Patient empowerment improves follow-up data collection after fetal surgery for spina bifida: institutional audit.

OBJECTIVES: To define and grade fetal and maternal adverse events following fetal surgery for spina bifida and to report on the impact of engaging patients in collecting follow-up data. METHODS: This prospective single-center audit included 100 consecutive patients undergoing fetal surgery for spina bifida between January 2012 and December 2021. In our setting, patients return to their referring unit for further pregnancy care and delivery. On discharge, referring hospitals were requested to return outcome data. For this audit, we prompted patients and referring hospitals to provide data in cases of missing outcomes. Outcomes were categorized as missing, returned spontaneously or returned following additional request, by the patient and/or referring center. Postoperative maternal and fetal complications until delivery were defined and graded according to Maternal and Fetal Adverse Event Terminology (MFAET) and the Clavien-Dindo classification. RESULTS: There were no maternal deaths, but severe maternal complications occurred in seven women (anemia in pregnancy, postpartum hemorrhage, pulmonary edema, lung atelectasis, urinary tract obstruction and placental abruption). No cases of uterine rupture were reported. Perinatal death occurred in 3% of fetuses and other severe fetal complications in 15% (perioperative fetal bradycardia/cardiac dysfunction, fistula-related oligohydramnios, chorioamnionitis and preterm prelabor rupture of membranes (PPROM) before 32 weeks). PPROM occurred in 42% of patients and, overall, delivery took place at a median gestational age of 35.3 weeks (interquartile range, 34.0-36.6 weeks). Information provided following additional request, from both centers and patients but mainly from the latter, reduced missing data by 21% for gestational age at delivery, 56% for uterine-scar status at birth and 67% for shunt insertion at 12 months. Compared with the generic Clavien-Dindo classification, the MFAET system ranked complications in a more clinically relevant way. CONCLUSIONS: The nature and rate of severe complications following fetal surgery for spina bifida were similar to those reported in other large series. Spontaneous return of outcome data by referring centers was low, yet patient empowerment improved data collection. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.

Neuroprotection is improved by watertightness of fetal spina bifida repair in the sheep model.

OBJECTIVES: A contributing factor to unsuccessful prenatal spina bifida aperta (SBA) repair via an open approach may be incomplete neurosurgical repair causing persistent in-utero leakage of cerebrospinal fluid (CSF) and exposure of the fetal spinal cord to amniotic fluid. We aimed to investigate the neurostructural and neurofunctional efficacy of watertight prenatal SBA repair in a validated SBA fetal lamb model. METHODS: A well-powered superiority study was conducted in the validated SBA fetal lamb model (n = 7 per group). The outcomes of lambs which underwent watertight or non-watertight multilayer repair through an open approach were compared to those of unrepaired SBA lambs (historical controls) at delivery (term = 145 days). At ∼75 days, fetal lambs underwent standardized induction of lumbar SBA. At ∼100 days, they were assigned to an either watertight or non-watertight layered repair group based on an intraoperative watertightness test using subcutaneous fluorescein injection. At 1-2 days postnatally, as primary outcome, we assessed reversal of hindbrain herniation using magnetic resonance imaging (MRI). Secondary proxies of neuroprotection were: absence of CSF leakage at the repair site; hindlimb motor function based on joint-movement score, locomotor grade and Motor Evoked Potential (MEP); four-score neuroprotection scale, encompassing live birth, complete hindbrain herniation reversal, absence of CSF leakage and joint-movement score ≥ 9/15; and brain and spinal cord histology and immunohistochemistry. As the watertightness test cannot be used clinically due to its invasiveness, we developed a potential surrogate intraoperative three-score skin-repair-quality scale based on visual assessment of the quality of the skin repair (suture inter-run distance ≤ 3 mm, absence of tear and absence of ischemia), with high quality defined by a score ≥ 2/3 and low quality by a score < 2/3, and assessed its relationship with improved outcome. RESULTS: Compared with unrepaired lambs, lambs with watertight repair achieved a high level of neuroprotection (neuroprotection score of 4/4 in 5/7 vs 0/7 lambs) as evidenced by: a significant 100% (vs 14%) reversal of hindbrain herniation on MRI; low CSF leakage (14% vs 100%); better hindlimb motor function, with higher joint-movement score, locomotor grade and MEP area under the curve and peak-to-peak amplitude; higher neuronal density in the hippocampus and corpus callosum; and higher reactive astrogliosis at the SBA lesion epicenter. Conversely, lambs with non-watertight SBA repair did not achieve the same level of neuroprotection (score of 4/4 in 1/7 lambs) compared with unrepaired lambs, with: a non-significant 86% (vs 14%) reversal of hindbrain herniation; high CSF leakage (43% vs 100%); no improvement in motor function; low brain neuron count in both the hippocampus and corpus callosum; and small spinal astroglial cell area at the epicenter. Both watertight layered repair and high (≥ 2/3) intraoperative skin-repair-quality score were associated with improved outcome, but the watertightness test and skin-repair-quality scale could not be used interchangeably due to result discrepancies. CONCLUSIONS: Watertight layered fetal SBA repair is neuroprotective since it improves brain and spinal-cord structure and function in the fetal lamb model. This translational research has important clinical implications. A neurosurgical technique that achieves watertightness should be adopted in all fetal centers to improve neuroprotection. Future clinical studies could assess whether a high skin-repair-quality score (≥ 2/3) correlates with neuroprotection. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Learning curves of open and endoscopic fetal spina bifida closure: systematic review and meta-analysis.

OBJECTIVE: The Management of Myelomeningocele Study (MOMS) trial demonstrated the safety and efficacy of open fetal surgery for spina bifida aperta (SBA). Recently developed alternative techniques may reduce maternal risks without compromising the fetal neuroprotective effects. The aim of this systematic review was to assess the learning curve (LC) of different fetal SBA closure techniques. METHODS: MEDLINE, Web of Science, EMBASE, Scopus and Cochrane databases and the gray literature were searched to identify relevant articles on fetal surgery for SBA, without language restriction, published between January 1980 and October 2018. Identified studies were reviewed systematically and those reporting all consecutive procedures and with postnatal follow-up ≥ 12 months were selected. Studies were included only if they reported outcome variables necessary to measure the LC, as defined by fetal safety and efficacy. Two authors independently retrieved data, assessed the quality of the studies and categorized observations into blocks of 30 patients. For meta-analysis, data were pooled using a random-effects model when heterogeneous. To measure the LC, we used two complementary methods. In the group-splitting method, competency was defined when the procedure provided results comparable to those in the MOMS trial for 12 outcome variables representing the immediate surgical outcome, short-term neonatal neuroprotection and long-term neuroprotection at ≥ 12 months of age. Then, when raw patient data were available, we performed cumulative sum analysis based on a composite binary outcome defining successful surgery. The composite outcome combined four clinically relevant variables for safety (absence of extreme preterm delivery < 30 weeks, absence of fetal death ≤ 7 days after surgery) and efficacy (reversal of hindbrain herniation and absence of any neonatal treatment of dehiscence or cerebrospinal fluid leakage at the closure site). RESULTS: Of 6024 search results, 17 (0.3%) studies were included, all of which had low, moderate or unclear risk of bias. Fetal SBA closure was performed using standard hysterotomy (11 studies), mini-hysterotomy (one study) or fetoscopy by either exteriorized-uterus single-layer closure (one study), percutaneous single-layer closure (three studies) or percutaneous two-layer closure (one study). Only outcomes for standard hysterotomy could be meta-analyzed. Overall, outcomes improved significantly with experience. Competency was reached after 35 consecutive cases for standard hysterotomy and was predicted to be achieved after ≥ 57 cases for mini-hysterotomy and ≥ 56 for percutaneous two-layer fetoscopy. For percutaneous and exteriorized-uterus single-layer fetoscopy, competency was not reached in the 81 and 28 cases available for analysis, respectively, and LC prediction analysis could not be performed. CONCLUSIONS: The number of cases operated is correlated with the outcome of fetal SBA closure, and the number of operated cases required to reach competency ranges from 35 for standard hysterotomy to ≥ 56-57 for minimally invasive modifications. Our observations provide important information for institutions looking to establish a new fetal center, develop a new fetal surgery technique or train their team, and inform referring clinicians, potential patients and third parties. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Curvas de aprendizaje del cierre de la espina bífida fetal mediante cirugía abierta y endoscópica: revisión sistemática y metaanálisis OBJETIVO: El ensayo del Estudio sobre la Gestión del Mielomeningocele (MOMS, por sus siglas en inglés) demostró la seguridad y eficacia de la cirugía fetal abierta para la espina bífida aperta (EBA). Las técnicas alternativas recientemente desarrolladas pueden reducir los riesgos de la madre sin comprometer los efectos neuroprotectores del feto. El objetivo de esta revisión sistemática fue evaluar la curva de aprendizaje (CA) de diferentes técnicas de cierre de la EBA fetal. MÉTODOS: Se realizaron búsquedas en las bases de datos de MEDLINE, Web of Science, EMBASE, Scopus y Cochrane, así como en la literatura gris, para identificar artículos relevantes sobre cirugía fetal para la EBA, sin restricción de idioma, publicados entre enero de 1980 y octubre de 2018. Se examinaron sistemáticamente los estudios identificados y se seleccionaron los que informaban de todos los procedimientos consecutivos y con seguimiento postnatal ≥12 meses. Los estudios se incluyeron sólo si informaban sobre las variables de resultado necesarias para medir la CA, definidas por la seguridad y la eficacia para el feto. Dos autores recuperaron los datos de forma independiente, evaluaron la calidad de los estudios y clasificaron las observaciones en bloques de 30 pacientes. Para el metaanálisis, los datos se agruparon mediante un modelo de efectos aleatorios cuando fueron heterogéneos. Para medir la CA, se usaron dos métodos complementarios. En el método de división de grupos, la competencia se definió cuando el procedimiento proporcionó resultados comparables a los del ensayo MOMS para 12 variables de resultados que representaban el resultado quirúrgico inmediato, la neuroprotección neonatal a corto plazo y la neuroprotección a largo plazo a ≥12 meses de edad. Luego, cuando se dispuso de los datos brutos de los pacientes, se realizó un análisis de suma acumulada basado en un resultado binario compuesto que definió el éxito de la cirugía. El resultado compuesto combinó cuatro variables clínicamente relevantes en cuanto a la seguridad (ausencia de parto pretérmino extremo <30 semanas; ausencia de muerte fetal a ≤7 días después de la cirugía) y eficacia (reducción de la hernia del rombencéfalo y ausencia de cualquier tratamiento neonatal de dehiscencia o derrame de líquido cefalorraquídeo en el lugar del cierre). RESULTADOS: De los 6024 resultados de la búsqueda, se incluyeron 17 (0,3%) estudios, todos ellos con un riesgo de sesgo bajo, moderado o incierto. El cierre de la EBA fetal se realizó mediante histerotomía estándar (11 estudios), mini histerotomía (un estudio) o fetoscopia, ya fuera mediante el cierre exteriorizado del útero de una sola capa (un estudio), el cierre percutáneo de una sola capa (tres estudios) o el cierre percutáneo de dos capas (un estudio). Sólo se pudieron metaanalizar los resultados de la histerotomía estándar. En general, los resultados mejoraron significativamente con la experiencia. Se alcanzó la competencia después de 35 casos consecutivos para la histerotomía estándar y se predijo que se alcanzaría después de ≥57 casos para la mini histerotomía y ≥56 para la fetoscopia percutánea de dos capas. En el caso de las fetoscopias percutánea y exteriorizada del útero de una sola capa, no se alcanzó la competencia en los 81 y 28 casos disponibles para el análisis, respectivamente, y no se pudo realizar el análisis de predicción de la CA. CONCLUSIONES: El número de casos operados está correlacionado con el resultado del cierre de la EBA fetal, y el número de casos operados necesarios para alcanzar la competencia estuvo entre 35 para la histerotomía estándar y ≥56-57 para las operaciones con mínima agresividad. Las observaciones realizadas proporcionan información importante para las instituciones que buscan establecer un nuevo centro fetal, desarrollar una nueva técnica de cirugía fetal o entrenar a su equipo, e informar a los médicos que remiten a especialistas a los posibles pacientes y a terceros. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Posterior fossa decompression for Chiari malformation type I: clinical and radiological presentation, outcome and complications in a retrospective series of 105 procedures.

OBJECTIVE: Determining clinical and radiological characteristics, complication rates and outcome for patients undergoing posterior fossa decompression (PFD) and duraplasty for Chiari malformation type I (CM-I). METHODS AND MATERIALS: Retrospective, single-university hospital study of all PFDs for CM-I between January 1995 and December 2016. RESULTS: PFD was performed in 105 patients with CM-I (n = 105), of whom 62 suffered from associated syringomyelia and 37 were pediatric cases. There was a female preponderance in the syringomyelia, non-syringomyelia, pediatric and adult groups (62.9%, 65.1%, 54.1% and 69.1%, respectively). Mean age at diagnosis was higher in the syringomyelia group (32.3 versus 26.9 years, p = 0.06) and in both groups delay for diagnosis was long (33.4 and 47.1 months, p = 0.50). Headache during Valsalva maneuver and on exertion was significantly more present in the non-syringomyelia group (27.9% versus 16.1%, p < 0.0001 and 39.5% versus 11.3%, p = 0.0009); cranial nerve dysfunction and cerebellar signs did not differ significantly (p = 0.07 and p = 0.53). Spinal cord syndrome was significantly more present in the syringomyelia group (72.6% versus 25.6%, p < 0.0001). Scoliosis was present in five patients, of whom four had syringomyelia. Duraplasty was performed in 101 surgeries. For 100 patients, PFD was the primary treatment of CM-I; 2 patients had previously undergone syrinx drainage and 3 ventriculoperitoneal shunting for hydrocephalus. Only one patient presented with hydrocephalus. Duraplasty (n = 101) was complicated by CSF leak in 4.0% (n = 4), symptomatic pseudomeningocele in 4.0% (n = 4), aseptic meningitis in 2.0% (n = 2) and hydrocephalus in 1.0% (n = 1). Osseous decompression without duraplasty was performed in only four highly selected patients, not allowing a comparative analysis with duraplasty. Post-operative symptom improvement is reported for 67.3% of all patients and stabilization of symptoms in 23.9%. Symptom improvement was significantly more frequent in the non-syringomyelia group (p = 0.03). Outcome seemed similarly good in the pediatric and the adult groups. CONCLUSION: PFD performed with duraplasty is a safe procedure. The majority of patients do report symptom stabilization or improvement. Non-syringomyelia patients reported significantly more frequent improvement of clinical symptoms.

Neurodevelopmental outcome of children with spina bifida aperta repaired prenatally vs postnatally: systematic review and meta-analysis.

OBJECTIVE: To assess the neurodevelopmental outcome of children with spina bifida aperta (SBA) treated prenatally as compared to those treated postnatally. METHODS: We performed a systematic review of the literature in PubMed/MEDLINE, EMBASE, Web of Science and The Cochrane Library, comparing the neurological outcome of infants with SBA treated prenatally vs postnatally. Only randomized controlled trials (RCTs) and non-randomized prospective controlled studies were included. The primary outcome assessed was neurodevelopmental impairment at the age of 1 year or later. Secondary outcomes were preterm birth, need for ventriculoperitoneal (VP) shunt by 12 months of age, absence of signs of hindbrain herniation at the first postnatal magnetic resonance imaging (MRI) evaluation and independent ambulation evaluated at 30 months. RESULTS: Of 11 359 studies identified through the electronic search, six met the inclusion criteria and were assessed in full text and two, one RCT and one prospective cohort study, were ultimately included in the meta-analysis. Sensitivity analysis did not show any difference between the outcomes of the RCT alone and those of the pooled RCT and prospective cohort study. This allowed neurodevelopmental assessment of 213 children between 14 and 53 months of age. Neurodevelopment was assessed by the Bayley Scales of Infant Development II (BSID-II) mental development index corrected for chronological age, with a cut-off of ≥ 70 (representing no more than 2 SD below the mean). The presence of neurodevelopmental impairment was similar between children who underwent prenatal (25/105 (23.8%)) and those who had postnatal (30/108 (27.8%)) repair of SBA (odds ratio (OR), 0.82 (95% CI, 0.43-1.56); P = 0.54), although the risk of prematurity was higher in the prenatal-repair group (OR, 17.62 (95% CI, 7.60-40.87); P < 0.0001). For every two fetuses operated on before birth, there was, compared with those operated on after birth, one additional premature birth (number needed to harm = 2 (95% CI, 1-3)). The need for VP shunt placement by 12 months of age was lower in the prenatal-repair group (45/109 (41.3%)) than in children that had postnatal repair (93/112 (83.0%); OR, 0.14 (95% CI, 0.08-0.26); P < 0.0001). Data on neurodevelopmental impairment in children with a shunt were available only for patients from the prenatal-surgery group of the RCT; in this subgroup, the likelihood for impairment was similar between children who did (7/39 (17.9%)) and those who did not (4/48 (8.3%)) have shunt placement (P = 0.21). At first postnatal MRI evaluation, no signs of hindbrain herniation were detected in 28/88 (31.8%) children who were operated on prenatally compared with 4/89 (4.5%) who had postnatal repair (OR, 9.45 (95% CI, 3.12-28.64); P < 0.0001). Independent ambulation at 30 months was achieved by 41/109 (37.6%) children who underwent prenatal repair compared with 21/111 (18.9%) who had postnatal repair (OR, 2.59 (95% CI, 1.39-4.86); P = 0.003). CONCLUSION: The risk of neurodevelopmental impairment in infants with SBA was similar between those who underwent prenatal and those who had postnatal surgical repair, despite an increased risk of prematurity in the prenatally repaired group. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Reliability of MR Imaging-Based Posterior Fossa and Brain Stem Measurements in Open Spinal Dysraphism in the Era of Fetal Surgery.

BACKGROUND AND PURPOSE: Fetal MR imaging is part of the comprehensive prenatal assessment of fetuses with open spinal dysraphism. We aimed to assess the reliability of brain stem and posterior fossa measurements; use the reliable measurements to characterize fetuses with open spinal dysraphism versus what can be observed in healthy age-matched controls; and document changes in those within 1 week after prenatal repair. MATERIALS AND METHODS: Retrospective evaluation of 349 MR imaging examinations took place, including 274 in controls and 52 in fetuses with open spinal dysraphism, of whom 23 underwent prenatal repair and had additional early postoperative MR images. We evaluated measurements of the brain stem and the posterior fossa and the ventricular width in all populations for their reliability and differences between the groups. RESULTS: The transverse cerebellar diameter, cerebellar herniation level, clivus-supraocciput angle, transverse diameter of the posterior fossa, posterior fossa area, and ventricular width showed an acceptable intra- and interobserver reliability (intraclass correlation coefficient > 0.5). In fetuses with open spinal dysraphism, these measurements were significantly different from those of healthy fetuses (all with P < .0001). Furthermore, they also changed significantly (P value range = .01 to < .0001) within 1 week after the fetal operation with an evolution toward normal, most evident for the clivus-supraocciput angle (65.9 ± 12.5°; 76.6 ± 10.9; P < .0001) and cerebellar herniation level (-9.9 ± 4.2 mm; -0.7 ± 5.2; P < .0001). CONCLUSIONS: In fetuses with open spinal dysraphism, brain stem measurements varied substantially between observers. However, measurements characterizing the posterior fossa could be reliably assessed and were significantly different from normal. Following a fetal operation, these deviations from normal values changed significantly within 1 week.

Pulmonary metastasis of a meningioma presenting as a solitary pulmonary nodule: 2 case reports.

Distant metastases of meningioma are rare, especially in grade 1 meningiomas. In a recent literature review, only 115 cases were found. In almost all published cases, the meningioma was treated several years before the metastasis was diagnosed. The lungs are the most frequent site of metastasis. We describe two patients treated for meningioma (one case grade 1, the other grade 3) who were referred to the Respiratory Oncology Unit because of the incidental finding of a pulmonary nodule on routine chest radiography. Both had undergone several neurosurgical procedures but the last operation was more than 7 years before in both cases. Positron emission tomography scan was suggestive of a malignant lung tumour. The lesions were surgically removed. Pathology confirmed meningioma in both cases with the same WHO grade, immunohistochemical and genetic profiles as the original meningioma. Both patients recovered well from thoracic surgery. The patient with grade 3 meningioma died three years later from intracranial recurrence. When a patient previously treated for meningioma develops a nodular lung lesion, metastasis of the meningioma should be in the differential diagnosis list. Because of the occurrence of distant metastasis even in grade I meningiomas, we suggest that the grading system should take into account genetic changes in the meningioma. Chromosome 1p and 14q losses possibly explain the aggressive behaviour of the grade 1 meningioma.

Outcome of 12 antenatally diagnosed fetal arachnoid cysts: case series and review of the literature.

OBJECTIVES: To investigate the natural history, associated abnormalities and outcome of 12 fetuses with arachnoid cyst diagnosed antenatally by ultrasound and magnetic resonance imaging and to compare the outcome with cases in the literature. METHODS: A retrospective study of all cases of antenatally detected fetal arachnoid cysts was performed in patients referred to a tertiary unit between 2007 and 2013. Associated abnormalities, pregnancy outcome and postnatal follow-up were analyzed. All papers about prenatally diagnosed arachnoid cysts, of the last 30 years, were evaluated (search terms in Pubmed: "prenatal diagnosis", "Arachnoid Cysts"). RESULTS: Fetal arachnoid cysts were diagnosed in 12 fetuses, 9 were females. The mean gestational age of diagnosis was 28 1/7 (range 19 1/7-34 2/7 weeks). A total of 9 cases were supratentorial, 3 were located in the posterior fossa. In 10 cases a fetal MRI was performed which confirmed brain compression in 4 out of 5 supratentorial arachnoid cyst. MRI did not reveal other malformations nor signs of nodular heterotopia. Only one fetus presented with additional major anomalies (bilateral ventricumomegaly of >20 mm and rhombencephalosynapsis) leading to a termination of pregnancy. Two neonates underwent endoscopic fenestration of the arachnoid cyst in the first week of life with no additional intervention in childhood. All but one (10/11) had a favorable postnatal outcome. This child suffered from visual impairment at autism was diagnosed at the age of 5. One child had a surgical correction of strabismus later in childhood. In one child the infratentorial arachnoid cyst regressed spontaneously on ultrasound and MRI in the postnatal period. CONCLUSIONS: The majority of arachnoid cysts in this series are of benign origin and remain stable. Based on the current series and the review of the literature, in the absence of other associated anomalies and when the karyotype is normal, the postnatal overall and neurological outcome is favorable. Large suprasellar arachnoid cysts however, may cause visual impairment and endocrinological disturbances. Rarely associated cerebral or cerebellar malformations are present. Modern postnatal management of suprasellar arachnoid cyst consists of endoscopic cystoventriculostomy.

Defining pseudoprogression in glioblastoma multiforme.

BACKGROUND AND PURPOSE: Pseudoprogression is a frequent phenomenon observed since the introduction of postoperative therapy with radiotherapy and temozolomide (RT/TMZ) in glioblastoma multiforme (GBM) patients. However, the criteria defining pseudoprogression, its incidence, the time of occurrence and its impact on therapy and outcome remain poorly defined. METHODS: The objective of this study is to compare two sets of criteria (liberal and stringent), defining pseudoprogression, in a cohort of patients treated before and after the introduction of RT/TMZ in the standard postoperative treatment. This retrospective review includes 136 unselected and consecutively treated patients with pathologically diagnosed GBM. RESULTS: Pseudoprogression was observed in 10 (12%) cases applying the stringent criteria, and in 18 (23%) patients when using the liberal criteria, in the cohort treated with RT/TMZ. Pseudoprogression was observed in only one patient treated with RT alone. The median time to pseudoprogression was 4 weeks after the end of RT. Patients with pseudoprogression had a median survival time of 28 months, compared with 12 months for patients without pseudoprogression. CONCLUSIONS: The incidence of pseudoprogression after RT/TMZ strongly depends on the applied criteria. However, regardless of the stringency of the criteria, the impact on survival remains the same.

Spinal dermal sinuses and dermal sinus-like stalks analysis of 14 cases with suggestions for embryologic mechanisms resulting in dermal sinus-like stalks.

BACKGROUND: Spinal dermal sinuses consist of an epithelium-lined tract extending from the skin towards the spinal cord, often resulting in infections or tethered cord syndrome. Recently, a variant called dermal sinus-like stalk was described as an analogous tract but not containing an epithelium-lined lumen. AIMS: We aimed to describe the findings in our patients, subdivide our specimens into both conditions, compare the characteristics of both groups and search for possible embryologic mechanisms of dermal sinus-like stalks. METHODS: We performed a retrospective analysis of all patients operated in our hospital for both conditions between 1996 and 2012. RESULTS: 14 patients were operated upon for spinal dermal sinuses (n = 5) and spinal dermal sinus like-stalks (n = 9). Patients were mainly referred from other hospitals due to skin abnormalities and were evaluated at mean age of 7 weeks and operated upon at mean age of 1 year and 2 months. Primary reason for referral was skin abnormalities in both groups, though there were two cases of meningitis in dermal sinus patients and 2 of recurrent urinary tract infections in dermal sinus-like stalk patients. Consistent with previous findings, dermal sinus-like stalk patients do not have a history of meningitis, lack dermoid or epidermoid tumours along their tract, and are histologically of pure mesodermal origin. Dermal sinus-like stalks might result from interposition of mesenchyme during primary or secondary neurulation. CONCLUSIONS: We consider dermal sinus-like stalks as a rare but currently under diagnosed condition with different clinical, pathological and probably also embryologic characteristics compared to spinal dermal sinuses.

Oesophageal perforation after anterior cervical surgery: management in four patients.

BACKGROUND: Oesophageal perforation related to anterior cervical surgery is an uncommon but well recognised and potentially life-threatening complication with an incidence of 0-3.4%. Our experience with this complication and a review of the literature are presented. METHOD: We retrospectively reviewed our clinical experience over 10 years and found four patients in whom an oesophageal perforation was recognised after anterior surgery for cervical spine trauma. In three patients the perforation was noticed in the early post-operative period and the other had a delayed presentation. In all patients, the hardware was removed, long-term intravenous antibiotics were administered and parenteral nutrition was instituted. In two patients a primary suture of the perforation was performed and in one of these an additional sternocleidomastoid myoplasty was carried out as well. One patient had conservative treatment and one died before closure of the perforation could be performed. FINDINGS: The two patients, in whom surgical repair of the perforation was performed, recovered well with residual neurological deficits as expected due to the cervical trauma. In the patient in whom conservative treatment was instituted, healing of the perforation occurred. One patient died due to systemic complications, indirectly related to the perforation. CONCLUSIONS: Although not very frequent and sometimes difficult to diagnose, oesophageal perforations after anterior cervical surgery constitute a potentially life-threatening complication. Diagnosis is made by imaging or endoscopic studies, but clinical suspicion is most important. Basic treatment consists of surgery with removal of hardware, drainage of abscesses, primary closure of the perforation if possible, parenteral nutrition and antibiotic therapy. Residual instability should be recognised in time and may be anticipated in patients in whom there has been little time for solid bony fusion. Successful management depends on early diagnosis and immediate institution of treatment.

Risk analysis of thrombo-embolic and recurrent bleeding events in the management of intracranial haemorrhage due to oral anticoagulation.

PURPOSES: Intracranial haemorrhage (ICH) is a rare but potentially devastating complication of oral anticoagulants (OAC). This raises the difficult clinical choice between either permanent cessation of OAC, or continuing OAC and if so, when to restart. To make this choice, one needs to balance the thrombo-embolic risk after cessation of OAC against the risk of recurrent intracranial haemorrhage when OAC are restarted. There are few published data to base this difficult clinical decision on. METHODS: We present an observational study of a consecutive series of 108 patients, collected prospectively and admitted to our department, with an OAC-related intracranial haemorrhage, in whom we assessed the thrombotic event rate and the recurrent intracranial bleeding rate during follow-up. RESULTS: In the 25 patients in whom OAC were reinstituted no new thrombo-embolic events occurred (0/506 unprotected patient-days). In the group of patients in whom OAC were not restarted (n = 81), the thrombo-embolic event rate was 8/11590 unprotected patient-days, of which only 2 were cerebrovascular thrombo-embolisms. The overall risk of a thrombo-embolic complication can be estimated to be 0.66 events/1000 patient-days at risk (95% exact confidence limits of 0.3 to 1.3 events/1000 patient-days at risk). In three patients the thrombo-embolic event was fatal. We saw recurrent intracranial bleeding in eight patients, 2 of which were fatal. Seven of these occurred before the restarting of the OAC. CONCLUSIONS: In OAC-related intracranial haemorrhages, OAC can be stopped safely for a considerable period, with a very low overall thrombotic event rate. The recurrent bleeding risk after restarting OAC is low. Recurrent bleeding mostly occurred before restarting OAC and is probably caused by insufficient or unsustained correction of the initial coagulation deficit. Immediate reversal of anticoagulation provides the patient with the best possible treatment options including surgery. OAC-related intracranial haemorrhages can therefore be actively treated.

Surgery and adjuvant dendritic cell-based tumour vaccination for patients with relapsed malignant glioma, a feasibility study.

Patients with relapsed malignant glioma have a poor prognosis. We developed a strategy of vaccination using autologous mature dendritic cells loaded with autologous tumour homogenate. In total, 12 patients with a median age of 36 years (range: 11-78) were treated. All had relapsing malignant glioma. After surgery, vaccines were given at weeks 1 and 3, and later every 4 weeks. A median of 5 (range: 2-7) vaccines was given. There were no serious adverse events except in one patient with gross residual tumour prior to vaccination, who repetitively developed vaccine-related peritumoral oedema. Minor toxicities were recorded in four out of 12 patients. In six patients with postoperative residual tumour, vaccination induced one stable disease during 8 weeks, and one partial response. Two of six patients with complete resection are in CCR for 3 years. Tumour vaccination for patients with relapsed malignant glioma is feasible and likely beneficial for patients with minimal residual tumour burden.

Jugular foramen schwannoma: surgical experience in six cases.

PURPOSE: Schwannomas of the lower cranial nerves are rare tumours. We have surgical experience in six patients. MATERIAL AND METHODS: We have retrospectively reviewed the files of the six patients with jugular foramen schwannomas that were treated in multidisciplinary collaboration in the departments of neurosurgery and ENT. RESULTS: From a clinical point of view, the most important finding was that these tumours usually mimic a vestibular schwannoma. The imaging findings however always allowed the differentiation. The surgical technique included in all cases a suboccipital approach for the intracranial part. When large, the extracranial extension had to be removed through a lateral cervical or transfacial approach. There was no mortality and minor morbidity, with good functional outcome. In subtotally removed tumours, recurrence occurred. CONCLUSION: A combined neurosurgical and ENT approach allows for safe removal of these often large skull base tumours.

A clinical comparison of non-traumatic acute subdural haematomas either related to coagulopathy or of arterial origin without coagulopathy.

BACKGROUND: Non-traumatic acute subdural haematomas enable study of the morbidity and mortality due to the haematoma without the effect of trauma. Whereas it is known that coagulation disorders worsen the outcome of spontaneous intracerebral haematomas, this has not been studied in non-traumatic acute subdural haematomas. METHODS: In a series of 13 non-traumatic acute subdural haematomas admitted to our department between January 1995 and March 2002, we had 9 coagulopathy associated haematomas and 3 haematomas corresponding to the syndrome of 'spontaneous acute subdural haematoma of arterial origin'. Both groups were compared. FINDINGS: Age and gender distribution were comparable. The bleeding source was a cortical artery in 2 of the 2 non-coagulopathy related haematomas operated on, but also in 2 of the 4 coagulopathy associated haematomas that underwent surgery. The average haematoma thickness was higher in the coagulopathy related haematomas. The mean Glasgow Coma Score on admission was 7.7 and the mortality rate was 55.6% in the coagulopathy related group. In the non-coagulopathy related haematomas the mean Glasgow Coma Score was 12.0 and the mortality rate 33.3%. The latter mortality rate corresponds well to that of a historical group of 'spontaneous acute subdural haematomas of arterial origin' collected from the literature. INTERPRETATION: The outcome was worse in the non-traumatic acute subdural haematomas that were associated with a coagulation deficiency. While in all non-traumatic acute subdural haematomas the interval to surgery should be minimized, early recognition and urgent correction of coagulation deficiencies is certainly indicated.