Surgical pathway proposal for severe paralytic scoliosis in adolescents with myelomeningocele.

PURPOSE: The increased life expectancy of patients with myelomeningocele led to the necessity of new management for addressing complex spine deformities with severe pelvic obliquity. Tethered cord release (TCR) and spinal fusion were described as treatment options. However, nowadays, the surgical strategy is not well defined and high rates of mechanical failures and complications are reported. Our aim was to propose a new two-stage surgical pathway to treat a selected group of severe myelomeningocele scoliosis. METHODS: This is a retrospective series of myelomeningocele scoliosis in paralytic adolescent patients treated with concurrent TCR and posterior spinal fusion (PSF), followed by delayed anterior fusion (ASF) through minimally invasive lateral approach. Inclusion criteria were as follows: evolutive scoliosis in tethered cord syndrome and paraplegia, main curve >70°, pelvic obliquity >20°, and age between 10 and 18 years. Clinical, surgical, and radiographic parameters were evaluated preoperatively and at a mean follow-up (FU) of 2.8 years. RESULTS: Six patients out of 58 met the inclusion criteria and were included in the study (mean age 12.3 years old). The mean curve and pelvic obliquity correction were 68.8% and 82%, respectively. No loss of correction was recorded at FU. One case of cerebrospinal fluid leakage requiring revision surgery was recorded. CONCLUSION: To the best of our knowledge, this is the first study proposing a two-stage surgical pathway for severe MMC spinopelvic deformity treatment. Concurrent TCR and PSF with delayed minimally invasive ASF permitted to minimize complications providing good fusion rates without loss of correction and implant failure.

Complications in AVM Surgery.

In AVM surgery perioperative complications can arise and can have serious perioperative consequences. Surgically related complications in AVM treatment, in many cases, can be avoided by paying attention to details:1. Careful selection of the patient: - addressing a patient with eloquent AVM to Gamma Knife treatment - preoperative treatment with selective embolization of the accessible deep feeders - preoperative gamma knife or embolize those patient with an over-expressed venous pattern2. Meticulous coagulation of deep medullary feeders: - Using dirty coagulation - Using dry non-stick coagulation - Using micro clips - Using laser - Reaching the choroidal vessel in the ventricle when possible - Avoiding occlusive coagulation with hemostatic agents3. Check and avoiding any residual of the AVM4. Keep the patient under pressure control during postoperative periodFulfilling these steps contributes to reduce complications in this difficult surgery, leading to a safer treatment that compares favorably with natural history of brain arteriovenous malformations.

Intracranial Dural Arteriovenous Fistulas: The Sinus and Non-Sinus Concept.

INTRODUCTION: Dural arteriovenous fistulas (dAVFs) account for 10-15% of all intracranial arteriovenous lesions. Different classification strategies have been proposed in the course of the years. None of them seems to guide the treatment strategy. OBJECTIVE: We expose the experience of the vascular group at Niguarda Hospital and we propose a very practical classification method based on the location of the shunt. We divide dAVF in sinus and non-sinus in order to simplify our daily practice, as this classification method is simply based on the involvement of the sinuses. MATERIAL AND METHODS: 477 intracranial dural arteriovenous fistulas have been treated. 376 underwent endovascular treatment and 101 underwent surgical treatment. Cavernous sinus DAVFs and Galen ampulla malformations have been excluded from this series as they represent a different pathology per se. 376 dAVFs treated by endovascular approach: 180 were sinus and 179 were non-sinus. 101 dAVFs treated with surgical approach: 15 were sinus and 86 were non-sinus. DISCUSSION: Of the 477 intracranial dAVF the recorded mortality and severe disability was 3% and morbidity less than 4%. All patients underwent a postoperative DSA with nearly 100% of complete occlusion of the fistula. At a mean follow-up of 5 years in one case there was a non-sinus fistula recurrence, due to the presence of a partial clipping of "piè" of the vein. CONCLUSIONS: The sinus and non-sinus concept has guided our institution for years and has led to good clinical results. This paper intends to share this practical classification with the neurosurgical community.

Clinical considerations on a right operculo-insular cavernous angioma: an illustrative case.

The insular cortex is considered one of the most complex regions of the brain, defined as the "hub" of somatosensory areas. Here, we examine the case of a surgically treated haemorrhagic cavernoma involving the middle and posterior insular cortex, presenting both sensory, gustative and speech symptoms. By reviewing the recent findings in humans' and primates' basic research, we illustrated clinical and radiological correlations of the reported case, confirming insular role in sensitive and gustatory functions.

Acute presentation of Chiari 1 malformation in children.

PURPOSE: To review the literature about the acute presentation of Chiari 1 malformation in children, with a focus on acute cervical cord syndromes with impairment of the cortico-spinal tract. To analyze the possible precipitating factors and the pathophysiology of the acute onset. METHOD: Illustrative case presentation with literature review. RESULTS: The literature includes just a few dozens of children with acute presentation of Chiari 1 malformation. The more frequent presentations consist of oropharyngeal dysfunction, cranial nerve impairment, high intracranial pressure, peripheral motor deficit. Acute impairment of cervical long tracts is very rare and we could find only 16 previously reported cases of cervical cord impairment with quadriparesis or hemiparesis. CONCLUSIONS: Nowadays, a lot of asymptomatic Chiari 1 malformations are frequently observed owing to the wide diffusion of magnetic resonance imaging. This raised the question about the management of these patients. Despite severe and even lethal manifestations being reported in previously asymptomatic patients, the absolute rarity of acute deterioration does not justify prophylactic surgery. The diagnosis of Chiari malformation may be initially difficult in patients with isolated, acute, and unusual presentations but physicians should bear in mind its possibility, because prompt cranio-cervical decompression may be decisive.

Surgical management of Chiari malformation type II.

BACKGROUND: Chiari malformation type II is present in almost all patients with myelomeningocele but usually remains asymptomatic. Symptoms are generally more severe in neonates, who have the worst prognosis. The association symptoms/hydrocephalus is well known, and first treatment usually consists of ensuring adequate ventricular drainage. Craniovertebral decompression may be required in patients who do not improve after drainage. However, mechanisms of symptom development are not yet completely understood, timing and techniques of surgery are not codified, long-term evolution is poorly reported, and there are few paper reporting clinical onset and treatment in older patients. METHODS: We reviewed our personal series of 42 consecutive symptomatic patients that required surgical treatment. Age at surgery ranged from 1 week to 44 years (mean 6.6 years). Surgical timing strictly depended on clinical conditions: urgent management in the more compromised patients (usually infants) and elective treatment before severe deterioration in patients with less severe conditions. All patients first underwent external ventricular drainage, which resolved the symptomatology in 17 cases (40%). Craniocervical decompression was required by 25 patients (60%) who received no benefit from the ventricular drainage. RESULTS: Early mortality (2 cases = 4.7%) occurred only in neonates. Clinical improvement was achieved in 37 of 40 survivors (92%). During a follow-up ranging from 2 to 20 years (mean 10.3 years), late mortality consisted of 4 cases (10%), mainly due to cardio-respiratory arrest. Twenty-two patients (55%) required surgery for shunt malfunction and 4 for cord detethering. Six patients (15%) required reoperation owing to symptom recurrence. CONCLUSION: Early treatment of symptomatic Chiari II malformations may warrant satisfactory results in a significant number of patients, even in neonates. Nevertheless, overall mortality remains relatively high, throughout the patient life. Formal transition programs and adult spina bifida care processes have become crucial.

Complications of Halo Placement.

BACKGROUND: The halo vest is widely used throughout the world to manage craniovertebral and cervical instabilities. It can be used for postoperative immobilization or as an alternative to surgical fixation. METHOD: In this paper we present some cases of severe complications from our own practice and review the literature on halo complications. RESULTS: Like any therapeutic manoeuvre, halo placement may be followed by various complications. In the meantime, modern techniques of fixation offer safe and immediate stabilization. CONCLUSION: The halo vest remains a formidable method for cervical immobilization. However, it should not be used a priori instead of surgery.

Craniovertebral fusion in an infant using struts of banked adult bone.

PURPOSE: The aim of this paper is to discuss the problems of craniocervical instability and craniocervical fusion in infancy. Despite the relative frequency of carniovertebral joint malformations, actual instability is quite rare in infancy. METHODS: An infant 8 months of age presented with tetraparesis and sleep apnea due to a complex malformation of her craniovertebral joint. An initial attempt at conservative treatment using a rigid neck collar failed, so the patient was surgically managed by the onlay placement of two autologous rib grafts. The rigid collar was maintained for 5 months. RESULTS: Both rib grafts progressively reabsorbed within a few months, while the clinical deficits recurred. Reoperation consisted of occipitocervical interposition of two robust struts of banked cadaveric adult fibula. This time, the skull appeared mature enough to allow immobilization by the halo system. Adequate occipitocervical fusion was eventually achieved, and the patient fully recovered. CONCLUSIONS: To the best of our knowledge, there is no other reported case of an infant undergoing craniovertebral fusion using cadaveric adult bone. When screw placement is not considered advisable to manage small infants, appropriate stability may be obtained using struts of robust cadaveric bone. A meticulous carpentry technique with graft interposition under compression and adequate postoperative immobilization remains mandatory.

Traumatic injuries to the craniovertebral junction: a review of rare events.

The craniovertebral junction is a specific region of the spine with unique anatomical and biomechanical properties that yields a wide variety of injury patterns. Junctional traumatic fractures and/or dislocations are widely reported in clinical practice, but we could identify only a subgroup of upper cervical spine traumatic injuries with very few cases reported in the literature, and for this reason may be considered rare. In some of these cases, the absence of spinal biomechanical instability, in association with moderate clinical symptoms (neck stiffness and pain) and the difficulty in fracture identification through standard cervical radiographs, leads to a high percentage of missed injuries. In other cases, traumatic events have been commonly described only in autopsy series due to the high degree of spinal biomechanical instability. Herein, we have summarized all the relevant literature concerning this issue and also included our cases, with the aim of emphasizing prompt diagnosis and correct management. We provide a guide for correctly identifying "rare" craniovertebral junction traumatic injuries.

Anterior spinal pseudomeningocele after C0-C2 traumatic injuries: role of the "dural transitional zone" in the etiopathogenesis.

PURPOSE: Spinal pseudomeningoceles (SPM) are extradural collections of CSF (cerebrospinal fluid); a frequent association with upper cervical injuries (UCI) has been observed. We propose a possible etiopathogenetic mechanism supporting the formation of cervical SPM based on some considerations. METHODS: We present four cases of SPM. All patients sustained a severe UCI. Three patients were symptomatic with delayed and progressive clinical signs. RESULTS: One patient was misinterpreted as epidural hematoma and operated on due to progressive signs with postoperative clinical improvement. The rest of patients were treated conservatively; spontaneous reduction of CSF collection occurred. From a radiological standpoint: (1) a line of demarcation separated the intradural cervical compartment from the anterior epidural space, (2) CSF epidural collection was never evident at C0-C2 level and extended from C2 downwards, and (3) shape of collection was similar to epidural hematomas suggesting a ball-valve mechanism. CONCLUSIONS: The dural layer at C0-C2 level is adherent to the thick ligamentous apparatus, as opposed to the segments below where it is solely covered by the posterior longitudinal ligament. A "transitional zone" of dura exists between the C0-C2 region and subaxial segment of the cervical spine. This watershed area constitutes a point of minor resistance. Lacerations of the meningeal layers, caused by severe UCI at the "transitional zone", drain CSF into the anterior epidural space and form SPM.

Single fracture in the atlas vertebral arch: analysis of an unusual event.

STUDY DESIGN: Retrospective study. OBJECTIVE: The aim of this study is to show that a single traumatic fracture of the atlas is unusual but not rare and requires specific management strategies, which can be highlighted by the study of the junctional ligaments and membranes. SUMMARY OF BACKGROUND DATA: A single traumatic fracture of the atlas arch is considered a rare event and has been analyzed in few case reports. Ligaments and membranes play a primary role in providing stability to the craniovertebral junction area. METHODS: Here, we report 10 cases of a single traumatic fracture of the atlas arch: 4 cases were part of our series of spine injuries and were studied by magnetic resonance imaging during the acute phase to assess the junctional ligaments. The remaining 6 cases were obtained from the Literature. RESULTS: Indirect signs of a traumatic ligamentous injury were found only in 1 patient. However, in all cases, these fractures were considered biomechanically stable and all patients were discharged with a rigid collar. During the follow-up, all patients reported an improvement in their neck pain and tenderness, with radiologic signs of bone healing. CONCLUSIONS: A single fracture of the atlas arch is an unusual traumatic event that results from slow-force impact. Medical experience in treating this specific fracture subtype is nowadays poor because of the paucity of the literature; thus, this unusual condition may either be underestimated or even overstudied and overtreated. Nowadays, it seems reasonable to assume that the management should be conservative in all cases and that magnetic resonance imaging often plays only a marginal role.

Integrated spine trauma team protocol: Combined neurosurgical and orthopedic experience for the management of traumatic spinal injuries.

Objectives: During the last decades, spine surgery has grown exponentially. In spite of that, it remains a surgical specialty without a well-defined own certification. It is usually carried out, separately, by neurosurgeons and orthopedic surgeons, even if there is an overlapping of competence and skills. Materials and Methods: In our hospital, from January 2019, a systematic protocol called integrated spine trauma team protocol (ISTTP) was implemented to improve the management of traumatic spinal injuries in a multidisciplinary way. It is characterized by a specific algorithm from diagnosis to postoperative care. According to the new protocol, orthopedic spinal surgeons and neurosurgeons work together as an integrated spine trauma team. The authors analyzed, retrospectively, the results obtained by comparing patients treated before and after the application of the ISTTP. Results: The new protocol allowed a statistically significant reduction in waiting time before surgery and complication rate. Moreover, early discharge of patients was recorded. To the best of our knowledge, this is the first study that described a specific algorithm for a standardized multidisciplinary management of the spinal trauma with combined orthopedic and neurosurgeon expertise. Conclusion: Our preliminary results suggest that the application of our ISTTP leads to better results for treating traumatic spinal injury (TSI).

The anterior stand-alone approach (ASAA) during the acute phase of spondylodiscitis: results in 40 consecutively treated patients.

PURPOSE: Spondylodiscitis mainly affects the anterior part of the spine. In this paper, we retrospectively analyze our experience with the anterior stand-alone approach (ASAA) in the treatment of spinal infections. METHODS: Forty consecutive patients with severe spondylodiscitis underwent the ASAA during the acute infective phase. Treatment consisted of disease debridement, vertebral body reconstruction using titanium expandable prostheses and anterior fixation. RESULTS: There was neither mortality nor major morbidity. Successful arthrodesis was achieved in 39 out of 40 patients who remained disease free throughout the follow-up period. Six months after treatment, one patient experienced pseudarthrosis and required supplemented posterior spinal fixation for vertebral instability. However, adequate arthrodesis was eventually obtained even in this patient. CONCLUSIONS: ASAA with spine reconstruction using synthetic materials during the acute infection phase was safe and effective. The infections were rapidly defeated, the patients were allowed to stand up early after the procedure and the length of hospital stay was significantly reduced.

Spinal dural arteriovenous fistulas: outcome and prognostic factors.

The aim of this study is to review the clinical outcome of patients treated for spinal dural arteriovenous malformations and investigate the presence of pretreatment indicators of outcome after short- and midterm follow-up. The authors retrospectively reviewed the records of 65 consecutive patients treated either surgically or endovascularly in 3 neurosurgery departments between 1989 and 2009. After treatment, 80% of patients reported improvement of at least 1 symptom. Motor symptoms improved more than sensory disorders, pain, or sphincter impairment. Spinal dural arteriovenous fistulas at the thoracic level, and in particular at the lower level, responded better than those in other spinal areas. Spinal dural arteriovenous fistula is a rare pathology with a malignant course that should be treated aggressively. This study appears to confirm that neurological status before treatment, anatomical location of the fistula, and symptoms at presentation are all factors that can predict outcome. Early diagnosis of spinal dural arteriovenous malformations in the lower thoracic area in patients with an Aminoff scale score < 3 was associated with the most favorable outcome.

Giant occipital meningocele in an 8-year-old child with Dandy-Walker malformation.

INTRODUCTION: The possibility of an association between Dandy-Walker malformation and occipital meningocele is well-known. However, just an overall number of about 40 cases have been previously reported. Giant occipital meningocele has been described only in three newborns. Incidence, pathology, clinical presentation, and proper management of this association are still poorly defined. REPORT OF THE CASE: An 8-year-old boy with Dandy-Walker malformation and giant (25 cm in diameter) occipital meningocele is presented. This boy was born without any apparent occipital mass and harbored no other significant malformations including hydrocephalus. On admission, he was neurologically intact and the giant occipital mass presented partially calcified cyst walls. Treatment consisted of the excision of the occipital malformation, cranioplasty, and cysto-peritoneal shunt. Outcome was excellent. CONCLUSIONS: To the best of our knowledge, among the few reported patients with Dandy-Walker malformation associated to occipital meningocele, this is the oldest one and the one with the largest occipital meningocele; he is unique with calcified walls of the occipital meningocele and the only one who survived the repair of the giant malformation. In Dandy-Walker malformation, occipital meningocele may develop and grow regardless of hydrocephalus. Giant size may be reached and the cyst may become calcified. Surgical repair may warrant favorable outcome.

Chiari malformation type 1: are we doing less with more? Illustrative case.

BACKGROUND: Classic treatment of Chiari malformation type 1 consists of foramen magnum decompression. Selected patients may require occipitocervical fixation, transoral odontoidectomy, tonsillectomy, and so forth. Treatment standardization does not yet exist, and some patients risk being overtreated. OBSERVATIONS: A 20-year-old man with headache and Chiari malformation type 1 underwent extradural bone decompression. One year later, he was managed with the extradural section of his filum terminale. Eighteen months later, the patient underwent monitoring of intracranial pressure, occipitocervical stabilization, transoral odontoidectomy, minimally invasive subpial tonsillectomy, and occipital cranioplasty. His headache never changed, and he progressively developed hemiparesis and swallowing and respiratory disturbances. Two years later, a new magnetic resonance imaging scan showed extended syringomyelia with scarce peritonsillar subarachnoid space. The umpteenth operation consisted of the removal of a constricting epidural scar, arachnoid dissection, total tonsillectomy, creation of a wide subarachnoid space, and dural sac augmentation. The patient's initial postoperative course was smooth, and his headache improved. However, 8 days after surgery, the patient acutely presented with vegetative disturbances and died because of malignant brainstem edema of unknown origin. LESSONS: The story of this patient is not so uncommon. He underwent all the possible surgical treatments rather than a timely adequate osteodural decompression. Probably, he received less with more.

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.

Aulus Cornelius Celsus and the Head Injuries.

Aulus Cornelius Celsus lived in Rome during the reigns of Augustus and Tiberius. He developed the scientific Latin, and his work De Medicina was the first medical textbook to be written in Latin, and subsequently the first medical treatise to be printed. He perhaps was a simple medical writer rather than a real physician, but his contributions to medical knowledge are outstanding. He described the tetrad of inflammation (dolor, rubor, calor, tumor), coined the term cancer, and reported several revolutionary surgical techniques. His eighth book, almost entirely dedicated to dislocations and fractures, provides an extensive description of head injuries such as extradural hematomas, lesions distant from the impact point, and intracranial damage in cases with no overlying fractures. He also provided the first description of brain swelling exceeding the level of the skull, described several surgical indications and craniotomy techniques, recommended treatment for depressed fractures (which had been previously considered untreatable), and detailed the surgical instruments employed. De Medicina was based on the previous Greek medical knowledge, but Celsus also provided original innovations, personal observations, and even comments and criticisms. Despite the fact that he was almost forgotten during the Middle Age, most of his concepts and surgical advices remained in the medical background of the following 2 millennia.

Giant calcified thoracic disk herniations: ossification of PLL or autonomous entity?

BACKGROUND: Giant calcified thoracic disk herniation (GCTD) is an uncommon event, which requires surgical treatment in less than 1% of patients. GCDTs are a specific subgroup of herniated thoracic disks occupying more than 40% of the spinal canal showing calcifications associated with a certain degree of ossification. In this paper, we are reporting our whole experience in the surgical management of GCTDs through anterior approaches. We believe that they present characteristics that associate them to the circumscribed type of Ossified Posterior Longitudinal Ligament (OPLL) with a possible common pathophysiology consisting in the dural violation. METHODS: Twenty-three consecutive patients with GCDTs were managed through anterior approaches during the period 1996-2019 at the Niguarda Hospital - Milan, Italy. Clinical data, radiological features, surgical reports, histological findings, and outcomes were reviewed. RESULTS: There was no mortality, whereas permanent morbidity consisted of 1 cases of worsened paraparesis due to accidental spinal cord contusion. One patient required reoperation to repair a postoperative cerebrospinal fluid (CSF) leakage. All patients underwent postoperative MRI which showed excellent decompression of cord and dural sac in all cases. Histological study of en-bloc removed GCTD showed typical calcification patterns of the PLL. CONCLUSIONS: GCDTs may be assimilated to the so-called "circumscribed type" of OPLL. The GCDTs may show the same radiological CT and MRI pattern of OPLL. The anterior accesses now represent the standard of care for GCTDs. The use of operative microscope and intraoperative monitoring is mandatory. The risk of CSF leakage can be markedly reduced by meticulous reconstruction of the dura and the placement of spinal drainage. Adequate exposition may sometimes require one or two levels of corpectomy with consequent vertebral body reconstruction and fixation of anterior column of the spine.