Craniosynostosis is a developmental craniofacial anomaly, resulting in impairment of brain development and abnormally shaped skull. The main cause of craniosynostosis is premature closure of one or more cranial sutures. It usually occurs as an isolated condition, but may also be associated with other malformations as part of complex syndromes. When left untreated, craniosynostosis can cause serious complications, such as developmental delay, facial abnormality, sensory, respiratory and neurological dysfunction, anomalies affecting the eye, and psychological disturbances. Thus, early diagnosis, expert surgical techniques, postoperative care, and adequate follow-up are of vital importance in treating craniosynostosis.
Some recurrent neuroradiological alterations can be found in 9p deletion syndrome. The presence of these signs on MRI of a trigonocephalic patient should raise the suspicion of an underlying chromosomal alteration, such as the 9p deletion syndrome and prompt genetic investigations.
BACKGROUND
The vein of Galen aneurismal malformations (VGAM) are rare arteriovenous malformations of the embryonic choroid plexus. They represent about 30% of paediatric neurovascular disorders and show diverse characteristics. The VGAM is constituted by a midline dilated venous structure that receives blood from abnormal macroscopic or microscopic arteriovenous shunting vessels. Two types of VGAM exist, the choroidal and the mural. The treatment represents a challenge with the therapeutic objective to preserve the normal brain development without creating new neurological deficits.
CASE SUMMARY
We present three cases of VGAM in the early postnatal period and their treatment. All patents were treated with the endovascular technique, which was successful. According to our experience, the endovascular technique is a safe and efficient mode of VGAM treatment.
CONCLUSION
The objective of treatment aims to child’s normal neurological development. A proper selection of patients and a thorough diagnostic workup is of vital importance. When the endovascular treatment is performed, the primary aim is not a complete VGAM exclusion at one time, which could produce a sudden reversal of blood flow with consequent venous infarction and ischemia. The aim is therefore to occlude as much of the VGAM as needed to relieve the congestive cardiac failure, to gain time and to create the conditions for a normal maturation of the neurovascular system. With the use of endovascular techniques, which represent not only the first choice of treatment but also the only safe therapeutic modality, the natural history of VGAM and their risks may be avoided safely.
BACKGROUND
The endoscopic third ventriculostomy (ETV) is a neuroendoscopical procedure that represents a more suitable alternative to the extracranial shunting. It consists of fenestrating the floor of the third ventricle and thus establishing a free flow of the cerebrospinal fluid from the ventricles to the site of resorption in the subarachnoid space. It offers a more physiological solution and a chance at a shunt-free life for children with hydrocephalus. The main indication for the procedure is obstructive hydrocephalus, however, it can also be useful in patients with other forms of hydrocephalus.
CASE SUMMARY
We present a treatment flow of a 9-year-old patient, diagnosed with an obstructive hydrocephalus due to tectal glioma that was successfully treated with an ETV. We review the important factors influencing the success rate such as age, aetiology, shunt history, preoperative planning and visualisation of the basilar artery.
CONCLUSION
Even though the ETV effectively controls obstructive hydrocephalus in more than 75% of all cases, the overall success rate of the procedure varies and could be approved by the correct preoperative patient selection.
Radical excision of lipoblastomas is the gold standard but is not always possible to achieve. Residual lesions can be managed conservatively, on the basis of the benign nature of the tumor and of its potential maturation to normal fat. Recurrence of lipoblastoma appears in 15 % of non-radical excisions but has not yet been described in the form of lipoblastomatosis. A second surgery is indicated if the tumor mass reaches large dimensions, if it increases in size, and if it threatens the functionality of vital organs such as the spinal cord.
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