Changes in multi-modality management of acromegaly in a tertiary centre over 2 decades.

PURPOSE: Acromegaly is a rare disease associated with chronic multisystem complications. New therapeutic strategies have emerged in the last decades, combining pituitary transsphenoidal surgery (TSS), radiotherapy or radiosurgery (RXT) and medical treatments. METHODS: This retrospective monocentric study focused on presentation, management and outcome of acromegaly patients diagnosed between 2000 and 2020, still followed up in 2020, with a minimum follow-up of 1 year, and comparison of the first vs. second decade of the study. RESULTS: 275 patients were included, 50 diagnosed before 2010 and 225 after 2010. 95% of them had normal IGF-1 levels (with or without treatment) at the last follow-up. Transsphenoidal surgery was more successful after 2010 (75% vs. 54%; p < 0.01), while tumor characteristics remained the same over time. The time from first treatment to biochemical control was shorter after 2010 than before (8 vs. 16 months; p = 0.03). Since 2010, RT was used less frequently (10% vs. 32%; p < 0.01) but more rapidly after surgery (26 vs. 53 months; p = 0.03). In patients requiring anti-secretory drugs after TSS, the time from first therapy to biochemical control was shorter after 2010 (16 vs. 29 months; p < 0.01). Tumor size, tumor invasiveness, baseline IGF-1 levels and Trouillas classification were identified as predictors of remission. CONCLUSION: The vast majority of patients with acromegaly now have successful disease control with a multimodal approach. They reached biochemical control sooner in the most recent half of the study period. Future work should focus on those patients who are still uncontrolled and on the sequelae of the disease.

Innovative treatments for meningiomas.

Multi-recurrent high-grade meningiomas remain an unmet medical need in neuro-oncology when iterative surgeries and radiation therapy sessions fail to control tumor growth. Nevertheless, the last 10years have been marked by multiple advances in the comprehension of meningioma tumorigenesis via the discovery of new driver mutations, the identification of activated intracellular signaling pathways, and DNA methylation analyses, providing multiple potential therapeutic targets. Today, Anti-VEGF and mTOR inhibitors are the most used and probably the most active drugs in aggressive meningiomas. Peptide radioactive radiation therapy aims to target SSTR2A receptors, which are strongly expressed in meningiomas, but have an insufficient effect in most aggressive meningiomas, requiring the development of new techniques to increase the dose applied to the tumor. Based on the multiple potential intracellular targets, multiple targeted therapy clinical trials targeting Pi3K-Akt-mTOR and MAP kinase pathways as well as cell cycle and particularly, cyclin D4-6 are ongoing. Recently discovered driver mutations, SMO, Akt, and PI3KCA, offer new targets but are mostly observed in benign meningiomas, limiting their clinical relevance mainly to rare aggressive skull base meningiomas. Therefore, NF2 mutation remains the most frequent mutation and main challenging target in high-grade meningioma. Recently, inhibitors of focal adhesion kinase (FAK), which is involved in tumor cell adhesion, were tested in a phase 2 clinical trial with interesting but insufficient activity. The Hippo pathway was demonstrated to interact with NF2/Merlin and could be a promising target in NF2-mutated meningiomas with ongoing multiple preclinical studies and a phase 1 clinical trial. Recent advances in immune landscape comprehension led to the proposal of the use of immunotherapy in meningiomas. Except in rare cases of MSH2/6 mutation or high tumor mass burden, the activity of PD-1 inhibitors remains limited; however, its combination with various radiation therapy modalities is particularly promising. On the whole, therapeutic management of high-grade meningiomas is still challenging even with multiple promising therapeutic targets and innovations.

Distribution of metals and radionuclides in the lichens Cladonia rangiferina and C. mitis from the past uranium mining region of Elliot Lake, Ontario, Canada.

The present study was performed in the Elliot Lake area (Ontario, Canada), a site of uranium mining and milling for nearly 40 years between 1950's and 1990's. Although mining activities ceased in the mid-1990's, the site hosts several tailings management areas (TMAs) which are under ongoing rehabilitation and monitoring. Several surveys using lichens as a biomonitoring tool were completed in the 1980s and the 1990s to assess the levels of contaminants. The present survey aimed to re-visit the historical surveys, and to determine the current status of environmental recovery of the area. Our survey consisted of sampling two lichen species, Cladonia rangiferina and C. mitis, in an area covering up to 50 km from the former mining operation and the TMAs. The results reported in this work indicated that the levels of metals and radionuclides, diagnostic of mining operations, have decreased over time: particularly, the U, Th and Pb levels in both lichen species dropped by about two orders of magnitude by the 2020's compared to the 1980's. Likewise, the Cs-137 levels in both lichen species reflect present day global background. The study provides a new set of present-day regional baseline elemental concentrations for other metals that are associated with mining (Cd, As, Ti, Cs). Finally, there were weak but statistically significant differences in the levels of some elements (U, Th, Cd) between the two lichens, suggesting these two species might have different capture mechanisms or retention abilities.

Nocardia farcinica cerebral abscess: A systematic review of treatment strategies.

PURPOSE: Nocardia farcinica is an opportunistic pathogen causing mainly pneumonia in immunocompromised patients, complicated in almost one-third of the cases by a thick-walled multiloculated cerebral abscess which induces significant morbidity and mortality. This review aims to assess the optimal treatment strategy for N. farcinica cerebral abscess. METHODS: Report of a case. Medline database was used to conduct a systematic review from inception to January 2020 looking for English-language articles focused on N. farcinica cerebral abscess, in accordance with the PRISMA guidelines. RESULTS: The research yielded 54 articles for a total of 58 patients. N. farcinica cerebral abscess displayed three different neuroimaging patterns: a single multiloculated abscess in half of the cases, multiple cerebral abscesses, or a small paraventricular abscess with meningitis. The patients who benefited from surgical excision of the abscess showed a trend towards a lower risk of surgical revision (8% versus 31%, P=0.06) and a lower mortality rate (8% versus 23%, P=0.18) than patients who benefited from needle aspiration. Twenty-two percent of the patients benefited from microbiological documentation from another site with a mortality rate of 23%. CONCLUSION: Urgent multimodal MRI is necessary in face with clinical suspicion of cerebral nocardiosis. In case of single or multiple small cerebral abscesses, microbiological documentation can be obtained with puncture of pseudotumoral visceral lesions. In case of large or symptomatic cerebral abscess, an aggressive surgical excision seems a reliable option and can be preferred over needle aspiration. Long-term antibiotic therapy with cotrimoxazole is necessary thereafter.

Meningiomas in patients with long-term exposition to progestins: Characteristics and outcome.

OBJECTIVE: The aim of this study was to describe progestin-associated meningiomas' characteristics, outcome and management. MATERIAL AND METHODS: We included 53 patients operated on and/or followed in the department for meningioma with progestin intake longer than one year and with recent drug discontinuation. RESULTS: Cyproterone acetate (CPA), nomegestrol acetate (NomA), and chlormadinone acetate (ChlA) were involved in most cases. Mean duration of progestin drugs intake was 17.5 years. Tumors were multiple in 66% of cases and were located in the anterior and the medial skull base in 71% of cases. Transitional subtype represented 16/25 tumors; 19 meningiomas were WHO grade I and 6 were grade II. The rate of transitional subtype and skull base location was significantly higher compared to matched operated meningioma general population. No difference was observed given WHO classification. But Ki67 proliferation index tends to be lower and 5/6 of the WHO grade II meningiomas were classified as WHO grade II because of brain invasion. Strong progesterone receptors expression was observed in most cases. After progestin discontinuation, a spontaneous visual recovery was observed in 6/10 patients. Under CPA (n=24) and ChlA/NomA (n=11), tumor volume decreased in 71% and 18% of patients, was stabilized in 25% and 64% of patients, and increased in 4% and 18% of patients, respectively. Volume outcome was related to meningioma location. CONCLUSIONS: Outcome at progestins discontinuation is favorable but different comparing CPA versus ChlA-NomA and comparing tumor location. Long-term follow-up is required. In most cases, simple observation is recommended and surgery should be avoided.

Management of patients suffering from hemorrhagic intracranial metastases: Propositions to help the neurosurgeon in emergency situations based on a literature review.

INTRODUCTION: Brain metastases are the most common intracranial neoplasm in adult patients, and one of the fearsome complications proves to be intratumoral hemorrhage. The neurosurgical management of patients harboring a bleeding brain metastasis is not fully established and there is still today an ongoing debate on the optimal management of these patients. The aim of this article is to provide the neurosurgeons with practical tools to assist in their decision-making process in the management of BMs. METHODS: We conducted a literature review of the relevant Pubmed, Cochrane, and Google scholar-indexed articles published between 2000 and 2019. The following key words were entered in the Pubmed search engine: [metastasis], [metastases], [brain metastases], [brain metastasis], [hemorrhage], [hematoma], [blood clot], [intracerebral hemorrhage], [intracranial hemorrhage]. The review was performed in accordance with the PRISMA recommendations. RESULTS: Based on PubMed, Cochrane, and Google scholar, 459 articles were retained, 392 were then removed because of their non-adequacy with the topic and, 9 articles were removed because they were not written in English language. So, 58 articles were analyzed. Radiological evaluation is crucial, but few traps exist. The frequency of overall brain tumor-related with intracranial hematoma is 7.2%, with a higher frequency for secondary tumors. The local recurrence rate after resection of a hemorrhagic metastasis seems to be better probably because of an easier "en bloc" resection thanks to the hematoma. An atypical presentation is reported in up to 4% in patients with chronic or acute subdural hematoma. Patients with subarachnoid hemorrhage and epidural hematoma are rare. A clear-cut correlation between the incidence of bleeding event in brain mets and prior stereotactic radiosurgery was not established. CONCLUSION: The current literature pertaining to the neurosurgical management of acute bleeding in brain metastasis is scant and the level of evidence remains low (experts 'opinions; class C). Herein we suggest a flowchart to assist in dealing with those difficult patients.

Peritonitis caused by Roseomonas mucosa after ventriculoperitoneal shunt revision: a case report.

Ventriculoperitoneal shunt (VPS) is an adequate treatment for congenital hydrocephalus or chronic hydrocephalus in adults. Yet, it is a surgery associated with a significant rate of complications amongst neurosurgical procedures, with frequent shunt obstructions and infections. We report the first-ever case of peritonitis caused by Roseomonas mucosa, shortly after the revision of a VPS ventricular catheter. Hardware removal and proper antibiotic therapy led to the patient's recovery. Roseomonas mucosa is an opportunistic skin pathogen with an antibiotic resistance profile to many beta-lactamines and a tropism for indwelling catheters and post-operative period. Nowadays, it should be taken into account in case of infection of indwelling catheters and for some implantable medical devices.

The interest of intraoperative scanner coupled to neuronavigation in traumatic or oncologic fractures of the cervical and upper thoracic spine requiring vertebral body height restoring procedures.

OBJECT: In recent years, the classical vertebroplasty has tended to be replaced by vertebral augmentation procedures. This article discusses the reliability of vertebral augmentation procedures using balloon kyphoplasty or a spine jack system, with intraoperative CT scan control coupled with the neuronavigation system as a treatment option for cervical and upper thoracic spine lesions. METHODS: In our neurosurgical department, in the past two years, 11 patients underwent either a kyphoplasty or a vertebral augmentation by a Spine Jack via a transpedicular route, under perioperative 3D imaging, for a total of 15 cervical/upper thoracic lesions. For these patients, we evaluated the clinical symptoms before and after surgery, the intraoperative and postoperative complications as well as the radiation exposure and the duration of their hospitalisation. RESULTS: We noted for all of the patients an improvement of the mean Karnofsky index, which improved from 50 to 80, and of the VAS that decreased from a mean of 75 to 15, as they were clearly alleviated after the operation. The radiation was lower for patients that were treated for 2 or more vertebrae, and much lower for the medical staff. The intraoperative complications rate (4 cement leakages for 15 vertebrae, 26%), was low and completely asymptomatic in all cases. The radiological follow-up examinations were satisfactory for all the patients. CONCLUSIONS: Our results suggest that percutaneous transpedicular vertebral augmentation techniques using intraoperative CT scan are a viable treatment for secondary lesions or traumatic compression fractures of the cervical and upper thoracic spine.

Optimizing medical postoperative care: Role of the hospitalist in a department of adult neurosurgery. Prospective comparative observational study.

INTRODUCTION: There is no consensus on responsibilities in postoperative care in neurosurgery, although this has grown in recent decades with an aging population and increasing chronic diseases, making management more complex due to patient age and comorbidity. Co-management between surgeons and medical physician has emerged in the English-speaking world and in the private sector in France, in orthopedic, vascular and neurosurgical departments, which moreover increasingly call for medical involvement. The present study therefore addressed the question of the role of the hospitalist in optimizing medical perioperative care in neurosurgery. We analyzed postoperative medical and surgical complications, requests for specialist opinions and hospital stay according to the presence of hospitalists. MATERIAL AND METHOD: We carried out a 6-month prospective comparative observational study in the Department of Adult Neurosurgery of La Timone University Hospital Centre (Marseille, France), from September 1, 2017 to February 28, 2018. All cranial or spinal neurology cases, whether managed surgically or not, whether emergency or scheduled, were included and followed up for 1 to 4 months. Patients managed in functional neurosurgery, pediatric neurosurgery or housed in other departments were excluded. The 229-patient-cohort was divided into 2 arms over 2 months in 2 different units of the Department (Unit A or Unit B), according to allocation of the hospitalist (in September in unit A and October in unit B) with follow-up until the final check-up. "Hospitalist-neurosurgery team" co-management in postoperative care was compared versus standard postoperative care provided by neurosurgeons with medical consultation. Endpoints comprised: length of stay, number of inpatient postoperative complications and medium-term complications with re-admission, and number of medical consultations. RESULTS: Groups were comparable in number, age, gender, reasons for admission, type of admission (emergency or scheduled) and comorbidity scales (ASA and Charlson). Mean length of stay was significantly reduced, from 10.13±7.76 days (95% Confidence Interval, 8.77-11.49) to 7.07±3.94 days (95% CI, 6.31-7.82) (p=0.0087), without and with the presence of a hospitalist, respectively. The rate of requests for specialist opinion depended on the department in question but was likewise significantly reduced, from 22.81% in Unit A to 14.29%, and from 19.15% in Unit B to 8%. The 30% decrease in medical complications (45% of overall complications) was not statistically significant. There were significant correlations between age, comorbidity on Charlson scale and immediate (but not medium-term) complications. CONCLUSION: Postoperative care in neurosurgery can be improved by the presence of a hospitalist, reducing the rates of complications, requests for specialist opinion and hospital stay. The job description, work schedule and organization and financing remain to be defined. Studying other organizations to supplement the present benchmarking could help strike a better balance between time-saving and reinforcement of the medical-surgical team on the one hand and enhanced budgeting for comorbidities and care optimization on the other.

Minimally invasive posterior fixation and anterior debridement-fusion for thoracolumbar spondylodiscitis: A 40-case series and review of the literature.

INTRODUCTION: Pyogenic spondylodiscitis is a rare disease, but incidence is increasing. Reported failure rates following conservative management range from 12% to 18%. The purpose of this study was to determine the safety and efficacy of posterior percutaneous pedicle screw fixation combined with anterior debridement and fusion (ADF) for infective spondylodiscitis in the thoracic and/or lumbar spine. METHODS: The retrospective study cohort comprised all patients without neurological deficit who underwent minimally invasive posterior and anterior surgery between April 2008 and April 2016 for thoracic and/or lumbar spondylodiscitis. RESULTS: Forty patients were eligible (16 female: 40%). The lumbar region was affected in 31 cases (77.5%). Source of infection was identified in only 22 cases (55%) and bacteriological identification was obtained in 32 cases (80%). Mean hospital stay was 14.8 days (range, 6-39 days). Complete recovery was achieved in 39 patients (97.5%) at 3 months' follow-up. Mean preoperative local kyphosis angle was 16.1o, versus 14o at 1-year (P>0.05). 36 patients (90%) had at least 1 year's follow-up, and fusion was obtained for all these cases. CONCLUSION: Two-stage minimally invasive surgery is effective and safe for the treatment of single or two-level thoracolumbar spondylodiscitis. It could be an alternative to conventional open surgery or conservative treatment.

Acromegaly in Carney complex.

PURPOSE: Carney complex (CNC) is a rare autosomal dominant syndrome, characterized by mucocutaneous pigmentation, cardiac, cutaneous myxomas and endocrine overactivity. It is generally caused by inactivating mutations in the PRKAR1A (protein kinase cAMP-dependent type I regulatory subunit alpha) gene. Acromegaly is an infrequent manifestation of CNC, reportedly diagnosed in 10% of patients. METHODS: We here report the case of a patient who was concomitantly diagnosed with Carney complex, due to a new mutation in PRKAR1A ((NM_002734.3:c.80_83del, p.(Ile27Lysfs*101 in exon 2), and acromegaly. In parallel, we conducted an extensive review of published case reports of acromegaly in the setting of CNC. RESULTS: The 43-year-old patient was diagnosed with an acromegaly due to a GH-secreting pituitary microadenoma resistant to somatostatin analogs. He underwent transsphenoidal surgery in our tertiary referral center, which found a pure GH-secreting adenoma. In the literature, we identified 57 cases (24 men, 33 women) of acromegaly in CNC patients. The median age at diagnosis was 28.8 ± 12 year and there were 6 cases of gigantism. Acromegaly revealed CNC in only 4 patients. 24 patients had a microadenoma and two carried pituitary hyperplasia and/or multiple adenomas, suggesting that CNC may result in a higher proportion of microadenoma as compared to non-CNC acromegaly. CONCLUSIONS: Although it rarely reveals CNC, acromegaly is diagnosed at a younger age in this setting, with a higher proportion of microadenomas.

Management of two cavernous sinus dural arteriovenous fistulae by direct microsurgical approach and catheterization of the superior ophthalmic vein.

BACKGROUND: In case of cavernous sinus dural arteriovenous fistula, transvenous embolization of the cavernous sinus via the inferior petrosal sinus is generally sufficient. However, when inferior petrosal sinus access is challenging, various alternative approaches have been reported, with corresponding difficulties and risks. CASE REPORTS: We report the management of two cases of life-threatening cavernous sinus dural arteriovenous fistula revealed by a typical cavernous sinus syndrome. Conventional approaches were unsuccessful, and a direct microsurgical approach was performed, with catheterization of the superior ophthalmic vein. This combined approach safely accessed the cavernous sinus, and obtained complete occlusion of the fistulae by Onyx® embolization. CONCLUSIONS: This procedure could be an interesting alternative option in the treatment of cavernous sinus dural arteriovenous fistula when conventional approaches are not possible.

Pre-surgical medical treatment, a major prognostic factor for long-term remission in acromegaly.

PURPOSE: To determine whether pre-surgical medical treatment (PSMT) using long-acting Somatostatin analogues in acromegaly may improve long-term surgical outcome and to determine decision making criteria. METHODS: This retrospective study included 110 consecutive patients newly diagnosed with acromegaly, who underwent surgery in a reference center (Marseille, France). The mean long-term follow-up period was 51.4 ± 36.5 (median 39.4) months. Sixty-four patients received PSMT during 3-18 (median 5) months before pituitary surgery. Remission was defined at early (3 months) evaluation and at last follow-up by GH nadir after oral glucose tolerance test < 0.4 µg/L and normal IGF-1. RESULTS: Pretreated and non-pretreated groups were comparable for the main confounding factors except for higher IGF-1 at diagnosis in PSMT patients. Remission rates were significantly different in pretreated or not pretreated groups (61.1% vs. 36.6%, respectively at long-term evaluation). In multivariate analysis, PSMT was significantly linked to 3 months (p < 0.01) and long-term remission (p < 0.01). Duration of PSMT was not significantly different in cured or non-cured patients, at both evaluation times. PSMT appeared to be more beneficial for patients with an invasive tumor. No patient with a tumor greater than 18 mm or mean GH level exceeding 35 ng/mL at diagnosis was cured by surgery alone (vs. 8 and 9 patients in the pretreated group, respectively). Patients with PSMT showed more transient mild hyponatremia after surgery. CONCLUSIONS: PSMT significantly improved short and long-term remission in patients with acromegaly, independent of its duration, especially in invasive adenomas.

Active cushing syndrome patients have increased ectopic fat deposition and bone marrow fat content compared to cured patients and healthy subjects: a pilot 1H-MRS study.

OBJECTIVE: Glucocorticoid excess is one of the most important causes of bone disorders. Bone marrow fat (BMF) has been identified as a l new mediator of bone metabolism. Cushing syndrome (CS), is a main regulator of adipose tissue distribution but its impact on BMF is unknown. The objective of the study was to evaluate the effect of chronic hypercortisolism on BMF. DESIGN: This was a cross-sectional study. Seventeen active and seventeen cured ACTH-dependent CS patients along with seventeen controls (matched with the active group for age and sex) were included. METHODS: the BMF content of the femoral neck and L3 vertebrae were measured by 1H-MRS on a 3-Tesla wide-bore magnet. BMD was evaluated in patients using dual-energy X-ray absorptiometry. RESULTS: Active CS patients had higher BMF content both in the femur (82.5±2.6%) and vertebrae (70.1±5.1%) compared to the controls (70.8±3.6%, p=0.013 and 49.0±3.7% p=0.005, respectively). In cured CS patients (average remission time of 43 months), BMF content was not different from controls at both sites (72.3±2.9% (femur) and 46.7%±5.3% (L3)). BMF content was positively correlated with age, fasting plasma glucose, HbA1c, triglycerides and visceral adipose tissue in the whole cohort and negatively correlated with BMD values in the CS patients . CONCLUSIONS: Accumulation of BMF is induced by hypercortisolism. In remission patients BMF reached values of controls. Further studies are needed to determine whether this increase in marrow adiposity in CS is associated with bone loss.

Rupture limit evaluation of human cerebral aneurysms wall: Experimental study.

BACKGROUND AND PURPOSE: Rupture risk of intracranial aneurysms is a major issue for public healthcare. A way to obtain an individual rupture risk assessment is a main objective of many research teams in the world. For many years, we have investigated the relationship between the mechanical properties of aneurysm wall tissues and the rupture risk. In this work, we try to go further and investigate rupture limit values. METHODS: Following surgical clipping, a specific conservation protocol was applied to aneurysmal tissues in order to preserve their mechanical properties. Thirty-nine intracranial aneurysms (27 females, 12 males) were tested using a uniaxial tensile test machine under physiological conditions, temperature, and saline isotonic solution. These represented 24 unruptured and 15 ruptured aneurysms. Stress/strain curves were then obtained for each sample, and a fitting algorithm was applied following a Yeoh hyperelastic model with 2 parameters. Moreover, uniaxial tensile tests were conducted until rupture of samples to obtain values of stress and strain rupture limit. RESULTS: The significant parameter a C2 of the hyperelastic Yeoh model, allowed us to classify samples' rigidity following the terminology we adopted in previous papers (Costalat et al., 2011; Sanchez et al., 2013): Soft, Stiff and Intermediate. Moreover, strain/stress rupture limit values were gathered and analyzed thanks to the tissue rigidity, the status of the aneurysm (initially ruptured or unruptured) and the gender of the patient. CONCLUSION: Strain rupture limit was found quite stable around 20% and seems not to be correlated with the status of the aneurysm (initially ruptured or unruptured), neither with the gender of the patient. However, stretch and stress rupture limit seems not to be independent on the rigidity. The study confirms that ruptured aneurysms mainly present a soft tissue and unruptured aneurysms present a stiff material.

Lack of functional remission in Cushing's syndrome.

INTRODUCTION: Hypercortisolism leads to severe clinical consequences persisting after the onset of remission. These physical sequelae of cortisol exposure are known to profoundly impact the patient's quality of life. As psychological factors may be correlated with this quality of life, our objective was to determine the specific weight of psychological determinants of quality of life in patients in remission from hypercortisolism. PATIENTS AND METHODS: In an observational study, 63 patients with hypercortisolism in remission were asked to complete exhaustive self-administered questionnaires including quality of life (WHOQoL-BREF and Cushing QoL), depression, anxiety, self-esteem, body image, and coping scales. Multivariate analyses were performed. Psychological variables relevant to the model were: anxiety, depression, self-esteem, body image, and positive thinking dimension of the Brief-COPE. Cortisol deficiency was defined as a potential confounder. RESULTS: The median time since remission was 3 years. Patients had significantly lower quality of life and body satisfaction score than the French population and patients with chronic diseases. Depression significantly impaired all WHOQoL and Cushing QoL domains. A low body satisfaction score significantly impaired social relationships quality of life score. In total, 42.9% of patients still needed working arrangements, 19% had disability or cessation of work. CONCLUSION: Patients in biological remission of hypercortisolism can rarely be considered as functionally cured: this is evidenced by altered quality of life, working arrangements, and chronic depression. A multidisciplinary management of these patients is thus mandatory on a long-term basis.

Multiple neurosurgical treatments for different members of the same family with Currarino syndrome.

INTRODUCTION: Currarino's syndrome (CS) is an autosomal dominant disorder of embryonic development causing a rare malformating syndrome characterized by a triad of an anorectal malformations, presacral mass (most commonly an anterior sacral meningocele) and sacral bony defects. Mutations of the HLXB9 gene have been identified in most CS cases, but a precise genotype-phenotype correlation has not been described so far. Family screening is obligatory. The diagnosis is usually made during childhood and rarely in adulthood. In this context, imaging, and especially MRI plays a major role in the diagnosis of this syndrome. Surgical management is provided by pediatric surgeons or neurosurgeons. FAMILIAL CASE REPORT: Here, we present a family case report with CS requiring different neurosurgical management. The son, a 3-year-old boy, developed a tethered spinal cord syndrome associated to a lipoma of the filum terminale, a sacro-coccygeal teratoma and an anal adhesion. A combined surgical approach permitted a good evolution on the urinary and digestive functions despite a persistent fecal incontinence. The 2-year-old daughter presented with a cyst of the thyreoglossal tract infected and fistulized to the skin. She was also followed for a very small lipoma of the filum terminale that required a neurosurgical approach. The father, 44-year-old, manifested functional digestive and urinary disorders caused by a giant anterior sacral meningocele. The ligation of the neck of the cyst and aspiration of the liquid inside in full through a posterior partial approach permit a complete collapse of the cyst with an instantly satisfactory clinical outcome. CONCLUSION: In these cases, cooperation between pediatric surgeons and neurosurgeons was crucial. The follow-up of these patients should be done in a spina bifida clinic. A geneticist evaluation must be offered to the patient in the case of a CS as well as a clinical evaluation of the relatives (parents, siblings).

Adjacent level spondylodiscitis in a patient with thoracic spondylodiscitis: A case report and review of the literature.

INTRODUCTION: Adjacent level spondylodiscitis (ALS) after primary surgery for thoracic spondylodiscitis is a very rare condition. CASE REPORT: We report the case of a 76-year-old man with this pathology. A first posterior minimally invasive approach combined with anterior approach to the thoracic spine was safely performed for thoracic spondylodiscitis. More than a year later, exploration of recurrent symptoms with 18FDG PET scan helped to diagnose ALS. Further surgery was performed. At 3-year follow-up examination showed no recurrence of the infection. DISCUSSION: ALS should be suspected during recurrent symptoms after spinal fusion surgery. Evaluation should be based on the results of 18FDG PET scan and surgery. CONCLUSION: Bacterial and histopathological analyses combined with an increase of spine fixation and adapted antimicrobial therapy are a safe management for ALS.