarterial connections in the presence of an intact ventricular septum is rare even in postnatal life. Two cases are described in which this combination of lesions was diagnosed in previable fetuses at autopsy. Both pregnancies were terminated for gross structural abnormalities discovered on antenatal ultrasound scans. In the first case, the pulmonary atresia was associated with intact ventricular septum, a double outlet left ventricle and hypoplasia of the tricuspid valve, right ventricle and arterial duct, a combination of lesions that would appear to be unique. In the second case, there was complete transposition and a hypoplastic left ventricle. The severity of the cardiac lesions in both fetuses was such that it is unlikely that either would have survived to term. In the first case no other malformation was present, but in the second there was also a large encephalocoele secondary to early amnion rupture, and a single umbilical artery. The association of the cardiac lesion with early amnion rupture in this second fetus would appear to be a chance event.

We report the case of a 43-year-old woman who presented with a spontaneous pneumocephalus, 37 years after a mastoidectomy. Clinical examination showed a cerebrospinal fluid leak, meningeal herniation in the superior part of the middle ear, and an audible noise from her ear when she stood up due to the entrance of air into the cranium. A computed tomography scan and magnetic resonance imaging showed the complete destruction of the tegmen tympani and the pneumocephalus in the temporal lobe. The patient underwent an emergency operation via a double middle-ear and subtemporal approach. The meningoencephalocoele and pneumocephalus were probably due to long term pressure upon too thin a tegmen tympani.

Pneumocephalus should be considered as a potential delayed post-operative complication of middle-ear surgery. Computed tomography and magnetic resonance imaging scanning supply accurate information and enable a planned surgical approach; they also allow a pathophysiological understanding and a correlation between the clinical signs and the radiological and peri-operative findings.

We report a case of post-operative frontal basal encephalocoele evaluated using a new magnetic resonance imaging (MRI) sequence, fast inversion recovery for myelin suppression (FIRMS). FIRMS was developed to enhance the differentiation between grey and white matter. In this case, the sequence was beneficial in distinguishing the encephalocoele from adjacent nasal mucosa and secretions.

We present an unusual case of generalized erosion of the skull base. We have not found a similar case reported in the world literature. The presenting symptom was spontaneous cerebrospinal fluid (CSF) rhinorrhoea which arose from a bony defect associated with herniation of the right temporal lobe into the sphenoid sinus. We discuss the management of such a case including imaging of the skull base and the endoscopic repair of the bony defect.