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.

Can GH Therapy Worsen a Clinically Silent Chiari Malformation? A Case Report and Systematic Literature Review.

BACKGROUND: The link between the effects of recombinant human growth hormone (rhGH) therapy in patients with growth hormone deficiency (GHD) and Chiari malformation type I (CM-1) is controversial. SUMMARY: We report the case of a patient with an unusual association of GHD due to ectopic posterior pituitary and CM-1. Our patient developed a headache and worsening of CM-1 after the initiation of rhGH therapy. Following an atlo-occipital decompression surgery, the patient was able to resume therapy with a marked growth improvement. Based on this observation, we provide a systematic review of the current literature about these two pathologies. KEY MESSAGES: A careful follow-up of all patients with CM-1 treated with GH is mandatory, paying particular attention to the appearance of any neurological signs and symptoms.

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.

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.

Endoscopic treatment of a fourth ventricle arachnoid cyst via the third ventricle: a case report.

BACKGROUND AND IMPORTANCE: Arachnoid cysts within the fourth ventricle have rarely been reported in the literature. Different procedures have been performed to restore a normal cerebrospinal fluid dynamic or pressure, including shunting and partial or complete excision of the cyst by open microsurgery. Cerebrospinal fluid shunts give only partial improvement of symptoms and are prone to malfunctions. The microsurgical excision of the cyst seems to offer the best chance of success. CLINICAL PRESENTATION: We report the case of a fourth ventricle arachnoid cyst successfully treated with a complete endoscopic cerebral procedure via the third ventricle. CONCLUSION: Endoscopic fenestration of fourth ventricle arachnoid cysts may be considered an effective neurosurgical treatment.