Myelomeningocele: Long-Term Neurosurgical Management.

Open spina bifida, also known as myelomeningocele (MMC), is the most challenging and severe birth defect of the central nervous system compatible with life and it is due to a failure in the dorsal fusion of the nascent neural tube during embryonic development. MMC is often accompanied by a constellation of collateral conditions, including hydrocephalus, Arnold - Chiari II malformation, brainstem disfunction, hydrosyringomyelia, tethered cord syndrome and scoliosis. Beyond early surgical repair of the dorsal defect, MMC requires lifelong cares. Several additional surgical procedures are generally necessary to improve the long-term outcomes of patients affected by MMC and multidisciplinary evaluations are crucial for early identification and management of the various medical condition that can accompany this pathology. In this chapter, the most common pathological entities associated with MMC are illustrated, focusing on clinical manifestations, treatment strategies and follow up recommendations.

Intracranial complications of sinogenic and otogenic infections in children: an ESPN survey on their occurrence in the pre-COVID and post-COVID era.

BACKGROUND: COVID-19 pandemic is thought to have changed the epidemiology of some pediatric neurosurgical disease: among them are the intracranial complications of sinusitis and otitis (ICSO). According to some studies on a limited number of cases, both streptococci-related sinusitis and ICSO would have increased immediately after the pandemic, although the reason is not clear yet (seasonal changes versus pandemic-related effects). The goal of the present survey of the European Society for Pediatric Neurosurgery (ESPN) was to collect a large number of cases from different European countries encompassing the pre-COVID (2017-2019), COVID (2020-2021), and post-COVID period (2022-June 2023) looking for possible epidemiological and/or clinical changes. MATERIAL AND METHODS: An English language questionnaire was sent to ESPN members about year of the event, patient's age and gender, presence of immune-deficit or other favoring risk factors, COVID infection, signs and symptoms at onset, site of primary infection, type of intracranial complication, identified germ, type and number of surgical operations, type and duration of medical treatment, clinical and radiological outcome, duration of the follow-up. RESULTS: Two hundred fifty-four cases were collected by 30 centers coming from 14 different European countries. There was a statistically significant difference between the post-COVID period (129 children, 86 cases/year, 50.7% of the whole series) and the COVID (40 children, 20 cases/year, 15.7%) or the pre-COVID period (85 children, 28.3 cases/year, 33.5%). Other significant differences concerned the presence of predisposing factors/concurrent diseases (higher in the pre-COVID period) and previous COVID infection (higher in the post-COVID period). No relevant differences occurred as far as demographic, microbiological, clinical, radiological, outcome, morbidity, and mortality data were concerned. Paranasal sinuses and middle ear/mastoid were the most involved primary site of infection (71% and 27%, respectively), while extradural or subdural empyema and brain abscess were the most common ICSO (73% and 17%, respectively). Surgery was required in 95% of cases (neurosurgical and ENT procedure in 71% and 62% of cases, respectively) while antibiotics in 99% of cases. After a 12.4-month follow-up, a full clinical and radiological recovery was obtained in 85% and 84% of cases, respectively. The mortality rate was 2.7%. CONCLUSIONS: These results suggest that the occurrence of ICSO was significantly increased after the pandemic. Such an increase seems to be related to the indirect effects of the pandemic (e.g., immunity debt) rather than to a direct effect of COVID infection or to seasonal fluctuations. ICSO remain challenging diseases but the pandemic did not affect the management strategies nor their prognosis. The epidemiological change of sinusitis/otitis and ICSO should alert about the appropriate follow-up of children with sinusitis/otitis.

Consensus statement from the international consensus meeting on post-traumatic cranioplasty.

BACKGROUND: Due to the lack of high-quality evidence which has hindered the development of evidence-based guidelines, there is a need to provide general guidance on cranioplasty (CP) following traumatic brain injury (TBI), as well as identify areas of ongoing uncertainty via a consensus-based approach. METHODS: The international consensus meeting on post-traumatic CP was held during the International Conference on Recent Advances in Neurotraumatology (ICRAN), in Naples, Italy, in June 2018. This meeting was endorsed by the Neurotrauma Committee of the World Federation of Neurosurgical Societies (WFNS), the NIHR Global Health Research Group on Neurotrauma, and several other neurotrauma organizations. Discussions and voting were organized around 5 pre-specified themes: (1) indications and technique, (2) materials, (3) timing, (4) hydrocephalus, and (5) paediatric CP. RESULTS: The participants discussed published evidence on each topic and proposed consensus statements, which were subject to ratification using anonymous real-time voting. Statements required an agreement threshold of more than 70% for inclusion in the final recommendations. CONCLUSIONS: This document is the first set of practical consensus-based clinical recommendations on post-traumatic CP, focusing on timing, materials, complications, and surgical procedures. Future research directions are also presented.

Spinal epidural abscess in COVID-19 patients.

OBJECTIVE: To report the peculiarity of spinal epidural abscess in COVID-19 patients, as we have observed an unusually high number of these patients following the outbreak of SARS-Corona Virus-2. METHODS: We reviewed the clinical documentation of six consecutive COVID-19 patients with primary spinal epidural abscess that we surgically managed over a 2-month period. These cases were analyzed for what concerns both the viral infection and the spinal abscess. RESULTS: The abscesses were primary in all cases indicating that no evident infective source was found. A primary abscess represents the rarest form of spinal epidural abscess, which is usually secondary to invasive procedures or spread from adjacent infective sites, such as spondylodiscitis, generally occurring in patients with diabetes, obesity, cancer, or other chronic diseases. In all cases, there was mild lymphopenia but the spinal abscess occurred regardless of the severity of the viral disease, immunologic state, or presence of bacteremia. Obesity was the only risk factor and was reported in two patients. All patients but one were hypertensive. The preferred localizations were cervical and thoracic, whereas classic abscess generally occur at the lumbar level. No patient had a history of pyogenic infection, even though previous asymptomatic bacterial contaminations were reported in three cases. CONCLUSION: We wonder about the concentration of this uncommon disease in such a short period. To our knowledge, cases of spinal epidural abscess in COVID-19 patients have not been reported to date. We hypothesize that, in our patients, the spinal infection could have depended on the coexistence of an initially asymptomatic bacterial contamination. The well-known COVID-19-related endotheliitis might have created the conditions for retrograde bacterial invasion to the correspondent spinal epidural space. Furthermore, spinal epidural abscess carries a significantly high morbidity and mortality. It is difficult to diagnose, especially in compromised COVID-19 patients but should be kept in mind as early diagnosis and treatment are crucial.

The management of emergency spinal surgery during the COVID-19 pandemic in Italy.

AIMS: The current pandemic caused by COVID-19 is the biggest challenge for national health systems for a century. While most medical resources are allocated to treat COVID-19 patients, several non-COVID-19 medical emergencies still need to be treated, including vertebral fractures and spinal cord compression. The aim of this paper is to report the early experience and an organizational protocol for emergency spinal surgery currently being used in a large metropolitan area by an integrated team of orthopaedic surgeons and neurosurgeons. METHODS: An organizational model is presented based on case centralization in hub hospitals and early management of surgical cases to reduce hospital stay. Data from all the patients admitted for emergency spinal surgery from the beginning of the outbreak were prospectively collected and compared to data from patients admitted for the same reason in the same time span in the previous year, and treated by the same integrated team. RESULTS: A total of 19 patients (11 males and eight females, with a mean age of 49.9 years (14 to 83)) were admitted either for vertebral fracture or spinal cord compression in a 19-day period, compared to the ten admitted in the previous year. No COVID-19 patients were treated. The mean time between admission and surgery was 1.7 days, significantly lower than 6.8 days the previous year (p < 0.001). CONCLUSION: The structural organization and the management protocol we describe allowed us to reduce the time to surgery and ultimately hospital stay, thereby maximizing the already stretched medical resources available. We hope that our early experience can be of value to the medical communities that will soon be in the same emergency situation. Cite this article: Bone Joint J 2020;102-B(6):671-676.

A rare case of cervical junction ligamentous cyst.

Ligamentous cyst is a cystic formation arising from degeneration of ligamentous structures all around the spinal cord. They can cause spinal cord compression, like synovial cyst. Unlike synovial cyst, there is no spinal instability in pathogenesis of ligamentous cyst. Differential diagnosis through pre-operative MRI is difficult and intraoperative findings plus histopathology are crucial to achieve a diagnosis. In this case report, we deal with a rare case of cervical junction ligamentous cyst. A 59-year-old Caucasian female was admitted in our ward with left-sided hemiparesis, cervical pain, and upper limb diffused paresthesias, due to an oval-shaped formation into ALL, of 13 mm in maximum diameter, with peripheral contrast enhancement. The patient underwent, under general anesthesia, a surgery through a posterolateral suboccipital approach which aimed to decompress the spinal cord and to drain the cyst with total removal of the compression by emptying the cyst and fulfilling it with muscle graft and glue. No posterior fixation was needed. After the surgery, symptoms improved and a post-operative MRI scan demonstrated the good result of the surgery.

Acute traumatic fractures to the craniovertebral junction: preliminary experience with the "MILD" score scale.

PURPOSE: Traumatic fractures to the craniovertebral junction (CVJ) are rare events requiring complex clinical management. Several classification systems are currently in use; however, recent improvements of junctional knowledge has focused attention on the role of ligaments and membranes in vertebral biomechanical stability. The aim of this study was to present our preliminary experience with the "MILD" score scale, which should allow fast and effective classification of all CVJ traumatic fractures based on vertebral instability in the acute setting. METHODS: A prospective study was conducted on 38 consecutive patients with 43 traumatic junctional fractures identified by computed tomography (CT) scan in the acute trauma phase. The MILD scale was applied to all fractures, and a score was obtained for each patient. All cases underwent magnetic resonance imaging (MRI) to assess the anatomical integrity of ligaments and membranes. RESULTS: Twenty-seven patients (71 %) were classified as MILD type 1 (0-1 points), showed a negative MRI, and healed with conservative treatment. Eight patients (21 %) were classified as MILD type 2 (2 points) and showed modest indirect signs of ligamentous injuries. Four of these patients healed with conservative treatment, while three patients underwent surgery due to wide bone fracture fragment displacement. Three patients (8 %) were classified as MILD type 3 (3 points), all of whom showed extensive ligamentous damage and underwent surgery. CONCLUSIONS: The close association between the MILD scale and spinal instability is promising, although further studies are warranted in order to confirm our preliminary data.

Surgery for intracerebral hemorrhage.

The surgical treatment of intracerebral hemorrhage (ICH) is one of the most controversial areas of neurosurgery. Randomized trials are inconclusive due to the small number of patients enrolled or because performed in pre CT era. We analyze the 232 patients admitted to the Niguarda Hospital of Milano, Italy, for ICH during the period January 2001-December 2002. Sixty patients were operated: 29 had secondary haemorrhage, and 30 a primary one. The strategy followed in these patients is presented. The series suggests that indication to surgery is a complex process including several factors that have to be all considered in each patient.

Surgical treatment of intracranial dural arteriovenous fistulae: role of venous drainage.

OBJECTIVE: This report focuses on the surgical management of aggressive intracranial dural arteriovenous fistulae (d-AVFs), which are defined as fistulae with arterialized leptomeningeal veins (red veins). Particular attention is paid to the accurate identification of the venous drainage pattern and to the choice of the proper treatment strategy. METHODS: Thirty-four consecutive patients with aggressive intracranial d-AVFs were treated between 1994 and 1998. Angiographic studies allowed the identification of two main types of aggressive lesions, i.e., d-AVFs with sinus drainage and reflow into leptomeningeal veins (12 patients), which we designated sinus fistulae, and d-AVFs drained exclusively by leptomeningeal veins without sinus interposition (22 patients), which we designated nonsinus fistulae. All patients underwent surgical treatment, which consisted of resection of the fistulous sinus tract in 12 cases of sinus fistulae and interruption of the draining veins at their dural origin in 22 cases of nonsinus fistulae. Surgical preparation via multistage transarterial embolization was required in all 12 cases of sinus fistulae and in 4 of 22 cases of nonsinus fistulae. RESULTS: The mortality rate was 0%, and there were no instances of lasting morbidity. Radioanatomic cures were achieved in all cases. There was no case of venous hypertension or venous infarction after resection of the affected sinus or interruption of the draining veins. No arteriovenous shunts recurred during the follow-up period. CONCLUSION: Careful preoperative identification of the venous drainage pattern seems critical for planning of the correct surgical strategy to treat aggressive intracranial d-AVFs. If the fistula exhibits sinus drainage with reflow into leptomeningeal veins, surgical excision of the fistulous sinus segment represents a safe and definitive treatment option. In these cases, the affected sinus may be safely removed, provided that preoperative angiograms demonstrate participation of the sinus in drainage of the lesion, indicating that the sinus is nonfunctional. Conversely, if the fistula exhibits pure leptomeningeal drainage, the sinus does not participate in drainage of the lesion and cannot be excised. In these cases, the best treatment involves interruption of the draining veins at the point at which they exit the dural wall of the sinus. This simple easy treatment has been proven to be safe and highly effective in permanently eliminating arteriovenous shunts.

Surgical treatment of poor-grade aneurysm patients.

Patients who are severely struck by subarachnoid hemorrhage (the so called poor-grades) have been usually considered almost hopeless. Conversely, it is now becoming apparent that a significant number of poor grades could be perhaps salvageable during the first hours after the hemorrhage. In this paper we are reporting the results of an aggressive management protocol including immediate intensive care management and early surgery. Early surgery was offered to all patients without vital brain destructions on CT-scan, with treatable intracranial hypertension and stabilization of vital parameters. By this attitude, among 32 initial unselected consecutive poor grades, we could manage by early surgery 15 patients (47%), obtaining 11 favourable outcomes (35%). These encouraging results in the treatment of patients otherwise destined to ominous consequences are now to be compared with the presently available less invasive endovascular techniques.

Transbasal approaches to aneurysms of the vertebro-basilar junction.

The most difficult aneurysms to be surgically treated are those of the vertebro-basilar junction area. This is due to their deep location and the proximity of brain stem and cranial nerves. Recently, new transbasal surgical approaches have been developed in order to realize a shorter and more direct access. Clival lesions, such as neoplasms, angiomas, and aneurysms, can now be safely faced through these routes. In this paper, we report our recent experience in transbasal approaches for the management of six consecutive patients, with aneurysms in this area. In four of these patients, the initial treatment consisted of an attempt at endovascular aneurysm obliteration using the Guglielmi Detachable Coil system, whereas, in the other two patients (basing on age, aneurysm size and neurological conditions), surgery was considered as the treatment of choice. Unfortunately, the endovascular treatment failed in all cases, and all patients had to be eventually operated on. In all cases, surgical clipping was performed through the combination of a transmastoid (asterional) approach with the suboccipital lateral approach. By this route, in all cases, parent arteries proved well delineated, the aneurysms could always be correctly clipped. Good long-term results were achieved in all cases but one. When surgery is to be performed, the importance of a thorough wide exposure of the whole vertebro-basilar junction area, as well as the importance of having multiple accesses to the lesion, cannot be overemphasized.

Multidisciplinary (surgical and endovascular) approach to intracranial aneurysms.

In spite of the availability of the new endovascular technique (GDC) to manage cerebral aneurysms, to date, the crucial question "which is the proper treatment in a given patient?" still remains unsettled. In order to check whether an answer is possible, we retrospectively reviewed a personal series of 192 consecutive patients with cerebral aneurysms (1993-1995). We found 164 patients who had been considered eligible for active aneurysm treatment. Treatment modality has been chosen case by case on the basis of patient conditions, and aneurysm size and location. Four groups of patients were identified: Group 1: 104 patients (63.4%) with subarachnoid hemorrhage (SAH) in whom the treatment of choice was surgery; Group 2: 27 SAH patients (16.4%) in whom the first choice was GDC; Group 3: 7 SAH patients (4.2%) who died before the scheduled treatment; Group 4: 26 patients (15%) with not ruptured aneurysm who had either surgery or GDC. Based on the results of this series (improvement of the overall results through the multidisciplinary approach), we have developed the guidelines to prospectively manage future cases of cerebral aneurysms with the purpose to rationalize the management, thus further improving the overall results.

Aneurysms of the vertebro-basilar junction area: preliminary experience in endovascular and surgical management.

Aneurysms of the vertebro-basilar junction area have been considered the most difficult to be surgically treated because of their deep location, the proximity of the brain stem and the cranial nerves. However, at present, new endovascular techniques and new transbasal surgical approaches offer valuable management strategies. This paper concerns six consecutive patients whom we managed either endovascularly or surgically during a period of eighteen months. Direct surgical treatment was offered to two young patients with relatively small aneurysms in good neurological condition, whereas the other four patients initially underwent an endovascular attempt at aneurysm obliteration using the Guglielmi detachable coil system. Unfortunately, interventional neuroradiology failed in three cases, and surgery had to be re-considered. Accordingly, a total of five patients underwent surgical clipping through the combination of a transmastoid retrolabyrinthine approach with the suboccipital lateral approach. This combination of approaches provided a good control of both vertebral arteries and basilar artery, and allowed the aneurysm to be correctly clipped in all cases. Good long-term results were achieved in all cases but one. Based on this preliminary experience, we would stress the importance of multidisciplinary approach with a treatment calibrated for each single case. Furthermore, if surgery is to be performed, the combination of transmastoid-retrolabyrinthine and suboccipital lateral approaches provides a wide exposure of the whole vertebro-basilar junction area and allows good access to the lesion.

Comparison of pediatric and adult cerebral arteriovenous malformations.

The authors report 19 consecutive children with cerebral arteriovenous malformations over the period 1978-1992. These patients are compared with a series of 120 consecutive adult patients with the same pathology, managed during the same period. The main clinical and angiographic features, as well as the treatment modalities and outcome are reviewed and compared. Children seem to harbour smaller and simpler lesions than adults. Furthermore, despite a more severe clinical presentation, children appears to fare better than adults. The possibility of evolution of brain arteriovenous malformations is discussed.

Complex arteriovenous fistula of the brain in an infant. Case report.

A rare case of complex cerebral arteriovenous fistula in an infant is reported. An 8-month-old boy first presented with a syndrome of increased intracranial pressure. Neuroradiological assessment showed a direct intracerebral arteriovenous shunt with marked venous engorgement. No hemorrhage was evident. Direct surgical treatment was decided. Clinical and radioanatomical cure was achieved. The main angiographic and pathophysiologic features of this unusual entity, as well as the available therapeutic options, are reviewed and discussed.

Surgical interruption of leptomeningeal drainage as treatment for intracranial dural arteriovenous fistulas without dural sinus drainage.

Intracranial dural arteriovenous fistulas (AVFs) have been recognized as acquired lesions that can behave aggressively depending on the pattern of venous drainage. Based on the type of venous drainage, they can be classified as fistulas drained only by venous sinuses, those drained by venous sinuses with retrograde flow in arterialized leptomeningeal veins, and fistulas drained solely by arterialized leptomeningeal veins. Serious symptoms, including hemorrhage and focal deficit, are related to the presence of arterialized leptomeningeal veins. In this paper, the authors report a consecutive series treated between 1988 and 1993 of 20 cases of intracranial dural AVFs with "pure leptomeningeal drainage." All patients underwent surgical interruption of the leptomeningeal draining veins. Based on the arterial supply, nine patients were managed by direct surgery, whereas 11 patients were prepared for surgery by means of preoperative arterial embolization. Radioanatomical cure of the fistula and good neurological recovery were achieved in 18 cases. Complete obliteration of the fistula was documented angiographically in two cases, but fatal hemorrhage occurred, probably due to partial thrombosis of the venous drainage. Based on this experience, the authors believe that surgical interruption of the draining veins is the best treatment option for intracranial dural AVFs. However, surgical results may be affected by the extension of postoperative thrombosis, which in turn may be related to the degree of preoperative venous engorgement.

"High risk" anterior basal skull fractures. Surgical treatment of 64 consecutive cases.

Anterior Basal Skull Fractures (ABSFs) may be complicated by Cerebrospinal Fluid (CSF) fistulae and intracranial infections. An initially non-operative management is usually suggested since most fistulae spontaneously stop within a few days thus requiring no surgical repair. However, if the fistula fails to stop or recurs, surgical treatment is to be considered. Furthermore, if the fracture is complicated by meningitis, there is a relative risk of recurring infections and surgical repair may be also considered. Finally, surgical repair may be suggested in cases of compound, comminuted, depressed, largely extended cranio-facial fractures (the so-called "fracas craniofaciaux") where spontaneous healing is considered unlikely and risk of infection is high. Accordingly we termed "high risk" fractures those associated with active (persistent or recurring) cerebrospinal fluid fistula, those with meningitis and the so-called "fracas craniofaciaux". In this paper, we report our personal experience in surgical treatment of 64 consecutive "high risk" anterior basal skull fractures. Thirty-seven patients had persistent or recurring fistulae, ten had intracranial infections and seventeen had severe bone derangement of the anterior skull base. The osteodural repairs were performed through bilateral or unilateral subfrontal approach. In 59 cases the initial procedure was successful whereas 4 patient needed additional surgery but were ultimately successfully treated. One patient died. No major permanent neurologic or neuropsychologic impairments were reported. On the basis of our experience, we think that intracranial repair is a very suitable treatment modality in facing "high risk" anterior basal skull fractures.

Delayed complications of ethmoid fractures: a "growing fracture" phenomenon.

Delayed complications of ethmoid fractures are considered relatively rare. However, meningitis, recurrence of previously ceased cerebrospinal fluid rhinorrhea and delayed onset of cerebrospinal fluid rhinorrhea are possible even years after trauma. We report 10 consecutive patients with delayed complications of ethmoid fractures, whom we treated over the past 11 years. All patients had previously sustained a closed head injury and had remained anosmic. Variously after trauma (ranging from 2 months to 31 years), these patients were re-admitted because of meningitis (6 cases), recurrence of previously ceased cerebrospinal fluid rhinorrhea (3 cases), and delayed onset of cerebrospinal fluid rhinorrhea (1 case). In all cases the delayed complications were associated with relatively large defects of the ethmoid bone. These bone lesions were now evident even in those patients whose radiological assessments had been normal after trauma. All patients underwent a successful surgical repair and remained well during the follow-up. We discuss the possibility that delayed complications of ethmoid fractures are due to a mechanism like that of "growing fractures" in children.

Leptomeningeal dissemination of juvenile pilocytic astrocytoma: case report.

The authors report a case of leptomeningeal spinal metastasis of chiasmatic juvenile pilocytic astrocytoma. A 7-year-old boy underwent surgery and radiation therapy for a chiasmatic pilocytic astrocytoma. Three years later he experienced a syndrome of medullary compression. A lumbosacral intradural extra-axial tumor was discovered and subtotally excised. Pathologic examination revealed a metastasis of the chiasmatic tumor without any evidence of malignant transformation. Only five cases of leptomeningeal spinal dissemination of pilocytic astrocytoma have been previously reported. The authors review and discuss the clinical and pathologic features of these cases.