Shunted Hydrocephalus: Who Has More Chances to Get Rid of the Shunt?

OBJECTIVE/BACKGROUND: The natural history and the outlook of patients with hydrocephalus are dramatically affected using cerebrospinal fluid shunts. The several issues related with a long-standing shunt should suffice to justify all the possible attempts to free the patient from it. This study focused on the role of secondary endoscopic third ventriculostomy (ETV) in the achievement of shunt removal in cases of shunt malfunction, and to identify patients who could benefit most from the procedure. METHODS: In the period of 2006-2015, ETV was attempted in 47 patients >6 months old with ventriculoperitoneal shunt malfunction who presented with increased ventricle size compared with the previous neuroradiological examinations; simultaneously the shunt was removed or ligated. RESULTS: The overall success rate of secondary ETV was 74% (shunt-free patients with normalized intracranial pressure and absence of symptoms attributable to hydrocephalus) in patients with a long shunt duration (up to 30 years). The number of previous shunt revision procedures (P = 0.026) and lower age (P = 0.017) correlate with the likelihood of secondary ETV failure, a score of 80 as ETV success score (calculated for both pediatric and adult patients, even if the score was meant for the pediatric population) correlates with secondary ETV success (P = 0.014). CONCLUSIONS: Many patients with shunt malfunction can benefit from secondary ETV even after decades of shunting. Age at secondary ETV, the number of previous shunt revisions, and the ETV success score can help to better identify the best candidates for the procedure.

Higher Failure of Endoscopic Third Ventriculostomy in Infants: The "Distensible" Skull Is the Culprit.

The efficacy of endoscopic third ventriculostomy (ETV) in childhood strongly correlates with age. Reasons for the higher failure rate in infants are under debate but the "distensible" head with its immature skull (which would prevent an adequate flow through the ETV and/or a sufficient pressure gradient between the cortical subarachnoid spaces and the venous sinuses) has been suggested as a possible cause. Our evidence of spontaneous conversion of a failed, but always patent, third ventriculostomy into an effective one occurring in a child after maturation of the skull supports this hypothesis.

Introduction of percutaneous-tunneled transfontanellar external ventricular drainage in the management of hydrocephalus in extremely low-birth-weight infants.

OBJECTIVE Hydrocephalus treatment in extremely low-birth-weight (ELBW) infants still represents a challenge for the pediatric neurosurgeon, particularly when the patient weighs far less than 1000 g. In such cases, the benefits in terms of neurological outcome following early treatment do not always outweigh the surgical risks, especially considering the great difference in the surgical risk before patient weight increases. To assess the efficacy and reliability of a percutaneous-tunneled, transfontanellar external ventricular drain (PTTEVD) in ELBW infants, the authors started a new protocol for the early surgical treatment of hydrocephalus. METHODS Ten cases of posthemorrhagic hydrocephalus (PHH) in ELBW infants (5 cases < 700 g, range for all cases 550-1000 g) were treated with a PTTEVD that was implanted at bedside as the first measure in a stepwise approach. RESULTS The average duration of the procedure was 7 minutes, and there was no blood loss. The drain remained in place for an average of 24 days (range 8-45 days). In all cases early control of the hydrocephalus was achieved. One patient had a single episode of CSF leakage (due to insufficient CSF removal). In another patient Enterococcus in the CSF sample was detected the day after abdominal surgery with ileostomy (infection resolved with intrathecal vancomycin). One patient died of Streptococcus sepsis, a systemic infection existing prior to drain placement that never resolved. One patient had Pseudomonas aeruginosa sepsis prior to drain insertion; a PTTEVD was implanted, the infection resolved, and the hydrocephalus was treated in the same way as with a traditional EVD, while the advantages of a quick, minimally invasive, bedside procedure were maintained. Once a patient reached 1 kg in weight, when necessary, a ventriculoperitoneal shunt was implanted and the PTTEVD was removed. CONCLUSIONS The introduction of PTTEVD placement in our standard protocol for the management of PHH has proved to be a wise option for small patients.

Seizure outcome of surgical treatment of focal epilepsy associated with low-grade tumors in children.

OBJECT: Low-grade tumor (LGT) is an increasingly recognized cause of focal epilepsies, particularly in children and young adults, and is frequently associated with cortical dysplasia. The optimal surgical treatment of epileptogenic LGTs in pediatric patients has not been fully established. METHODS: In the present study, the authors retrospectively reviewed 30 patients (age range 3-18 years) who underwent surgery for histopathologically confirmed LGTs, in which seizures were the only clinical manifestation. The patients were divided into 2 groups according to the type of surgical treatment: patients in Group A (20 cases) underwent only tumor removal (lesionectomy), whereas patients in Group B (11 cases) underwent removal of the tumor and the adjacent epileptogenic zone (tailored surgery). One of the patients, who underwent 2 operations, is included in both groups. Follow-up ranged from 1 to 17 years. RESULTS: Sixteen (80%) of 20 patients in Group A had an Engel Class I outcome. In this group, 3 of 4 patients who were in Engel Classes II and III had temporomesial lesions. All patients in Group B had temporomesial tumors and were seizure free (Engel Class I). In this series, in temporolateral and extratemporal tumor locations, lesionectomy yielded a good seizure outcome. In addition, a young age at seizure onset (in particular < 4 years) was associated with a poor seizure outcome. CONCLUSIONS: Tailored resection in temporomesial LGTs was associated with excellent seizure outcome, indicating that an adequate presurgical evaluation including extensive neurophysiological evaluation (long-term videoelectroencephalography monitoring) to plan appropriate surgical strategy is advised.

Second-look surgery for ependymoma: the Italian experience.

OBJECT: Complete ependymoma resection ensures a better prognosis for children with this tumor, but the complete excision of infratentorial ependymomas involves serious risks. Second-look surgery for tumor remnants may be less harmful and enable complete removal. There is a potential, although still unclear, role for neoadjuvant chemotherapy in preparation for further surgery. METHODS: Since 1994, the authors have adopted two successive protocols for intracranial ependymoma, both including a phase of adjuvant chemotherapy for children with surgical tumor remnants with a plan for potential second-look surgery before radiotherapy. RESULTS: In the first protocol, 9 of 63 children underwent further surgery, and 6 became tumor free with no additional sequelae. Their prognosis for progression-free survival and freedom from local relapse was comparable to that of children who were operated on only once. In the second protocol, efforts were made to achieve complete resection and 29 of 110 patients underwent reoperations: 9 after the first surgery, 17 after chemotherapy, and 3 soon after radiotherapy. Fourteen of the 29 patients became tumor free, 1 of them with worsening neurological symptoms. The outcome of the 66 patients who became tumor free after 1 operation was compared with that of the 14 who became tumor free after reoperation. The 3-year progression-free survival of the 66 patients compared with the 14 other patients was 71.4% ± 6.9% and 90% ± 9.5%, respectively; the 3-year freedom from local relapse was 84.7% ± 5.9% and 90% ± 9.5%, respectively; and the 3-year overall survival was 85.9% ± 5.4% and 87.5% ± 11.7%, respectively. CONCLUSIONS: Second-look surgery proved feasible with no major morbidity, and results improved with time. Local tumor control was comparable in patients undergoing 1 or more resections.

Congenital cystic eye: from prenatal diagnosis to therapeutic management and surgical treatment.

Only 28 cases of congenital cystic eye have been reported in the literature. The main issue in such cases is differential diagnosis between this malformation and different cystic malformations and masses of the orbital cavity and eyeball, the most common of which is microphthalmia with cyst. Both malformations arise from incomplete closure of the fetal optic vesicle in different stages of embryonic development. We present a case of congenital cystic eye, associated with coloboma and corneal dermoid of the fellow eye and with left brachiocephaly, discussing differential diagnosis with microphthalmia with cyst and illustrating the treatment we planned and performed. The patient first underwent a surgical excision of the left corneal dermoid, then a resection of the right orbital cyst. The last step was to perform a craniotomy and cranial vault remodeling. All the operations were planned and performed using a team approach. The team comprised an ophthalmologist, a plastic surgeon, and a neurosurgeon, and the result was a successful outcome.

Cavernous malformations of the central nervous system in the pediatric age group.

OBJECTIVE: The main clinico-diagnostic features, risk factors and associated diseases of cavernous malformations (CMs), also called cavernous angiomas or cavernomas, of the central nervous system (CNS) in children are described, and the most relevant differences compared to the affected adult population are pointed out, focusing on the management of pediatric patients harboring cranial and spinal CMs. MATERIALS: This was a retrospective study of a series of 42 children symptomatic for CMs of the cranial and spinal compartments (35 supratentorial brain lesions, 5 infratentorial and 2 in the spinal region) operated on between 1975 and 2005, with a clinical follow-up ranging from 12 to 192 months. The results were compared with those found in the most recent literature dealing with pediatric CMs of the CNS. RESULTS: Surgical treatment produced excellent or good results in 69% of our 42 children. Unchanged neurological deficits were observed in 23.8% of cases, while morbidity from surgical procedures was 7.14%. Mortality was absent in this series. These surgical results are comparable with those found in the literature, where morbidity and mortality rates from surgery are 8.8 and 1.13%, respectively, and are mostly associated with procedures for the excision of deep, critically located cavernomas. CONCLUSION: CMs represent the most common CNS vascular lesion in children, although their incidence is 4 times lower than that of the adult population. The natural history of pediatric CMs throughout the neuraxis seems to be more aggressive than in adult patients; these lesions have higher rates of growth and hemorrhage, larger dimensions and often atypical radiological pictures at diagnosis. Beside the familial form of the disease, which is more often associated with multiple lesions and an earlier age of clinical presentation, the major risk factor for CMs in children seems to be radiotherapy for CNS tumors. Furthermore, a greater number of CMs coexistent with mixed angiomatous lesions have been reported in children than in adults. Surgical results are related to the preoperative neurological status of the children; symptomatic patients who are operated on early, before they develop severe neurological deficits or long-standing seizures, may achieve the best clinical outcome. Radiosurgery does not seem to be advisable in children as an alternative treatment for deep CMs or those causing epilepsy.

Endoscopic endonasal odontoidectomy in a patient affected by Down syndrome: technical case report.

OBJECTIVE: Atlantoaxial instability and odontoid invagination may occur in Down syndrome. The treatment of this lesion may require an odontoidectomy. In similar circumstances, a transoral procedure may be hampered by a macroglossia which is part of Down syndrome. In a situation occurring in an 11-year-old child affected by atloaxial instability and os odontoideum, the persistence of ventral compression after a previous atloaxial fixation induced us to perform an endoscopic endonasal odontoidectomy. We feel that this is an elective indication for endoscopic endonasal odontoidectomy. CLINICAL PRESENTATION: An 11-year-old child, affected by Down syndrome, presented with progressive tetraparesis with severe superior diplegia. He was wheelchair-bound. Recently, respiratory disturbances had appeared. INTERVENTION: The patient underwent an endoscopic endonasal odontoidectomy. The postoperative course was uneventful, and the patient experienced improvement of the preoperative symptoms. CONCLUSION: An endonasal technique is indicated for patients presenting with narrowness of the oral cavity, such as in the case reported, where the narrowness due to pediatric age and the macroglossia characteristic of Down syndrome hamper the transoral approach.

Combined endoscopy-assisted cranionasal approach for resection of infantile myofibromatosis of the ethmoid and anterior skull base. Case report.

The advent and widespread development of endonasal endoscopic techniques have recently expanded the frontiers of skull base surgery. The reduced invasiveness, wider and adjustable visualization of the operative field, and lack of postoperative cosmetic defects are well-known advantages of the endonasal endoscopic approaches compared with traditional surgical exposures both in adults and in children. The need to avoid disruption of facial growth centers and permanent tooth roots represents a further special consideration in favor of these endoscopic techniques in children. The authors report on a case of solitary myofibroma involving the ethmoid, mesial orbits, and anterior skull base with intracranial intradural expansion in a 17-month-old girl. The occurrence of such proliferative disease along the skull base is exceedingly rare. The tumor was successfully excised via an endoscopy-assisted cranionasal approach in which a transcranial microsurgical exposure was combined with endonasal endoscopic access to ensure a radical resection and optimize skull base reconstruction. To the authors' knowledge, the patient in this case is the youngest reported patient in the literature who has undergone treatment with this surgical strategy. The outcome in this patient underscores the feasibility and safety of endoscopic endonasal surgery even in toddlers and early childhood.

Seizure outcome of lesionectomy in glioneuronal tumors associated with epilepsy in children.

OBJECT: Glioneuronal tumors (ganglioglioma, dysembryoplastic neuroepithelial tumors [DNTs]) are commonly associated with partial seizures. The optimal surgical treatment of such tumors, however, has not been fully established; it is still unclear whether lesionectomy itself can be used to control seizures or if epileptogenic areas adjacent to the tumor should also be removed. To address this uncertainty, the authors analyzed seizure outcome in a series of children with epileptogenic glioneuronal tumors that had been treated only by lesionectomy. METHODS: The authors retrospectively reviewed 15 children surgically treated for glioneuronal tumors associated with epilepsy. Patients ranged in age from 3 to 18 years (mean 12.6 years); there were 12 boys and three girls. The interval between onset of seizures and surgery ranged from 0.5 to 16 years (mean 6.1 years). Ten patients (66.6%) suffered complex partial seizures and five (33.3%) simple partial seizures. Seizure frequency varied from several per day to one per month. Nine tumors (60%) were temporal and six extratemporal; in all patients resection was limited to the tumor. The follow-up duration ranged from 1 to 11 years (mean 5.6 years). Gross-total removal was achieved in 13 patients and subtotal in two. The histological diagnosis was ganglioglioma in 11 cases and DNT in four. At last follow up 13 patients (86.6%) were Engel Class I, one was Engel Class II, and one was Engel Class III. CONCLUSIONS: The results of this study indicate that lesionectomy may provide good long-term seizure control in the majority of children with epileptogenic glioneuronal tumors.