Interobserver variability in the classification of childhood maculopapular cutaneous mastocytosis.

BACKGROUND: Maculopapular cutaneous mastocytosis (MPCM) in children is classified in two variants: (i) monomorphic variant, presenting with the small macules or papules typically seen in adult patients; and (ii) polymorphic variant with larger lesions of variable size and shape, typically seen in children. The definition of polymorphic and monomorphic variants is mostly intuitive, and a validation of this classification has not been done. OBJECTIVE: To study interobserver variability in the classification of MPCM in two groups of observers: mastocytosis experts and general dermatologists. MATERIALS AND METHODS: Nineteen cases of childhood MPCM were shown blindly, for classification as monomorphic or polymorphic type, to 10 independent observers (eight dermatologists, one allergist and one haematologist) from Europe and North America with a vast experience in the management of paediatric mastocytosis. Also, the same cases were shown on a screen to 129 general dermatologists attending a meeting; their votes were registered by remote controls. The interobserver variability kappa coefficient (with 95% confidence interval) was calculated to measure the reliability of the correlation. RESULTS: The value of kappa interobserver variability coefficient for the group of 10 experts (95% confidence interval) was 0.39 (0.18-0.63), which is considered as 'fair'. The value of kappa interobserver variability coefficient for the group of 129 general dermatologists (95% confidence interval) was 0.17 (0.06-0.39), which is considered as 'slight'. A complete agreement of all 10 experts was achieved in only four of 19 cases (21.1%) The most voted choice was concordant between the two groups in only 11 of the 19 cases. CONCLUSIONS: We failed to validate the classification system of childhood MPCM in monomorphic and polymorphic types. While the rate of agreement was low for mastocytosis experts, it was nearly the agreement expected by chance in general dermatologists.

Cytoreductive nephrectomy in metastatic renal cell carcinoma: outcome of patients treated with a multidisciplinary, algorithm-driven approach.

PURPOSE: Metastatic renal cell carcinoma (mRCC) represents a significant and rising burden of disease, with rapidly evolving treatment modalities. The role of cytoreductive nephrectomy (CN) is controversial in this setting. As such, London Cancer has pursued a multidisciplinary team (MDT) approach when assessing suitability for surgery. METHODS: A retrospective analysis of treatment-naive synchronous mRCC patients, managed via a renal-specialist MDT, was conducted between January 2015 and December 2018. An MDT selection algorithm for CN-using the International Metastatic Renal Cell Carcinoma Database Consortium score (IMDC), performance status and metastatic disease burden-was developed. RESULTS: 87 treatment-naive synchronous mRCC patients received either CN (n = 18), Systemic therapy (ST) alone (n = 43) or Best supportive care (BSC) (n = 26). Progression free survival (PFS) and overall survival (OS) were assessed. 51% and 39% were IMDC intermediate and poor risk. Median PFS was 28.6 months and 4.5 months in the CN group and ST alone group, respectively, Hazard Ratio for death was 3.63 [(95% CI 1.68-7.83) p < 0.05]. OS remains immature for the CN group, but a median OS of 12.8 months was observed in the ST group and 5.0 months for BSC. 1-year OS rate for CN, ST and BSC groups was 77.8%, 55.8% and 23.10%, respectively. CONCLUSION: These findings describe outcomes of an unselected series of patients treated via an MDT-driven, protocolised treatment pathway. MDT pathway-based decision making may improve patient selection for CN. Further research is needed to evaluate the role of CN amongst a growing landscape of treatment strategies, including immune checkpoint inhibitors and combination therapies. Multi-disciplinary team, pathway-based treatment strategy may improve patient selection for cytoreductive nephrectomy in patients with metastatic renal cell carcinoma.

Pre- and postoperative fMRI and clinical memory performance in temporal lobe epilepsy.

OBJECTIVE: The present study aimed to examine and compare memory processing in patients with unilateral temporal lobe epilepsy (TLE) before and after surgery using functional magnetic resonance imaging (fMRI). METHODS: Seventeen preoperative patients with unilateral TLE (nine left, eight right) and eight healthy controls were recruited. They performed a complex visual scene-encoding task during fMRI to measure memory activation in the mesial temporal lobe. Their memory performance was evaluated using standardised neuropsychological tests. After unilateral temporal lobe resection (either temporal lobectomy, selective amygdalohippocampectomy or lesionectomy), the same fMRI paradigm and neuropsychological tests were administered to the patient group. RESULTS: Left-TLE patients demonstrated a decline in verbal memory after left temporal lobe resection. Their postoperative verbal and visual memory performance was positively associated with postoperative functional activation in the right mesial temporal lobe, whereas the postoperative memory performance of right-TLE patients was positively associated with postoperative functional activation in the left mesial temporal lobe, contralateral to their respective side of resection. CONCLUSION: Postoperative memory performance was significantly associated with functional activation contralateral to the side of resection in patients with unilateral TLE, and the function of the contralateral mesial temporal lobe might play an important role in supporting memory performance after temporal lobe resection.

The surgical treatment of intracranial aneurysms based on computer tomographic angiography alone--streamlining the acute mananagement of symptomatic aneurysms.

BACKGROUND: We aimed to prospectively assess the usefulness of computer tomographic angiography (CTA) in streamlining the management of symptomatic intracranial aneurysms in a tertiary neurosurgical unit, from admission to surgery. METHODS: We performed a prospective evaluation over a 2-year period of all symptomatic intracranial aneurysms managed according to a standardized departmental protocol, to assess how CTA has impacted the decision-making process pertaining to the suitability of this investigation to proceed directly to surgery. FINDINGS: A total of 90 patients with intracranial aneurysms were treated over the 2-year period. 23 (26%) underwent endovascular occlusion while 67 (74%) patients underwent a surgical clipping procedure. In the surgical group, 22 (33%) patients had their aneurysms clipped based on CTA alone, while 45 (67%) required additional conventional angiography prior to surgery. Thus around one quarter of all patients treated for symptomatic intracranial aneurysms in our unit had their aneurysm secured surgically based solely on CTA. INTERPRETATION: CTA significantly influences the acute management of symptomatic intracranial aneurysms by streamlining the decision-making process during the early and acute management of these lesions.

Role of magnetic resonance imaging for preoperative evaluation of patients with refractory epilepsy.

OBJECTIVE: To investigate the magnetic resonance imaging characteristics of patients with refractory epilepsy and the relationship to progression to surgery. DESIGN: Prospective observational study. SETTING: University teaching hospital, Hong Kong. PATIENTS: Patients undergoing preoperative evaluation for epilepsy surgery. MAIN OUTCOME MEASURE: Cranial magnetic resonance imaging findings, correlation with electroencephalographic results, and percentage of patients who were considered suitable candidates for surgery. RESULTS: Structural abnormalities associated with refractory epilepsy in 100 consecutive patients were mesial temporal sclerosis (30%), neocortical sclerosis (23%), vascular malformation (7%), neuronal migration disorders (7%), and tumours (5%). Normal brain scans were found for 28% of patients. Fourteen of 30 (46%) patients with medial temporal lobe lesions at magnetic resonance imaging were suitable candidates for surgery compared with 8/42 (19%) patients with extrahippocampal lesions (odds ratio=3.7; 95% confidence interval, 1.3-10.6; P<0.012). CONCLUSION: Mesial temporal sclerosis was the most common pathology in patients with refractory epilepsy. At the Prince of Wales Hospital, for patients who have undergone a basic magnetic resonance imaging protocol and surface electroencephalography, the result of cranial magnetic resonance imaging is an important determinant for whether patients will undergo surgery.

The use of stereotactic navigation guidance in minimally invasive transnasal nasopharyngectomy: a comparison with the conventional open transfacial approach.

The purpose of this paper is to study the efficacy of applying stereotactic navigation guidance to nasopharyngectomy via a minimally invasive transnasal approach as compared with the conventional open transfacial approaches. The nasopharynx is the centre of the anterior skull base, which is remote from the surface of the facial skeleton. It is well known that there are several surgical approaches for access to resect tumours from the nasopharynx. However, the open techniques have been associated with much morbidity and only provide access to, and identification of, the ipsilateral internal carotid artery that forms the lateral boundary and resection limit of the nasopharynx. The coupling of stereotactic navigation guidance and a minimally invasive transnasal approach for nasopharyngectomy allows the surgeon to identify and protect the internal carotid artery bilaterally at the nasopharynx. This technique reduces operating time and morbidity to a minimum and yet is oncologically sound for resecting nasopharyngeal lesions. We compare 15 patients who underwent the stereotactic navigation guidance approach with 20 patients who received a conventional open transfacial approach.

Subcranial anterior skull base dural repair with galeal frontalis flap.

The pericranial flap has been widely used to repair surgically created anterior skull base defects during craniofacial resection. This report describes a technique of subcranial dural repair with a galeal frontalis flap after resection of an ethmoidal adenoid cystic carcinoma.

Predicting delayed ischemic deficits after aneurysmal subarachnoid hemorrhage using a transient hyperemic response test of cerebral autoregulation.

OBJECTIVE: To assess whether the development of delayed ischemic deficits (DIDs) after aneurysmal subarachnoid hemorrhage can be predicted using transcranial Doppler ultrasonography and the transient hyperemic response test (THRT). METHODS: An increase in the middle cerebral artery peak flow velocity (FV) of more than 9% of baseline values after 5 to 9 seconds of carotid artery compression was defined as a normal THRT result, indicating good autoregulatory reserve. The transcranial Doppler criteria for vasospasm were a FV of more than 120 cm/s and a Lindegaard ratio of more than 3. Twenty patients with no immediate postoperative neurological deficits were studied. The FVs at all of the major cerebral arteries were measured daily after surgery, and the THRT results were assessed bilaterally. RESULTS: Five of six patients with abnormal THRT results in the first examination after surgery (primary THRT impairment) developed DIDs; none of the remaining patients developed DIDs (Fisher exact test, P = 0.0004). All five patients with DIDs initially exhibited low FVs but all subsequently developed increases in FVs to values of more than 150 cm/s and four exhibited FVs of more than 200 cm/s. The time of onset of DIDs corresponded to the time of onset of moderate vasospasm (FV > 150 cm/s). None of the patients with initially normal THRT results developed DIDs, although four patients did exhibit late (secondary) THRT impairment, which was associated with FVs of more than 120 cm/s. CONCLUSION: When the effects of primarily impaired (after surgery) autoregulation are magnified by vasospasm, the risk of DIDs seems to be very high. Vasospasm alone does not seem to cause DIDs. The development of DIDs could therefore be predicted using the THRT for patients after aneurysm clipping.

Prediction of cerebral ischaemia during carotid endarterectomy with preoperative CO2-reactivity studies and angiography.

The objective of this study was to assess the value of combining the preoperative CO2 cerebrovascular reactivity index (CO2RI) with carotid and cerebral angiography in predicting the risk of severe cerebral ischaemia (SCI) during carotid endarterectomy (CEA). Seventy-four consecutive patients scheduled for CEA underwent preoperative digital subtraction angiography and CO2-reactivity tests. During CEA, cerebral function monitor (CFM) was used to document cortical electrical activity, whilst transcranial Doppler measured the middle cerebral artery flow velocity (FV). A persistent fall in CFM voltage and/or a fall in FV > or = 60% on internal carotid artery (ICA) clamping were used as criteria for defining SCI. Complete data from 59 patients were obtained for final analysis. Twelve cases showed a fall in FV > or = 60%; 11 of these also showed a sustained fall in CFM voltage. Using logistic regression, the risk of SCI was found to be negatively associated with (1) contralateral CO2RI, (2) the percentage stenosis of the contralateral ICA, and (3) the difference between ipsilateral and contralateral CO2RI. Using these factors, a logistic regression model for predicting the risk of SCI was established which provided a sensitivity of 75% and specificity of 100%. The risk of SCI during CEA was related to the contralateral ICA stenosis and the CO2RI of both cerebral hemispheres. This information may assist in presurgical planning and help to select asymptomatic carotid lesions for surgery.

The role of computed tomographic angiography in the diagnosis of intracranial aneurysms and emergent aneurysm clipping.

Conventional cerebral angiography has always been regarded as the gold standard for intracranial aneurysm detection. However, conventional angiography has the disadvantages of being invasive and time consuming. We present here 30 patients who underwent computed tomographic angiography (CTA) with three-dimensional reconstruction for the detection of intracranial aneurysms. All of these patients had subarachnoid hemorrhage or suspected intracranial aneurysms. CTA was performed in all patients with the use of a General Electric Hispeed Advantage helical scanner. Iohexol, 135 ml, was used as the contrast agent. Twenty-five patients also underwent conventional angiography for comparison. The five patients who underwent CTA only did not have conventional angiography because of poor clinical condition, and four of them subsequently died. Five patients had subarachnoid hemorrhage, but the results of both CTA and conventional angiography were negative for aneurysms. One patient had an incidental finding of a 3-mm left posterior communicating artery aneurysm on CTA, which was confirmed by conventional angiography. In the remaining 19 patients, 19 saccular aneurysms and 1 fusiform aneurysm were detected by CTA. Locations and sizes were confirmed by conventional angiography in all except two. The first exception was a patient who had a 2.5-mm anterior communicating artery aneurysm detected by CTA but not by conventional angiography. Surgical exploration confirmed the CTA diagnosis. The other exception was a patient in whom a 2-mm right posterior communicating artery aneurysm was detected by CTA but in whom conventional angiography showed a 2-mm left posterior communicating artery aneurysm. Unfortunately, there was no surgical confirmation in this case because the family of the patient refused surgery. Our results have demonstrated that CTA is a quick, reliable, and relatively simple diagnostic tool for intracranial aneurysms. In an emergent situation, such as a deteriorating patient with a hematoma, it is superior to either empiric exploration or infusion computed tomographic scans because it delineates the orientation and configuration of the aneurysm and its associated vascular anatomy.

An anterior intradural neurenteric cyst of the cervical spine: complete excision through central corpectomy approach--case report.

The case of a cervical intraspinal neurenteric cyst in a 6-year-old boy is reported. The anterior intradural location of the cyst was confirmed by preoperative magnetic resonance imaging. Complete excision of the cyst was achieved through the anterior central corpectomy approach. The vertebral defect was reconstructed with autogenous fibular graft. The child made a complete neurological recovery without a mechanical problem either at the neck or at the graft donor site.