Ruptured sinus of Valsalva aneurysm is a rare entity in children. To our knowledge, this is the youngest child reported in the literature with a sinus of Valsalva ruptured aneurysm being successfully treated percutaneously using an AmplatzerTM Duct Occluder II Additional Sizes device.

We present two procedures of transcatheter closure: that of an aorto-pulmonary window in a 12-month-old infant with a body weight of 7 kilograms, and that of ruptured sinus of Valsalva aneurysm into the right atrium in an adult patient. In the first case, we applied the muscular ventricular septal defect Cardio-O-Fix, while in the other we applied the patent ductus arteriosus Cardio-O-Fix occluder. The procedures were successful in both patients, and we achieved complete closure of the unwanted shunts.

Objective: To study the adaptive potential of the right ventricular myocardium after 30 days of mechanical-induced overload in rats from two different age groups. Materials and methods: We banded the pulmonary trunk, so as to increase the systolic work load of the right ventricle, in 19 adult Sprague-Dawley rats at the age of 10 weeks, and 16 weanlings when they were 3 weeks-old, using 10 adults and 10 weanlings as controls. We analysed the functional adaptation and structural changes of the right ventricular myocardium, blood vessels and interstitial tissue after 30 days of increased afterload. Results: The increased workload induced an increase of the right ventricular weight and free wall thickness in animals from both age groups when compared to controls. These changes were mostly related to cardiomyocytic hypertrophy, as confirmed by the expression of myocardial hypertrophic markers, without any apparent increase of their number, a “reactive” fibrosis especially evident in the adult rats, with p-value less than 0.0001, and a more extensive neocapillary network in the weanlings compared to the adults aubsequent to banding, the p-value being less than 0.0001. Conclusion: In response to right ventricular afterload, weanlings showed a higher adaptive capillary growth, which hampered the development of fibrosis as seen in the adult rats. Age seems to be a risk factor for adverse structural-functional changes of right ventricle subjected to increased workload.

Multiple congenital dehiscence of the skull base is rare and can give rise to spontaneous CSF rhinorrhoea. A search of the world literature revealed only five reports of CSF leak with more than one concomitant skull base defect. When treating a patient with spontaneous CSF rhinorrhoea the possibility of its originating from the middle ear and eustachian tube should be considered. An intrathecal injection of fluorescein is useful in establishing the site of a CSF leak especially when a computed tomography scan (CT) or magnetic resonance image (MRI) has not localized the site. We discuss a case of a 72-year-old lady presenting with CSF rhinorrhoea, who had an anterior skull base defect localized with the help of intrathecal fluorescein and repaired surgically. Subsequent to this she had a further episode of CSF rhinorrhoea that originated from a middle-ear meningocele that was then repaired.

Congenital posterior laryngeal clefts are rare anomalies, being first described by Richter in 1792, and first operated upon successfully by Pettersson in 1955. Most clefts present within the first few months of life, with only a few cases being documented as presenting in adulthood. We present the case of a 50-year-old lady presenting with a previously undiagnosed posterior laryngeal cleft with an associated tracheoesophageal fistula. The defect was graded as 2 on the Benjamin and Inglis classification system of posterior laryngeal clefts. Following a literature review, we believe that our patient is the oldest to present with this congenital defect.

While evaluating a 61-year-old patient for stridor we incidentally detected a double epiglottis. The patient was also diagnosed of having Weyer's acrofacial dysostosis which is characterized by hexadactyly affecting all four extremities, small and deeply set nails, dental deformities with small, conical teeth and mandibular hypoplasia. The double epiglottis was not the cause for the stridor. Because of the covert symptomatology of double epiglottis it is suggested that the association with Weyer's syndrome is common. Embryological evidence and a review of the literature on laryngeal abnormalities is discussed.

Over a four-year period 72 children with ear abnormalities have been referred for assessment by the extraoral osseointegrated implant team at The Queen Elizabeth Hospital, Birmingham. Thirty-two children have been judged suitable for rehabilitation. Twelve children have completed rehabilitation using bone-anchored hearing aids and/or auricular prostheses. Two fixtures (seven per cent of those loaded) have dislodged and required replacement. Audiological assessment of the bone-anchored hearing aid users shows only small improvements in their aided thresholds, compared to thresholds obtained with their previous aid. However all now have thresholds of 30 dB(A) or better and report a marked improvement in sound quality. When surveyed, hearing aid and prosthesis users report high levels of satisfaction with this form of rehabilitation. The technique adds a new dimension to the management of children with aural anomalies. The approach and results of a multidisciplinary programme are reported.

There have been few accounts of multi-channel cochlear implants in patients with congenital structural deformities of the inner ear which are associated with severe and sometimes progressive deafness. These malformations can now be recognized easily on 2 plane thin section high resolution CT studies which are mandatory for the pre-implantation assessment. However, no attempt seems to have been made to describe which of these malformations would be suitable for an implant or for which would this procedure be contra-indicated. True Mondini deformity of both the cochlea and dilated vestibular aqueduct type would appear suitable for a multi channel implant, but this type of implant should not be used for a primitive otocyst, severe labyrinthine dysplasia or the characteristic X-linked deformity.