Christopher Chandler

Congenital midline cleft of the posterior arch of atlas: a rare cause of symptomatic cervical canal stenosis.

Abstract. Developmental symptomatic C1 canal stenosis is very rare. We describe the computed tomography (CT) and magnetic resonance imaging (MRI) appearances in a 8-year-old child who presented with progressive upper and lower limb neurological symptoms and in whom imaging revealed the medial posterior hemiarches of a bifid C1 to be inturned and compressing the cervical cord. This particular configuration of the posterior arch of atlas is frequently associated with other craniocervical bony anomalies and presents with neurological symptoms early in life. Early CT or MRI examination of patients with symptomatic posterior arch of C1 defects is necessary, in order to detect such an appearance, since surgical treatment may prevent neurological deterioration.

Primary spinal malignant schwannoma

Malignant schwannomas are uncommon primary tumours of nerve sheath origin. They are rarely found within the spine and spinal canal, and little is known about their management in this unusual location. We describe the presentation of three patients with primary spinal malignant schwannomas and discuss the surgical management.

Review of practices in myelomeningocoele repair at King's College Hospital, London.

Myelomeningocoele remains a significant neurosurgical challenge. We have carried out a retrospective study evaluating the practices of myelomeningocoele repair at our institute over the last 5 years. Additionally, for patients that have been followed up for at least 12 months, we have investigated parameters describing functional outcome up to this period. With the recent Management of Myelomeningocoele (MOMS) study re-igniting interest in the condition, we aim to provide contemporary practice and outcome data to add to the literature.

Assessment of diaphragm function in lumbocostovertebral syndrome

Diaphragm function testing can play a key role in determining the management of respiratory problems of infants with multiple anomalies. The diaphragm is the main respiratory muscle in infancy, thus diaphragmatic maldevelopment may cause respiratory embarrassment. Diagnosis and treatment, however, can be hindered by the lack of appropriate quantitative assessments of neonatal diaphragm function. We report the use of magnetic stimulation of the phrenic nerves to facilitate management of the persisting supplementary oxygen requirement of an infant with lumbocostovertebral syndrome. A male infant, birth weight 3.04 kg, was born at term to non-consanguineous parents. The mother had mild gestational diabetes mellitus. Antenatal ultrasound examination revealed a thoracolumbar meningocoele, an Arnold Chiari malformation and a right talipes. The infant also had a scoliosis, due to hemivertebrae between T8 and L3, a small right hemithorax with absent ribs (8 to 12) and a large lumbar hernia (Fig. 1). The infant was dependent on supplementary oxygen from birth, which persisted after the meningocoele was closed. MRI and ultrasound examination excluded a diaphragmatic hernia, but not an eventration. It was unclear, therefore, whether the supplementary oxygen requirement was due solely to the probable right lung hypoplasia or diaphragmatic malfunction was a contributory factor and the infant would benefit from a plication. Respiratory and diaphragmatic function testing was undertaken. Lung volume measurement revealed a functional residual capacity of 20 ml/kg (reference range 24–36 ml/kg), that is the infant had moderate lung hypoplasia. Diaphragm function was assessed by measurement of the maximal inspiratory pressure (MIP) and the transdiaphragmatic pressure generated by magnetic stimulation of the right and left phrenic nerves [5]. The MIP was 60 cmH2O (reference range 42–105 cmH2O) [2] and phrenic nerve stimulation revealed similar right and left transdiaphragmatic pressures (left 3.8 cmH2O; right 3.8 cmH2O). As a consequence, surgical plication of the right hemidiaphragm was deemed unnecessary. The infant was discharged home at 2 months of age still oxygen dependent. The lumbocostovertebral syndrome is rare [1] and includes hemivertebra, absent ribs, meningomyelocoele Fig. 1 Chest and abdominal radiograph taken to identify the position of the umbilical artery catheter. Note hemivertebrae, rightsided lumbar hernia and abnormal rib configuration

Silent diabetes mellitus, periodontitis and a new case of thalamic abscess

Brain abscess is an unusual complication of uncontrolled diabetes. A solitary thalamic abscess is an uncommon type of brain abscess. We report a case of thalamic abscess, whereupon diabetes mellitus and periodontitis were diagnosed. The diagnosis and management of thalamic abscess, and the interplay of type 2 diabetes and periodontitis are discussed. A 56-year-old, Caucasian, man with no medical or travel history, presented with 5-day symptoms of meningeal irritation. Body mass index 30.6 kg/m2. CT demonstrated a solitary midline lesion with neoplasia as a differential diagnosis. It was biopsied and cultures grew Streptococcus milleri. He was treated by stereotactic puncture, external drainage and targeted intrathecal and systemic antibiotic therapy. HIV negative but glycated haemoglobin (HbA1c) 10.7% (93 mmol/mol). Dental examination revealed a small molar abscess. Radiological resolution of the thalamic abscess occurred within 2 months. Diabetes improved with 7 weeks of insulin, and maintained on metformin, HbA1c 6.9% (51 mmol/mol). There was no residual neurological disability.

Traumatic Sequential Bilateral Extradural Haematomas in a Child

A five year old girl presented with increasing drowsiness, persistent headaches and vomiting following a fall one day previously. She appeared drowsy however a GCS of 15 was elicited. A computed tomography (CT) scan revealed a high density extradural haematoma centred on the right side of the posterior fossa (Fig. 1a). There was moderate hydrocephalus but no evidence of a skull fracture. The extradural haematoma was subsequently evacuated via a posterior fossa craniotomy. A bleeding source was identified at the torcula. Hitch stitches were placed around the margins and, at the end of the procedure, complete haemostasis had been achieved. Two days post operatively, the patient started to experience headaches and blurred vision. A repeat CT scan revealed a hyperdense extradural haematoma centred over the left parieto-occipital region and 4.2 cm superior to the previous haematoma (Fig. 1b). A left occipital craniotomy was performed and the haematoma was removed. A thin strip of thrombus adherent to the distal superior sagittal sinus on the left side, and distant to the site of the previous evacuation suggested the site of origin. The child made a good post operative recovery and was discharged six days later. Discussion

PP84. ENDOSCOPIC RESECTION OF A THIRD VENTRICULAR PILOCYTIC ASTROCYTOMA WITH PILOMYXOID FEATURES IN AN 86-YEAR OLD PATIENT: CASE REPORT AND REVIEW OF THE LITERATURE

AbstractOBJECTIVE: Pilocytic astrocytomas are extremely rare in octagenarians especially those occurring in the third ventricle. They are similar histopathologically to pilomyxoid astrocytomas but the latter are more aggressive. We report the case of an 86-year old patient presenting with confusion and lethargy secondary to a third ventricular lesion causing obstructive hydrocephalus. The lesion was approached endoscopically and sub-totally resected. Histologically the lesion was classified as a pilocytic astrocytoma with pilomyxoid features. The patient made a full recovery and did not require a shunt. MATERIALS AND METHODS: Case report with pictures and video of the operative approach as well as discussion of the imaging sequences and histology, and review of the literature. RESULTS: Sub-total resection of the tumour was achieved using a monoportal image-guided endoscopic approach. Normal cerebrospinal fluid circulation was restored and a shunt was not required. Histologically, the mass was confirmed to be a pilocytic astrocytoma with pilomyxoid features. The patient made a full recovery with resolution of her symptoms. No such case has been reported in the literature to date. CONCLUSION: This is the first report of a third-ventricular pilocytic astrocytomas with pilomyxoid features in an octogenarian. The endoscopic approach in the case at hand, allowed safe direct visualisation and sub-total resection of the lesion, providing histological diagnosis to guide management and obviating the need for a shunt. The minimally invasive nature of the approach translated into full recovery, despite the age of the patient.

Germinoma recurrence in the conus medullaris 12 years after remission of primary intracranial lesion in the pituitary

Intracranial germinomas are rare primary central nervous system tumours that are highly sensitive to radiotherapy and chemotherapy. Recurrences are infrequent, with the majority occurring within 5 years. Although multidisciplinary treatments have resulted in good event-free survival, long-term outcomes, over decades, are relatively poorly reported. We present a rare case of a recurrence in the conus medullaris 12 years after complete remission of the primary pituitary germinoma. To the best of our knowledge, this represents the first case of a significantly delayed spinal recurrence, occurring at a very distant and uncommon site, from the primary tumour. This case highlights the importance of long-term follow-up, even after complete remission, in order to identify and limit disability from late spinal recurrences in a relatively young patient population. We consider the literature regarding identifying high risk patients, and the role of craniospinal irradiation in limiting late spinal recurrences.