The diagnostic value of the pulsatility curve to predict shunt responsiveness in patients with idiopathic normal pressure hydrocephalus.

OBJECTIVE: The aim of this study was to investigate the diagnostic accuracy of the pulsatility curve to predict shunt response in patients with idiopathic normal pressure hydrocephalus (iNPH). METHODS: Lumbar cerebrospinal fluid dynamics were derived from an automatic lumbar infusion test (LIT) protocol. All patients were treated with ventriculoperitoneal shunting and re-examined 6 months after shunting. Patient demographics and outcomes were gathered in a prospective, electronic database that spanned from January 2012 to January 2020. A validated iNPH scale was used to assess patients preoperatively and 6 months postoperatively. The relationship of the relative pulse pressure coefficient (RPPC), delta amplitude, successful lowering of amplitude, and the pressure-value at a hypothetical amplitude of zero (P0), resistance to outflow (Rout), and outcome, were assessed using receiver operating characteristic (ROC) curves. RESULTS: We included 38 patients. The RPPC, delta amplitude, successful lowering of amplitude, and P0 parameters did not predict shunt response. Mean P0 was 0.5 (IQR 0.4-0.9) in improved patients and 0.4 (IQR 0-1.2) in non-improved patients. The delta amplitude was 0.16 kPa (IQR 0.10-0.23) in improved patients and 0.18 kPa (IQR 0.11-0.24) in non-improved patients. Furthermore, we found a technical failure rate of pulsatility curve measurements of 32%. CONCLUSION: Pulsatility curve results were not suitable in predicting shunt response in our cohort. The diagnostic value of LIT in case of normal pressure hydrocephalus should be subject to more rigorous research.

Surgical intervention for hydrocephalus in infancy; etiology, age and treatment data in a Dutch cohort.

PURPOSE: To gain insight into the patient characteristics of surgically treated hydrocephalus in the Netherlands, we report the first data from the Dutch Quality Registry NeuroSurgery (QNRS) database for infants with hydrocephalus requiring surgical intervention. METHODS: We used the prospectively gathered database concerning infants ≤ 2 years of age surgically treated for hydrocephalus. We report data from start of registry, concerning etiology, age, and treatment of patients registered. We compared data with the Hydrocephalus Clinical Research Network (HCRN), a multicenter network of pediatric neurosurgical institutions in North America. RESULTS: A total of 359 operated infants was registered in the period from 2010 to 2017. A drop in patients registered was seen in 2015, possibly due to revisions of the database. Most infants were operated on between 1 and 6 months of age. Cause of hydrocephalus was predominantly intracranial hemorrhage, followed by congenital causes. The proportion of infants with aqueduct stenosis and myelomeningocele as cause of hydrocephalus stayed relatively stable during this period of registration. Initial shunting was performed in 40% and reservoir/ETV as initial treatment was done in 60%. In both groups, 50% needed revision surgery. CONCLUSIONS: The first data concerning surgically treated pediatric hydrocephalus from a prospectively collected Dutch register are presented, showing similar results when comparing to the HCRN database.

Endoscopy-assisted craniosynostosis surgery followed by helmet therapy.

BACKGROUND: Surgical methods to treat craniosynostosis have evolved from a simple strip craniectomy to a diverse spectrum of partial or complete cranial vault remodeling with excellent results but often with high comorbidity. Therefore, minimal invasive craniosynostosis surgery has been explored in the last few decades. The main goal of minimal invasive craniosynostosis surgery is to reduce the morbidity and invasiveness of classical surgical procedures, with equal long-term results, both functional as well as cosmetic. METHODS: To reach these goals, we adopted endoscopy-assisted craniosynostosis surgery (EACS) supplemented with helmet molding therapy in 2005. RESULTS: We present in detail our surgical technique used for scaphocephaly, trigonocephaly, plagiocephaly, complex multisutural, and syndromic cases of craniosynostosis. CONCLUSIONS: We conclude that EACS with helmet therapy is a safe and suitable treatment option for any type of craniosynostosis, if performed at an early age, preferably around 3 months of age.

Management and consequences of postoperative fluctuations in plasma sodium concentration after pediatric brain tumor surgery in the sellar region: a national cohort analysis.

PURPOSE: Severe fluctuations in plasma sodium concentration and plasma osmolarity, including central diabetes insipidus (CDI), may have significant influence on postoperative morbidity and mortality after pediatric brain tumor surgery.The aim of this study was to describe the frequency, severity and neurological consequences of these fluctuations in pediatric brain tumor survivors. METHODS: A retrospective, multi-institutional chart review was conducted among all children who underwent brain tumor surgery in the sellar or suprasellar region in seven university hospitals in the Netherlands between January 2004 and December 2013. RESULTS: Postoperative CDI was observed in 67.5% of 120 included children. Fluctuations of plasma sodium concentration ≥ 10 mmol/L/24 h during the first ten postoperative days were seen in 75.3% of patients with CDI, with a maximum delta of 46 mmol/L/24 h. When compared to patients without CDI, altered mental status occurred more frequently in patients with postoperative CDI (5.1 vs. 23.5% respectively, p = 0.009). Low plasma sodium concentration was related to altered mental status and the occurrence of seizures. Frequency and severity of fluctuations in plasma sodium concentration during the first ten postoperative days were significantly higher in patients with permanent CDI at last follow-up than in patients with transient CDI or without CDI (p = 0.007). CONCLUSION: Postoperative CDI is a common complication after pediatric brain tumor surgery in the sellar or suprasellar region. Extreme plasma sodium concentrations and large intra-day fluctuations still occur and seem to influence the postoperative neurological course. These results illustrate the need for intensive monitoring in a highly experienced center.

Spontaneous remission of acromegaly and Cushing's disease following pituitary apoplexy: Two case reports.

In this double case report, we present two special cases of pituitary apoplexy. First, we describe a patient with growth hormone deficiency despite clinical suspicion of acromegaly. Imaging showed evidence of a recent pituitary apoplexy, which might have caused spontaneous remission of the acromegaly before presentation at our outpatient clinic. Second, we describe a patient who presented with spontaneous remission of Cushing's disease after pituitary apoplexy, followed by a spontaneous remission of a relapse of the Cushing's disease due to a second pituitary apoplexy. These cases show that patients in spontaneous remission of hormonally active pituitary adenomas should be suspected of a pituitary apoplexy. Furthermore, even after spontaneous remission following pituitary apoplexy, careful long-term follow-up of these patients is mandatory, as relapses of hormonal hypersecretion can occur.

Endoscopic transsphenoidal pituitary surgery: a good and safe primary treatment option for Cushing's disease, even in case of macroadenomas or invasive adenomas.

CONTEXT: Although the endoscopic technique of transsphenoidal pituitary surgery (TS) has been widely adopted, reports on its results in Cushing's disease (CD) are still scarce and no studies have investigated long-term recurrence rates. This is the largest endoscopic series published till now. OBJECTIVE: To gain insight into the role of endoscopic TS as a primary treatment option for CD, especially in patients with magnetic resonance imaging (MRI)-negative CD and (invasive) macroadenomas. DESIGN: Retrospective cohort study. PATIENTS AND METHODS: The medical records of 86 patients with CD who underwent endoscopic TS were examined. Data on preoperative and postoperative evaluation, perioperative complications, and follow-up were collected. Remission was defined as disappearance of clinical symptoms with a fasting plasma cortisol level ≤ 50 nmol/l either basal or after 1 mg dexamethasone. RESULTS: The remission rate in different adenoma subclasses varied significantly: 60% in MRI-negative CD (n=20), 83% in microadenomas (n=35), 94% in noninvasive macroadenomas (n=16), and 40% in macroadenomas that invaded the cavernous sinus (n=15). The recurrence rate was 16% after 71 ± 39 months of follow-up (mean ± S.D., range 10-165 months). CONCLUSIONS: Endoscopic TS is a safe and effective treatment for all patients with CD. Recurrence rates after endoscopic TS are comparable with those reported for microscopic TS. Our data suggest that in patients with noninvasive and invasive macroadenomas, the endoscopic technique of TS should be the treatment of choice as remission rates seem to be higher than those reported for microscopic TS, although no comparative study has been performed.

Acquired progressive hypotonia in infancy: consider compressive cervical myelopathy.

UNLABELLED: This case report presents a rare cause of progressive hypotonia due to a congenital bony defect of the atlas in a 2-month-old girl. The patient was initially referred to the paediatric department with feeding problems. Within days after admission she developed progressive hypotonia and showed decreased reflexes of the upper extremities. Magnetic resonance imaging showed compressive myelopathy at the level of vertebra C1. After laminectomy of the C1-vertebra the neurological symptoms resolved. CONCLUSION: Most anomalies of the vertebras are asymptomatic. Only symptomatic anomalies with compression and neurological symptoms need surgery.

The combined supraorbital keyhole-endoscopic endonasal transsphenoidal approach to sellar, perisellar and frontal skull base tumors: surgical technique.

INTRODUCTION: Extended endoscopic endonasal transsphenoidal approaches (extended EETA) are increasingly being explored for lesions around the sella and the frontal skull base. These approaches, however, require significant surgical expertise and training that can only be obtained in high-volume centers and therefore these approaches are not generalizable to the whole neurosurgical community. Also, these approaches require significant skull base destruction and reconstruction, which comes with a high risk of CSF fistulas. The aim of this article is to describe a combined supraorbital keyhole-endoscopic endonasal transsphenoidal approach as an alternative surgical strategy to the extended EETA that is easier to perform and that leaves the skull base anatomy more intact. TECHNIQUE: Two fairly common neurosurgical approaches, the supraorbital keyhole approach and the endoscopic endonasal transsphenoidal approach, are combined into a single-stage or two-stage surgical procedure. The procedure can be performed as a single neurosurgeon-serial approach and as a two neurosurgeon-parallel simultaneous approach. The philosophy and technique of this combined approach will be described. CONCLUSION: The combined supraorbital keyhole-EETA approach can be used without extra surgical training or expertise and with preservation of skull base anatomy for sellar, perisellar and frontal skull base tumors.

Repeated transsphenoidal pituitary surgery (TS) via the endoscopic technique: a good therapeutic option for recurrent or persistent Cushing's disease (CD).

BACKGROUND: No data on results of repeated transsphenoidal surgery via the endoscopic technique for patients with persistent or recurrent Cushing's disease are available. DESIGN AND PATIENTS: We retrospectively evaluated the remission rates and complications of repeated transsphenoidal surgery via the endoscopic technique in 14 patients with persistent (N = 6) or recurrent (N = 8) Cushing's disease treated in our centre between 1999 and 2007. MAIN OUTCOMES: Remission was defined as the disappearance of symptoms of hypercortisolism with basal plasma cortisol level < or = 50 nmol/l 24-48 h after glucocorticoid withdrawal and/or suppression of plasma cortisol level < or = 50 nmol/l after 1 mg dexamethasone overnight within the first 3 months after transsphenoidal surgery. RESULTS: With repeated endoscopic transsphenoidal surgery a remission rate of 10/14 (71%) was achieved. No patient had a relapse during a median follow-up of 24 months. Cerebrospinal fluid leakage was the most frequent complication (6 patients) and 11 patients required hormonal substitution after surgery. The success of repeated transsphenoidal surgery could not be predicted by visualization of an adenoma on MRI before first or second surgery, histopathological confirmation of an ACTH secreting adenoma after first or second surgery, treatment with cortisol lowering agents before first or second surgery, the operation technique used during the first surgery, persistent vs. recurrent disease after the first surgery, age, gender and interval between the two surgeries. CONCLUSION: Repeated transsphenoidal surgery via the endoscopic technique is a good treatment option for selected patients with recurrent or persistent Cushing's disease following primary pituitary surgery.

Hemophilia a and spinal epidural hematoma in children.

Hemophilia A is an X-linked bleeding disorder, caused by deficient or defective blood coagulation factor VIII. The most characteristic symptoms of the severe forms of hemophilia A are joint and muscle bleeds. Intracranial hemorrhage occurs only in 3-10% of the patients. Spinal epidural hematomas are rarely seen. We describe a 13-month-old boy with hemophilia A who was admitted to the hospital because of irritability and unspecified pain for the past two days. There was no history of evident trauma, no fever. Physical investigation showed no skin lesions or hematomas and no obvious cause for the pain. Neurological examination showed a dysphoric toddler, mainly in the fetal position. No neurological abnormalities were found except for a miosis of the right pupil due to a suspected Horner syndrome. Magnetic resonance imaging of the spine showed an extensive epidural hematoma. The boy was successfully treated with intensive replacement therapy during three weeks and did not require surgical intervention. There was a rapid and complete clinical resolution. In conclusion, rare hematomas should be considered and searched for in children with bleeding disorders and not well understood complaints. Early diagnosis is important for the neurological outcome.

Giant cavernous hemangiomas: report of three cases.

Giant cavernous hemangiomas occur very rarely, and little has been reported about their behavior. In this case report three cavernous hemangiomas with a diametric measure between 6 cm and 7 cm and distinct features will be described. A 36-year-old female patient presented with headache and nausea. A CT scan disclosed a large circumscribed tumor with strong contrast enhancement in the temporo-parieto-occipital region of the right cerebral hemisphere and extension into the right cerebellar hemisphere. A 35-year-old woman was admitted to our emergency ward with a generalized seizure and a dilated pupil. The CT scan showed an extensive left frontal lesion containing a substantial hyperintense part, suspicious for hemorrhage. A 3-year-old girl was admitted with generalized seizure and progressively declining consciousness. A large left frontotemporal paraventricular multi-cystic lesion was encountered on the CT scan. All three patients were operated on. Two recovered very well. In the case of the 3-year-old girl stable disease was reached. Giant cavernomas do not differ from average-sized cavernomas in clinical, surgical or histopathological presentation but may differ radiologically. However, the possible diagnosis of cavernoma can be overlooked, due to their size and possible differential diagnosis.

Transsphenoidal pituitary surgery via the endoscopic technique: results in 35 consecutive patients with Cushing's disease.

OBJECTIVE AND DESIGN: The endoscopic technique has been recently introduced in the field of transsphenoidal pituitary surgery. This technique allows inspection of sellar, supra- and parasellar structures and removal of the tumor under direct visualization, is minimally traumatic and permits easier reoperations. This is the first report on the results of endoscopic surgery for patients with Cushing's disease. Our aim was to retrospectively analyze the results of pituitary surgery in 35 consecutive patients with Cushing's disease operated in our hospital after the introduction of the endoscopic technique (1998-2004). METHODS: Remission was defined as suppression of plasma cortisol (< or =50 nmol/L) after 1 mg dexamethasone overnight determined in the first 3 months after surgery and disappearance of clinical signs and symptoms of hypercortisolism. The patients were followed for an average of 27 months (range 4 to 81 months, median 20 months). RESULTS: Pituitary MRI showed a macroadenoma in 6 patients, a microadenoma in 17 patients and no adenoma in 12 patients. After the initial surgery 27 patients (77%) were in remission. None of the patients had a relapse during follow-up. In the remaining 8 patients hypercortisolemia persisted after surgery. Three of them had a second endoscopic pituitary surgery resulting in remission in two patients. In one patient a second endoscopic pituitary surgery will soon follow. The remaining four patients were treated with radiotherapy postoperatively. Two of them were at the time of data collection in remission. One patient from the remission group had a serious epistaxis and three patients had cerebrospinal fluid leakage, one requiring an external lumbar drain, shortly after surgery. No complications were recorded in the failure group. Postoperatively 34% of all patients required substitution with levothyroxine, 40% required substitution with glucocorticoids, 17% received estrogens or testosterone and 6% still required desmopressin. CONCLUSIONS: Endoscopic transsphenoidal pituitary surgery resulted in our series of patients with Cushings disease in an excellent postoperative remission rate. A randomized clinical trial, comparing endoscopic and conventional pituitary surgery in patients with Cushings disease, is needed to determine the pros and cons of both techniques.

Cerebrospinal fluid leakage as complication of treatment with cabergoline for macroprolactinomas.

Treatment of patients with prolactinomas consists primarily of dopamine agonists (DA). Cerebrospinal fluid (CSF) leakage has sporadically been reported in patients with macroprolactinomas treated with short-acting DA such as bromocriptine. Little is known on the incidence of this complication in patients treated with the long-acting D2 specific DA cabergoline. We report three patients with CSF leakage shortly after initiation of cabergoline treatment for macroprolactinoma. All three patients responded rapidly to cabergoline (CAB) by shrinkage of the tumor and release of the optic chiasm compression. The CSF leakage occurred within 10 days after initiation of treatment. CAB treatment was not discontinued. In one patient the CSF leakage ceased spontaneously, with no additional therapy. The second patient had a surgical repair of the CSF fistula, permitting cabergoline to be continued without a recurrence of the CSF leakage. The third patient refused surgical repair of the sellar defect. In this patient the cabergoline dosage was temporarily decreased with no effect on the CSF leakage. Four years later, the CSF leakage is unchanged in this patient, whilst no other complications occurred during the follow-up. No infectious complications occurred in these three patients. In conclusion, patients with large, invasive macroprolactinomas are at risk of CSF leakage during medical treatment with CAB. It is advisable to warn these patients for occurrence of this complication and to monitor them closely especially during the first months of treatment.

Dumbbell trigeminal schwannoma in a child: complete removal by a one-stage pterional surgical approach.

OBJECTIVE: The objective was to describe a rare case of a trigeminal schwannoma in a child and the surgical procedure performed for therapy. PATIENT AND METHODS: A 6-year-old girl presented with tiredness, dysarthric speech and cerebellar symptoms. Imaging studies revealed a unilateral dumbbell-shaped tumour, extending into both the middle and posterior fossa, centred over Meckel's cave. One-stage surgery was performed by pterional craniotomy. The tumour was first debulked in the middle fossa, then peeled from the wall of the cavernous sinus, followed by extirpation of the tumour from the posterior fossa. The tumour extended to the caudal cranial nerves and was completely removed. Trigeminal fascicles were distributed throughout the tumour. Histopathological examination revealed a schwannoma. CONCLUSION: Trigeminal schwannoma is a tumour that occurs rarely in childhood. Although several, often multistaged surgical strategies have been reported in the literature, this tumour was eradicated by a one-stage pterional approach.

Massive reduction of tumour load and normalisation of hyperprolactinaemia after high dose cabergoline in metastasised prolactinoma causing thoracic syringomyelia.

In 1970 a 20 year old woman presented with a pituitary chromophobe adenoma for which she underwent transfrontal pituitary surgery. In 1978 she had to be reoperated on because of local tumour recurrence, resulting in hypopituitarism. Bromocriptine (5 mg/day) was given for 15 years, but the plasma prolactin levels remained elevated. In 2000 the patient presented with signs and symptoms suggestive of a spinal cord lesion at the mid-thoracic level. A magnetic resonance imaging (MRI) scan showed an extensive leptomeningeal mass extending from the brainstem to L5, with a thoracic syringomyelia at the T7-T8 level. The plasma prolactin level was very high (5114 microg/l). A biopsy showed the presence of a metastasised prolactinoma. On administration of high dose cabergoline, 0.5 mg twice a day orally, the plasma prolactin levels decreased within one month and then normalised within 26 months. Tumour load reduced considerably but unfortunately, her signs and symptoms did not improve. This case illustrates that a high dose dopamine agonist might be an important therapeutic option in patients with a metastasised prolactinoma.

The use of a head-mounted display for visualization in neuroendoscopy.

OBJECTIVE: To evaluate the clinical use of a head-mounted display (HMD) for visualization in all neuroendoscopic procedures. MATERIALS AND METHODS: We retrospectively evaluated all endoscopic and endoscope-controlled procedures in which the HMD was used in our department between July 1999 and June 2002. RESULTS: A total of 269 endoscopic procedures were performed. In 147 cases intraventricular endoscopic procedures were carried out, mostly third ventriculocisternostomies, for which a fiberscope was used exclusively. Thirty intracranial cysts were fenestrated or removed (colloid cysts) with the help of various endoscopes. A total of 87 endoscopic transsphenoidal surgeries were performed with a lensscope. In only one case was it necessary to abandon use of the HMD due to inferior visualization; in all other cases visualization by the HMD was thought to be sufficient. Fatigue of the surgeon due to wearing the helmet did not occur. All surgeons had the impression that visual strain was decreased in comparison to looking at a monitor from a distance. The working position was considered to be more comfortable when wearing the HMD, and eye-hand coordination was improved. No technical problems occurred with the system. CONCLUSIONS: The HMD is a new visualization tool in neurosurgery that may improve the ergonomics of neuroendoscopic and endoscope-controlled procedures.

The influence of surgical experience on the rate of intraoperative aneurysm rupture and its impact on aneurysm treatment outcome.

BACKGROUND: The influence of surgical experience on the result of aneurysm surgery remains unclear. To determine the impact of surgical experience we considered the occurrence of intraoperative aneurysm rupture (IAR) during microneurosurgery for intracranial aneurysms as an objective factor that could be evaluated. METHODS: A retrospective study was performed on 379 consecutive patients with 490 cerebral aneurysms operated upon from 1989 to 1995. RESULTS: IAR occurred in 6.7% of aneurysms and 8.7% of patients. There was a direct inverse relationship between the annual caseload of the surgeon and the risk of IAR. New neurological deficits (NND) occurred in 21% of patients with IAR, which accounts for 1.8% of NND in all patients with aneurysms. CONCLUSION: Although there seems to be a direct relationship between surgical experience and the risk of IAR, the impact on the overall treatment outcome of cerebral aneurysms is rather limited.

Combined endovascular-microsurgical treatment of tentorial-incisural dural arteriovenous malformations. Report of five cases.

Dural arteriovenous malformations of the tentorial edge are rare lesions with an unfavorable clinical course if left untreated. As yet no optimum treatment has been established. We retrospectively evaluated the results of our therapy concept, which consists of endovascular embolization followed by microsurgical obliteration, and compared the results to the relevant literature in order to clarify which therapeutic regimen seems to be optimum at the present time. In all of our five patients the lesion was completely obliterated, as proven by angiography, with favorable clinical results and low morbidity. Several other clinical series confirm our results, so we conclude that combined endovascular therapy and microsurgery is the optimum treatment for these lesions.

Delayed ischemic optic neuropathy after surgery on skull base meningiomas successfully treated with nimodipine and rheological therapy: report of two cases.

Two patients operated on because of skull base meningiomas experienced delayed ischemic optic neuropathy with loss of vision in one eye on days 6 and 12 after surgery. Treatment with nimodipine and rheological therapy was effective in restoring visual acuity. Possible pathophysiological mechanisms and treatment options are discussed.

Endoscopic treatment of mesencephalic ependymal cysts: technical case report.

OBJECTIVE: To evaluate the results of different neuroendoscopic surgical procedures for the treatment of mesencephalic ependymal cysts. METHODS: The clinical records of five patients treated for symptomatic mesencephalic ependymal cysts were retrospectively studied. Two patients had been operated on using an endoscope-assisted microsurgical technique and three patients using a pure endoscopic procedure. RESULTS: Adequate fenestration of the cysts was achieved in all of the patients. Two of the patients were symptom-free, and the other three had improved significantly after a mean follow-up duration of 14 months. There was no surgical morbidity. CONCLUSION: Mesencephalic ependymal cysts can be treated adequately, resulting in a low morbidity rate, using neuroendoscopy or endoscope-assisted microsurgery. This specific surgical technique is presented.