Surgical strategy in treatment of metopic synostosis in a single centre experience: technical note and quantitative analysis of the outcomes.

PURPOSE: Trigonocephaly is the most common craniosynostosis involving orbits. Although some degree of agreement has been reached regarding surgical timing and indications for treatment, there is no consensus regarding the ideal operative technique to guarantee an optimal morphological outcome. The purpose of this study is to describe both strategies and to compare morphological outcomes by means of morphological surface analysis obtained from three-dimensional (3D) stereophotogrammetry, with two different techniques. METHODS: We retrospectively investigated 43 patients with metopic synostosis surgically treated between 2004 and 2020. Two different techniques were applied, addressed as technique A and B. Ten patients undergone postoperative 3d stereophotogrammetry were enrolled, and cephalometric measurements were taken and compared to a cohort of unaffected patients matched by age and gender. RESULTS: Comparison of the groups demonstrated a hypercorrection of the metopic angle of the second technique, associated with a slightly lower correction of the interfrontoparietal diameter. The metopic angle showed to be significantly undercorrected with the first method. CONCLUSIONS: Alternated barrel staving technique appears to be a quick and satisfactory method in cranial remodelling for metopic synostosis. It guarantees an optimal aesthetic result in the first years after surgery.

Endoscopic transventricular biopsy in brainstem diffuse midline glioma: how I do it.

BACKGROUND: Brainstem diffuse midline gliomas represent infiltrative and rare pediatric tumors with a dismal prognosis. Surgical biopsy is emerging as a valid technique to define diagnosis and molecular markers for future targeted therapies. METHOD: We describe the key steps of an endoscopic trans-ventricular biopsy of a brainstem diffuse midline glioma and associated ventriculomegaly. The relevant surgical anatomy along with an illustrative video is described. CONCLUSION: The endoscopic third ventriculostomy combined with a punch biopsy of a brainstem diffuse midline glioma associated with ventriculomegaly represent a feasible and low-risk procedure to simultaneously treat incipient hydrocephalus and molecular diagnosis for future treatment and research.

Percutaneous Transfontanellar External Ventricular Drainage in an Extremely Low Birth Weight Infant: 2-Dimensional Operative Video.

Intraventricular hemorrhage and the subsequent development of posthemorrhagic hydrocephalus (PHH) is one of the most serious complication of prematurity, especially in extremely low birth weight infants.1 Neurodevelopmental delay, epilepsy, and severe cognitive impairment represent common sequelae of PHH.2,3 A ventriculoperitoneal shunt insertion in such premature infants is associated with higher rates of skin erosion, infection, and shunt failure.4 One therapeutic option is represented by the use of temporary cerebrospinal fluid diversion procedures (such as external ventricular drainage, subcutaneous reservoir, and ventriculosubgaleal shunt) to gain time avoiding the PHH secondary damages.5,6 An extremely low birth weight (birth weight = 653 g) infant at 24 + 4 gestational age weeks presented with a grade III intraventricular hemorrhage and periventricular hemorrhagic infarction 5 days after birth. Serial transfontanellar ultrasound disclosed a progressive PHH. Progressive symptomatic PHH, pulmonary hemodynamic instability, and suboptimal general prematurity conditions were the main factors that led to plan a percutaneous transfontanellar ultrasound-guided external ventricular drainage at the neonatal intensive care unit. The illustrated procedure represents a bedside minimally invasive, effective, reversible, and sparing-time choice alternative to other temporary cerebrospinal fluid diversion techniques. This edited, 2-dimensional operative video highlights the key surgical steps of the proposed procedure (Video 1). All relevant patient identifiers have been removed from the video. Nevertheless, the parent's consent was obtained regarding the procedure, video recording, and redistribution for educational purposes.

Early de-tethering: analysis of urological and clinical consequences in a series of 40 children.

PURPOSE: Early de-tethering procedures are performed on spinal dysraphisms to prevent neuro-urological deterioration caused by growth. Partial lipoma removal may cause delayed deterioration by re-tethering, while complete removal may increase the risk of postoperative worsening. The present study evaluates the risk of postoperative deterioration and the protective potential of intraoperative neurophysiological monitoring (IOM), with a special reference to the conus lipomas treated with the radical approach. METHODS: Forty toddlers (< 24 months) underwent complete perioperative neurological and urological assessment, including urodynamic study (UDS). The dysraphisms were subgrouped according to Pang's classification. IOM was applied in all patients: transcranial motor evoked potentials (tMep) combined with mapping were recorded in all cases while bulbocavernosus reflex (BCR) was evaluable just in 7 cases. RESULTS: At preoperative evaluation, 11 children already had UDS impairment and 2 had motor disturbances before neurosurgery. At 1-month follow-up, preoperative motor disturbances were stable, 7/11 UDS alterations normalized, and the remaining 4 were stable. At 6-month follow-up, all motor deficits and 8/11 preoperative UDS alterations had improved. Unfortunately, 7 children with previously normal UDS experienced a new impairment after surgery: 2/7 normalized while 5/7 did not recover. This postoperative permanent urodynamic impairment occurred in 4 chaotic lipoma (CLchaos) and in one terminal myelocystocele (TMC) that means a surgical deterioration rate of 22% for the high risk cases. CONCLUSIONS: This small highly selected series confirms that early de-tethering may stop or revert the spontaneous neuro-urological deterioration: in fact, preoperative UDS impairment was frequent (27.5%) and improved in all the low surgical risk cases (limited dorsal myeloschisis, filar, transitional and dorsal lipomas). On the contrary, in CLchaos and TMC, early de-tethering was unable to revert preoperative UDS impairment, and radical surgery carried a high risk of new neuro-urological deterioration directly caused by the operation. In our experience, IOM had a protective role for motor functions, while it was less effective for the neuro-urological ones, probably due to the anesthesiology regimens applied. In conclusion, among the dysraphisms, CLchoas proved to be the worst enemy that often camouflages at MRI. Affording it without all possible IOM weapons carries a high risk to harm the patient.

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.