Archives of Pediatric Neurosurgery

Mercedes Benz pattern craniosynostosis associated with Chiari malformation type 1: A case report of a rare condition

Leonam de Oliveira Silva — 2024-07-15

Introduction: Craniosynostosis results from the premature fusion of cranial sutures and is classified as complex when it affects multiple sutures. The abnormal and rare fusion of the sagittal and bilateral lambdoid sutures is known as the Mercedes-Benz (CMB) pattern responsible for up to 0.7% of craniosynostoses. It's characterized by flattening of the occipital bone and sub-lambdoid depression. Additionally, the Arnold-Chiari malformation, one of the anomalies associated, occurs due to the junction of deformities in the posterior fossa and rhombencephalon. Type 1 (CM1) is the most incident and less severe, represented by the pointed shape of the cerebellar tonsils.

Objective: The objective of this article is to report a rare case presentation of Mercedes Benz pattern Craniosynostosis associated with Arnold Chiari malformation

Case presentation: Male patient, 11 months old, diagnosed with CMB associated with CM1, presenting with anterior turribrachycephaly and occipital brachycephaly. The diagnosis was made after the 10th month of life, with typical findings on computed tomography and cerebellar tonsil herniation (CTH) on magnetic resonance imaging (MRI). No signs of neurodevelopmental delay were observed, and there is no history of cranial malformations in the family. At 11 months, the patient underwent neurosurgery involving cranial parietal remodeling to correct craniosynostosis, along with posterior fossa decompression to address CM1.

Conclusion: Neurosurgical techniques like parietal remodeling and posterior decompression, despite their complexity, play a central role in addressing CMB. It's crucial to discuss this rare condition to ensure early diagnosis and treatment, facilitating an efficient approach and preventing potential later consequences.

Chronic recurrent multifocal osteomyelitis: A challenging diagnosis

Tatiana Protzenko — 2024-09-01

Introduction. Chronic recurrent multifocal osteomyelitis (CRMO) is a pediatric autoinflammatory disorder that is characterized by multiple sterile inflammatory bone lesions with a relapsing and remitting course. CRMO belongs to the autoinflammatory family of rheumatologic disorders based on absence of significant titers of autoantibodies and autoreactive T-lymphocytes. In absence of pathognomonic clinical, radiographic or pathological features, diagnosis can be challenging.

Case presentation. We report an unusual case of a 3-year-old girl presenting back pain, neurogenic bladder and syringomyelia. A tethered cord was diagnosed and treated, improving urinary function. Despite the surgical treatment, back pain was persistent. A whole body MRI showed multiple lesions compatible with chronic recurrent multifocal osteomyelitis.

Conclusion. CRMO shares imaging features with other diseases. It is important for neurosurgeons and radiologists to be able to differentiate other diseases from CRMO because prognosis varies from completely benign to frankly malignant.

Biparietal expansion with partial suturectomy of the sagittal suture and linear frontal osteotomies for the scaphocephaly correction in children over 12 months of age

Antonio Heredia-Gutierrez — 2024-09-01

Introduction: Sagittal craniosynostosis (SC) has a prevalence of 1: 2500 live births and represents 40-60% of all craniosynostosis. The objective of this work is to share the preliminary results, with the use of the surgical technique mentioned above for the treatment of SC in children older than 12 months.

Methods: A retrospective study from April 2019 to September 2023. Four patients with a diagnosis of non-syndromic scaphocephaly were evaluated, who met the following inclusion criteria: (i) age over 12 months, (ii) CT scan with 3D reconstruction, (iii) scaphocephaly due to postoperative restenosis and (iv) patients operated on with this surgical technique.

Results: There were 2 females (50%) and 2 males (50%), with an average age of 39.25 months. All four patients had complete closure of the sagittal suture; there was no evidence of entrapment of the superior sagittal sinus by the stenosed suture. Papilledema was evaluated with the modified Frisén scale. All four patients had grade 2 bilateral papilledema preoperatively and this reversed 6 months postoperatively. The cranial index (CI) preoperatively was 64 (range 58-72) and postoperatively 72.25 (range 66-83). There was no need to reoperate on any patient and we had no complications

Conclusion: This surgical technique is presented as an option for the treatment of scaphocephaly in children older than 12 months, and in scaphocephaly due to postoperative restenosis, proving to be a reproducible and effective procedure to achieve adequate brain decompression and reach satisfactory aesthetic results.

Pre- and postoperative intracranial volumes in children with non-syndromic craniosynostosis using 3D volumetric technique

Gustavo Sampaio — 2024-09-01

Introduction: Corrective surgery for craniosynostosis presents several challenges to students during their training, with obtaining practical experience being one of the main obstacles. The neurocranium is formed by the bony structures that surround the brain, thus ensuring its protection. During the first years of life, the baby's skull grows exponentially. Understanding the normal growth and development of the cranial shape is essential for monitoring cranial development, detecting abnormalities, and evaluating the long-term results of craniosynostosis surgery.

Objectives: To study the cranial volume gained after surgical treatment of craniosynostosis using 3D printing technology.

Methodology: 26 patients who underwent craniosynostosis surgery, from 2019 to 2022 were selected. Preoperative, immediate, and late postoperative (3 months) tomography examination was performed. Reconstructing the exams using the Blender program with cranial volume calculation. 3D printing of skulls using Sethi 3D printer. Evaluation of results using Student's t-test with independent samples.

Results: The study involved 26 patients, with ten presenting scaphocephaly treated with Renier's "H" cranial remodeling, five with trigonocephaly, five with plagiocephaly, and two with brachycephaly treated with front-orbital advancement (FOA). Cranial volumes were measured before and after surgery. For patients undergoing Renier's "H" treatment, there was an average increase of 224 cm³ between the late postoperative and preoperative stages, with smaller differences between the immediate postoperative and preoperative stages. For those treated with FOA, the average difference between late postoperative and preoperative stages was 138.8 cm³, while between immediate postoperative and preoperative stages was 129.7 cm³.

Conclusion: Cranial volume gained by patients undergoing Renier's h technique and Fronto-orbital advancement is significant, showing that it may be possible to use less invasive techniques to take advantage of patients' natural volumetric gain.

Distraction osteogenesis in craniosynosotosis: technical note

João Pedro Leite Pereira — 2024-09-01

Introduction: Craniosynostosis is a group of diseases whose main characteristic is the premature fusion of one or more cranial sutures. They can be subdivided into non-syndromic and syndromic. The posterior vault distraction osteogenesis (PVDO) has become the standard procedure for managing the intracranial hypertension of these patients. The objective of this paper is to report the experience of the Sobrapar Hospital (Campinas, Brazil) in treating and following up on syndromic craniosynostosis patients subjected to PVDO and highlight the lessons learned throughout this process.

Methods: The medical records of the patients treated at the Sobrapar Hospital between 2013 and 2023 were reviewed.

Results: The total of patients initially candidates to PVDO was 79, and 75 of them proceeded to the surgery. The mean age of surgery was 8,16 months old (ranging from 4 months to 12 years old). The length of stay ranged from 2 to 14 days, with a mean of 3,29 days. The distraction achieved ranged from 13 mm to 30 mm, with a mean distance of 23 mm. The postoperative complications were mostly cutaneous. CSF leak occurred in 3 patients.

Conclusion: PVDO provides a progressive formation of a vascularized bone, its intracranial volume expansion is greater than the FOA, increasing the posterior fossa’s size (even when the craniotomy is supratorcular) and it improves Chiari’s symptoms, even when the radiologic abnormalities are not necessarily solved. A specialized and multidisciplinary team is essential to improving the success rates of the surgery.

Prognostic and post-surgical updates in the treatment of non-syndromic craniosynostosis: a systematic review

Brenda Martins da Silva — 2024-09-01

Introduction: Craniosynostosis represents a rare congenital condition characterized by the premature fusion of one or more cranial sutures. The majority of cases of this disorder are non-syndromic in nature.

Objective: This study aims to conduct an analysis and comparison of available surgical techniques, evaluating their respective postoperative outcomes in the treatment of non-syndromic craniosynostosis. The focus lies on assessing functional, aesthetic, and neurological outcomes.

Methods: A systematic review was undertaken, encompassing the search for clinical trials (including randomized trials), systematic reviews, and meta-analyses available online in both Portuguese and English languages. The search terms "prognosis" and "non-syndromic craniosynostosis" were utilized along with the boolean operator "AND". Articles published between 2018 and 2023 were filtered through the Virtual Health Library (VHL) and PubMed platforms, specifically targeting studies presenting prognostic data related to craniosynostosis in pediatric patients up to 12 months of age.

Results: The most favorable prognostic outcomes identified in this investigation were associated with conventional cranioplasty and minimally invasive endoscopic craniectomy techniques. Notably, conventional cranioplasty demonstrated superior intellectual outcomes among patients undergoing this procedure, with an average intelligence quotient of 111.7 ± 13 and academic performance aligning closely with national averages across various domains. Conversely, minimally invasive endoscopic craniectomy exhibited low rates of blood transfusion, complications, and reoperations.

Conclusion: The findings underscore the superior cognitive prognosis associated with conventional cranioplasty, positioning it as a highly applicable surgical option for addressing craniosynostosis, supported by robust evidence.

The impact of neurosurgery on the neuropsychological development of patients with craniosynostosis: A scoping review

Jéssica Luchi Ferreira — 2024-09-01

Introduction: Neuropsychological assessment evaluates the cognitive profile by identifying deficits and enabling monitoring. At HRAC-USP, patients with craniosynostoses undergo this assessment due to impacts on neuropsychomotor development (DNPM). These conditions may cause cranial fusion, restricting brain growth and adversely affecting cognitive functions. Evaluation is crucial for guiding therapeutic and surgical approaches and ensuring better outcomes. Objective: To identify how surgical procedures impact the neuropsychological profile of patients with syndromic and isolated craniosynostoses through a scoping review.

Methodology: This scoping review was conducted in March 2024 using PubMed, Embase, BVS/Lilacs, and Scopus databases; 1,215 articles were found and 18 original articles (E1-18) in English were selected that addressed the question: "What is the impact of surgery on neuropsychological aspects in patients with craniosynostoses?"

Results: Studies on craniosynostosis highlighted the predominance of isolated cases (83.3%), with surgeries performed before one year of age associated with better cognitive outcomes. The cranial expansion technique (38.8%) was effective in non-syndromic cases, while cranial remodeling showed varied results. After surgery, there were overall improvements in DNPM, but also risks of language delay and motor deficits, especially in patients operated on at older ages. Conclusion: Surgery for craniosynostosis can improve neuropsychological outcomes, especially when performed early and with appropriate surgical techniques. More invasive procedures may lead to better outcomes, including increased Total Intelligence Quotient and better academic performance. It is crucial to consider such factors to optimize outcomes, reduce neurodevelopmental risks, and continuously monitor language and executive functions.

Posterior cranial vault distraction osteogenesis: a literature review

Aline Rabelo Rodrigues — 2024-09-01

Introduction:
Craniosynostosis is a congenital condition where one or more cranial sutures close prematurely, limiting skull growth and causing cranial deformities. Posterior cranial vault distraction (PCVD) is used to relieve intracranial pressure and improve cranial aesthetics. Its incidence is about 1 in 2000–2500 live births.

Methods:
This systematic review followed PRISMA guidelines. We searched PubMed, Scielo, and Lilacs for studies on PCVD in craniosynostosis, focusing on success, morbidity, and mortality. Six articles were included in the final review. Data were extracted and risk of bias was assessed using the ROBINS-I tool.

Results:
The included studies reported significant increases in intracranial volume (144 mL to 281 mL) post-PCVD. Most patients showed stable results without recurrence. Complications included wound infection (29%) and cerebrospinal fluid leaks, though few required re-operations. PCVD improved intracranial pressure symptoms in most cases.

Conclusion:
PCVD is an effective technique for treating craniosynostosis, increasing intracranial volume and improving symptoms of intracranial hypertension. Despite some complications, it remains a reliable and stable alternative for cranial expansion.

Hydroxiapatite morceau at frontal advancement: techinical note

Tiago de Paiva Cavalcante — 2024-09-01

Introduction: In this work, we demonstrate a modification in the frontal advancement technique, using a hydroxyapatite “morceau”, through a series of cases.

Technical note: The technical improvement consists of applying a piece of solid hydroxyapatite, freely cut and molded, under the fronto-orbital band, ensuring the advancement of the band itself, as well as facilitating early reossification. The service's experience, through a series of 20 cases, reflected good outcomes, guaranteed by frontal reossification in all cases. There were no complications associated with the new technical stage in any of the cases in the series.

Conclusion: Therefore, the results favor the use of hydroxyapatite Morceaux as an advantageous strategy for the treatment of craniostenosis with frontal advancement.

Advancing Neurosurgery Through Translational Medicine: The Modern Surgeon's Role

Ricardo Santos de Oliveira — 2024-09-01

In the dynamic field of neurosurgery, the concept of translational medicine plays a pivotal role in bridging the gap between scientific discoveries and clinical applications. This editorial delves into the evolving landscape of neurosurgery, highlighting the integral role of modern neurosurgeons in research, learning, and patient care.

Translational Medicine: Connecting Bench to Bedside

Translational medicine refers to the process of applying findings from basic science research to enhance human health and well-being. In neurosurgery, this often involves integrating insights from disciplines like neuroscience, genetics, and biomedical engineering into clinical practice. For example, discoveries in neuroimaging techniques or molecular biology can lead to novel treatments for neurological disorders.

The Modern Neurosurgeon: A Scientist and Clinician

Today’s neurosurgeon is not merely a technician but a scientist at heart. Engaging in research is not just an academic pursuit but a fundamental aspect of enhancing patient care. By actively participating in or leading research initiatives, neurosurgeons contribute to expanding the boundaries of knowledge and improving treatment outcomes. They collaborate with basic scientists, engineers, and other medical specialists to innovate and develop new therapies.

Learning and Innovation

Continuous learning is ingrained in the ethos of neurosurgery. Advances in technology and surgical techniques require neurosurgeons to stay abreast of the latest developments through conferences, workshops, and lifelong learning. Moreover, the feedback loop between research and clinical practice fosters an environment of continuous improvement. Lessons learned from patient outcomes inform future research directions, creating a virtuous cycle of innovation.

Patient-Centric Care

At its core, neurosurgery is about alleviating suffering and restoring function. Translational research allows neurosurgeons to offer more personalized treatments based on genetic, molecular, and imaging data. Tailoring therapies to individual patient profiles enhances efficacy and minimizes risks. Furthermore, participation in clinical trials enables neurosurgeons to offer cutting-edge treatments to patients who may have exhausted conventional options.

Challenges and Opportunities

While the integration of translational medicine into neurosurgery holds immense promise, it also presents challenges. Research endeavors require substantial resources, collaboration across disciplines, and rigorous ethical considerations. Moreover, translating laboratory findings into clinical applications demands patience and persistence.

Conclusion

The modern neurosurgeon’s role in translational medicine is multifaceted and indispensable. By embracing research, continuous learning, and patient-centered care, neurosurgeons not only advance the field but also improve the lives of their patients. As technology and knowledge continue to evolve, the collaboration between basic scientists and clinicians will be pivotal in shaping the future of neurosurgery, ultimately leading to better outcomes and enhanced quality of life for patients worldwide.

Editorial: Craniosynostosis

Leopoldo Mandic Furtado — 2024-09-01

In this edition of Archives of Pediatric Neurosurgery, which is entirely dedicated to craniosynostosis, we are pleased to present the foremost evidence on the foundations, concepts, diagnosis, and treatment modalities of this condition, based on well-designed studies in this field.

Craniosynostosis is a rare congenital condition caused by the premature fusion of one or more skull sutures, leading to deformities in head shape, intracranial hypertension, and potentially negative impacts on neuropsychological development. Medical students, neurosurgery residents, young neurosurgeons, and even more experienced neurosurgeons should be aware of the various aspects of this condition to enhance early detection, improve treatment recommendations, and ultimately, the patient's quality of life.

Currently, advancements in fields such as genetics, neurosciences, and surgical techniques have resulted in reduced blood loss, shorter hospital stays, and improved cosmetic and neuropsychological outcomes. Therefore, we aim to disseminate valuable information on the state-of-the-art research in craniosynostosis to stimulate the development and consolidation of evidence based approaches and guide pediatric neurosurgeons in their decision-making.