TROPHY registry - status report.

INTRODUCTION: The TROPHY registry has been established to conduct an international multicenter prospective data collection on the surgical management of neonatal intraventricular hemorrhage (IVH)-related hydrocephalus to possibly contribute to future guidelines. The registry allows comparing the techniques established to treat hydrocephalus, such as external ventricular drainage (EVD), ventricular access device (VAD), ventricular subgaleal shunt (VSGS), and neuroendoscopic lavage (NEL). This first status report of the registry presents the results of the standard of care survey of participating centers assessed upon online registration. METHODS: On the standard of treatment forms, each center indicated the institutional protocol of interventions performed for neonatal post-hemorrhagic hydrocephalus (nPHH) for a time period of 2 years (Y1 and Y2) before starting the active participation in the registry. In addition, the amount of patients enrolled so far and allocated to a treatment approach are reported. RESULTS: According to the standard of treatment forms completed by 56 registered centers, fewer EVDs (Y1 55% Y2 46%) were used while more centers have implemented NEL (Y1 39%; Y2 52%) to treat nPHH. VAD (Y1 66%; Y2 66%) and VSGS (Y1 42%; Y2 41%) were used at a consistent rate during the 2 years. The majority of the centers used at least two different techniques to treat nPHH (43%), while 27% used only one technique, 21% used three, and 7% used even four different techniques. Patient data of 110 infants treated surgically between 9/2018 and 2/2021 (13% EVD, 15% VAD, 30% VSGS, and 43% NEL) were contributed by 29 centers. CONCLUSIONS: Our results emphasize the varying strategies used for the treatment of nPHH. The international TROPHY registry has entered into a phase of growing patient recruitment. Further evaluation will be performed and published according to the registry protocol.

Volume change of cranial arachnoid cysts after successful endoscopic fenestration in symptomatic children.

INTRODUCTION: Endoscopic fenestration remains a first-line treatment option for symptomatic arachnoid cysts. After fenestration, the cyst does not collapse but reaches an equilibrium state. The aim of this study was to evaluate the change in cyst volume following successful fenestration and symptomatic improvement. METHODS: Cyst volume was measured on serial MR scans of 4 children (1 female, 3 males) with symptomatic arachnoid cysts (middle fossa n = 2, choroidal fissure n = 1 and posterior fossa n = 1), who experienced symptom resolution after endoscopic fenestration. Average follow-up was 20.5 months (range 3-48). RESULTS: Significant cyst volume reduction was seen in all four patients. In patient 1, preoperative cyst volume was 336 cm3 and decreased to 194 cm3 at 7 months (42% reduction). In patient 2, preoperative volume was 12.64 cm3 and reduced to 1.51 cm3 at 3 months (88% reduction). In patient 3, preoperative volume was 105 cm3 and reduced to 72 cm3 in 2 months (30% reduction). In patient 4, preoperative volume was 125 cm3 and reduced to 54 cm3 at 7 months (56% reduction). All remained stable after 7 months and there has been no late increase in volume. CONCLUSIONS: Significant reduction in arachnoid cyst volume at the order of 30-40% is seen after successful endoscopic fenestration. The cyst volume appears to decrease gradually in the first 3-7 months and reaches a plateau after that. Complete resolution of symptoms in the presence of residual volume may indicate that cyst volume below a threshold may not correlate directly with clinical status.

Large balloon dilation vs. mechanical lithotripsy for the management of large bile duct stones: a prospective randomized study.

OBJECTIVES: The removal of large bile duct stones (>12 mm) after endoscopic sphincterotomy (EST) remains a challenging issue in therapeutic endoscopy. The aim of this prospective, randomized, controlled trial was to compare the effectiveness and complications of EST followed by large balloon dilation (LBD) with that of EST followed by mechanical lithotripsy (ML) for the management of large bile duct stones. METHODS: A total of 90 patients with large bile duct stones (12-20 mm) were randomized to EST followed by LBD (n=45) or EST followed by ML (n=45). Success rate was determined with a final cholangiogram, whereas type and rate of post-procedure complications were assessed prospectively. RESULTS: Complete bile duct stone removal was accomplished in 97.7% of patients subjected to EST-LBD as compared with 91.1% of those subjected to EST-ML (P=0.36). Post-procedure complications were observed in two (4.4%) patients subjected to EST-LBD and in nine (20%) patients subjected to EST-ML (P=0.049). Rates of pancreatitis were similar between the two groups (one case in each), as was post-endoscopic retrograde cholangio pancreatography (ERCP) hemorrhage (one case in each group). None of the patients subjected to EST-LBD developed cholangitis, while this was seen in six patients subjected to EST-ML (0.0 vs. 13.3%, P=0.026). One patient subjected to EST-ML developed perforation, which was successfully managed conservatively. None of our patients with complications died. CONCLUSIONS: EST followed by LBD is equally effective as EST followed by ML for the removal of large bile duct stones, although it is associated with fewer complications.

Malfunction of SynchroMed II baclofen pump delivers a near-lethal baclofen overdose.

INTRODUCTION: Intrathecal baclofen therapy using implantable pumps is an established treatment for spasticity. The pumps occasionally experience serious malfunction. CASE REPORT: A 12-year-old girl suffering from spastic diplegia was implanted with a Medtronic SynchroMed II pump (Medtronic Inc., Minneapolis, Minn., USA). During a refill at 3 months 19 ml of baclofen were still in the pump. It was assumed that there was a lumbar catheter obstruction and a revision was performed. At 11 months she was receiving 180 microg/day. When she presented for refill, there were again 19 ml of baclofen in the reservoir. The pump was refilled, stopped and restarted at a lower dose. Ten minutes after restart the patient was complaining that she could not move her legs. Within the next 50 min she lapsed into coma, from a presumed baclofen overdose. She was intubated and ventilated. The reservoir was emptied of baclofen and the pump stopped. Seventeen hours after the baclofen overdose, the patient woke up gradually with no new neurological deficits. The pump was removed a week later. Medtronic laboratories examined the pump and reported no technical fault. DISCUSSION: The implanted Medtronic SynchroMed II pump suffered an unusual malfunction. It is postulated that the pump had suffered a motor stall, and when it was restarted, it gave an unusually high, potentially lethal, dose to the patient. CONCLUSION: Physicians who implant pumps for intrathecal baclofen administration need to be aware that these devices may suffer unheralded catastrophic failure that can lead to potentially lethal overdose administration.

Acquired Chiari I malformation in a child with corrected diastematomyelia disappeared after thickened filum division.

INTRODUCTION: In recent years there has been renewed interest in the relation between Chiari I malformation and spinal cord tethering. CASE REPORT: A 3-year-old girl presented with right leg pain. At spinal MR scan there was diastematomyelia with a bony peg at L1 and syringomyelia at T10-11. She had a surgical repair of the diastematomyelia without filum division. Symptoms settled postoperatively. A few months later she started displaying weakness in the right leg and loss of bulkiness of the right calf. A new MR scan showed a new Chiari I malformation, which was not present in the preoperative MR scan. The syringomyelia cavity was smaller. There was low-ending conus medullaris at L3 and a thickened filum. The filum was divided 15 months after the first operation. At MR scan obtained 18 months after filum division, the previously noted acquired Chiari I malformation had disappeared. DISCUSSION: There is no obvious explanation for the formation of acquired Chiari I malformation and its disappearance after filum division. Recently, there has been description of patients with Chiari I malformation and normal posterior fossa volume, who were treated with filum division, and their Chiari malformation regressed without craniovertebral decompression surgery. Nevertheless, it is difficult to explain how traction on the lowest aspect of the spinal cord could lead to herniation of the cerebellar tonsils through the foramen magnum.

Systematic review: endoscopic and imaging-based techniques in the assessment of portal haemodynamics and the risk of variceal bleeding.

BACKGROUND: Invasive measurement of the hepatic venous pressure gradient (HVPG) is regarded as the gold standard for risk stratification and the evaluation of pharmaceutical agents in patients with portal hypertension. AIM: To review the techniques for endoscopic and imaging-based assessment of portal haemodynamics, with particular emphasis on trials where the results were compared with HVPG or direct portal pressure measurement. METHODS: Systematic search of the MEDLINE electronic database with keywords: portal hypertension, variceal bleeding, variceal pressure, endoscopic ultrasound, Doppler ultrasonography, magnetic resonance angiography, CT angiography, hepatic venous pressure gradient. RESULTS: Computed tomography angiography and endoscopic ultrasound (EUS) have been both employed for the diagnosis of complications of portal hypertension and for the evaluation of the efficacy of endoscopic therapy. Colour Doppler ultrasonography and magnetic resonance angiography has given discrepant results. Endoscopic variceal pressure measurements either alone or combined with simultaneous EUS, correlate well with HVPG and risk of variceal bleeding and have a low interobserver variability. CONCLUSIONS: Endoscopic and imaging-based measurements of portal haemodynamics provide an alternate means for the assessment of complications of portal hypertension. Further studies are required to validate their use in risk stratification and the evaluation of drug therapies in patients with portal hypertension.

Brain tissue oxygenation changes in children during the first 24 h following head injury.

AIM: The aim of this study is to assess the changes of brain tissue oxygen levels in children during the first 24 h following head injury and its correlation with changes of intracranial pressure and clinical outcome. METHOD: Invasive monitoring of partial brain tissue oxygen tension (PbtO(2)) using the Licox (Integra Neurosciences, Plainsboro, NJ, USA) oxygen probe was performed in children with severe head injury requiring ventilation, during the years 2002-2005. The study focused in the recordings of the first 24 h following injury. RESULTS: There were four patients (three males, one female) with an age range of 2-12 years. All injuries were due to motor vehicle accidents. The Glasgow Coma Score ranged from 5 to 9. All patients had diffuse axonal injuries on Computed Tomography scan. One patient underwent a bilateral decompressive craniectomy. The total duration of monitoring was 567.84 h. During the first 24 h, the mean PbtO(2) was 4.2 mmHg, 12.7 mmHg, 21.8 mmHg, and 25.1 mmHg in each patient. Fifteen episodes of ICP>20 mmHg were seen in the first 24 h of monitoring. Nine of these episodes were accompanied by a reduction in PbtO(2) levels. The Glasgow Outcome Score at 1 year was good recovery (GOS 3) in three patients and severe disability in one patient. There were no complications from the monitoring. CONCLUSIONS: In children with head injury, rise in ICP may be accompanied by fall in PbtO(2). However, low brain oxygen levels during the first 24 h following head injury may not correlate necessarily with poor outcome.

Identification and characterisation of childhood cerebellar tumours by in vivo proton MRS.

(1)H MRS has great potential for the clinical investigation of childhood brain tumours, but the low incidence in, and difficulties of performing trials on, children have hampered progress in this area. Most studies have used a long-TE, thus limiting the metabolite information obtained, and multivariate analysis has been largely unexplored. Thirty-five children with untreated cerebellar tumours (18 medulloblastomas, 12 pilocytic astrocytomas and five ependymomas) were investigated using a single-voxel short-TE PRESS sequence on a 1.5 T scanner. Spectra were analysed using LCModel to yield metabolite profiles, and key metabolite assignments were verified by comparison with high-resolution magic-angle-spinning NMR of representative tumour biopsy samples. In addition to univariate metabolite comparisons, the use of multivariate classifiers was investigated. Principal component analysis was used for dimension reduction, and linear discriminant analysis was used for variable selection and classification. A bootstrap cross-validation method suitable for estimating the true performance of classifiers in small datasets was used. The discriminant function coefficients were stable and showed that medulloblastomas were characterised by high taurine, phosphocholine and glutamate and low glutamine, astrocytomas were distinguished by low creatine and high N-acetylaspartate, and ependymomas were differentiated by high myo-inositol and glycerophosphocholine. The same metabolite features were seen in NMR spectra of ex vivo samples. Successful classification was achieved for glial-cell (astrocytoma + ependymoma) versus non-glial-cell (medulloblastoma) tumours, with a bootstrap 0.632 + error, e(B.632+), of 5.3%. For astrocytoma vs medulloblastoma and astrocytoma vs medulloblastoma vs ependymoma classification, the e(B.632+) was 6.9% and 7.1%, respectively. The study showed that (1)H MRS detects key differences in the metabolite profiles for the main types of childhood cerebellar tumours and that discriminant analysis of metabolite profiles is a promising tool for classification. The findings warrant confirmation by larger multi-centre studies.

Comparison of prenatal and postnatal MRI findings in the evaluation of intrauterine CNS anomalies requiring postnatal neurosurgical treatment.

AIM: To assess the diagnostic capability of fetal magnetic resonance imaging (MRI) in children suspected antenatally to harbor central nervous system (CNS) defects that require immediate postnatal neurosurgical treatment. MATERIALS AND METHODS: Between 2003 and 2005, 13 fetal MRI scans were performed in mothers suspected to have fetuses with congenital CNS defects that would require surgery soon after birth. Comparisons between antenatal and postnatal scans were made with emphasis on diagnostic accuracy of antenatal examinations. RESULTS: All mothers were scanned using heavily T2-weighted fat-saturated sequences, allowing rapid acquisitions to avoid movement artefacts. Imaging quality was satisfactory in all patients. Diagnoses made antenatally were: myelomeningocele in seven, meningocele in one, diastematomyelia in one, occipital meningocele in one, and isolated hydrocephalus in three children. Of the seven children with antenatal diagnosis of myelomeningocele, one proved to have spinal lipoma postnatally. The patient who antenatally was diagnosed with meningocele proved to have spinal lipoma postnatally. These two were early antenatal MR scans. Antenatal diagnosis of hydrocephalus was made in five of the six confirmed myelomeningocele patients, which was verified postnatally. Antenatal diagnosis of Chiari II malformation was made in all six confirmed myelomeningocele patients. The antenatal diagnoses of occipital meningo-encephalocele and isolated hydrocephalus were verified postnatally. Antenatal diagnosis of diastematomyelia was not verified postnatally. CONCLUSION: Fetal MRI scanning is an effective, noninvasive method of assessing in-utero CNS abnormalities. The diagnostic accuracy has improved to allow prediction of clinical outcome and counseling for possible treatment, but is not perfect yet to allow counseling for termination of pregnancy.

Pattern, management and outcome of cervical spine injuries associated with head injuries in paediatric patients.

INTRODUCTION: This study examines the management and outcome of cervical spine injuries in children with head injuries, to assess the need for surgical treatment. MATERIAL AND METHODS: We performed a retrospective analysis (1995-2005) of 445 children admitted intubated and ventilated to the intensive care unit with head injuries. OUTCOME MEASURES: Frankel grade for spinal injuries and Glasgow Outcome Scale (GOS) for head injuries. RESULTS: Cervical spine injuries were detected in 11 patients (incidence 2.5%, mean age: 6.3 years, range: 21 months-15 years). The injuries were: C1/2 distraction: 2; C1/2 subluxation: 2; odontoid peg fracture with C1/2 dislocation and cord transection: 1; disruption of posterior longitudinal ligament at C2: 1; odontoid peg fracture with C2/3 distraction: 1; C2/3 subluxation: 1; C3 lamina fracture: 1; C3/4 facet fracture: 1; C6/7 fracture dislocation with cord transection: 1. One patient was managed operatively, ten patients nonoperatively, two with halo vests and eight with hard collars. There were three deaths (mortality 27%) associated with severe head injuries. At 6 months follow-up, two patients remained quadriplegic (Frankel Grade A), one of them ventilator dependent, one had residual motor function but of no practical use (Frankel Grade C), five had good spinal outcome (Frankel Grade E). Seven patients had good head injury outcomes (GOS 5), one remained disabled (GOS 3). CONCLUSION: Most children with cervical injury can be managed nonoperatively with good outcomes. Surgical management may be necessary in severe unstable injuries.

Clinical trial: The effect of somatostatin vs. octreotide in preventing post-endoscopic increase in hepatic venous pressure gradient in cirrhotics with bleeding varices.

BACKGROUND: Hepatic venous pressure gradient (HVPG) increases significantly after endoscopic therapy in patients with bleeding oesophageal varices, which may precipitate further haemorrhage. Whether vasoactive drugs can suppress these changes remains unknown. AIM: To investigate the efficacy of somatostatin when compared with octreotide in preventing the post-endoscopic increase in HVPG during acute bleeding and whether the changes affect outcome. METHODS: Thirty-three cirrhotics with bleeding varices were randomized to receive somatostatin (n = 17) or octreotide (n = 16) under double-blind conditions, soon after their admission. HVPG measurements were performed before and immediately after endoscopic treatment. RESULTS: In the somatostatin group, postendotherapy HVPG values did not change significantly when compared with pre-treatment values (18.9 vs. 17.2, P = 0.092). Conversely, in the octreotide group, HVPG increased significantly after endoscopy (18.2 vs. 20.8, P = 0.003). The probability of 6-week survival without treatment failure was significantly higher in the somatostatin group (P = 0.024). Post-endoscopic HVPG value was independently associated with 6-week failure. CONCLUSIONS: Somatostatin, but not octreotide, effectively prevents the post-endoscopic increase in HVPG, which may be associated with low probability of treatment failure.

Surgical management of spasticity of cerebral origin in children.

In children, spasticity is commonly seen in the context of cerebral palsy (CP), but also following head injury, cerebral infarct or other brain insults. CP is a wide term used to describe a constellation of symptoms that characterise the physical impairment of movement due to abnormal brain development. The management of spasticity is tailored according to the clinical picture of the child. Ambulatory mild spastic diplegics tend to reach the maximum of their disability in the first few years of life, and change little after the age of 5-7 years. Such patients who are between 3-5 years and who attempt to mobilise with walking frames are often good candidates for either dorsal rhizotomy or intrathecal baclofen (ITB) administration with the implantation of an indwelling pump. Non-ambulatory mild spastic diplegics and spastic quadriplegics have more profound spasticity, painful spasms, orthopaedic deformities, and difficulties with daily care and posture. ITB has become established as the first line of surgical treatment for these patients. In the last decade, there has been a definite trend away from ablative treatments and towards reversible stimulation and infusion systems. Current pumps have practical limitations but, in the next decade, it is anticipated that technological improvements will render the pumps more patient friendly.

Bilateral exudative retinal detachment in a child with orbital pseudotumor.

We describe a 15-year-old girl who presented with bilateral exudative retinal detachment, a previously unreported complication, due to orbital pseudotumor. She initially responded to steroids, but subsequently became steroid dependent. Azathioprine was effective in controlling further relapses during follow-up of 22 months.

Incidence of vascular malformations in spontaneous intra-cerebral haemorrhage in children.

AIM: To assess the incidence of vascular malformations in children presenting with non-traumatic intra-cerebral haemorrhage and outline the need for radiological investigations. MATERIALS AND METHODS: Between 1993 and 2002, 26 children presented with spontaneous intra-cerebral haemorrhage at a mean age of 26 months (range 1-192 months). RESULTS: Twenty-two children had, as first treatment, surgical removal of the haematoma and any malformation found. One patient with no evidence of vascular malformation had conservative treatment, two had embolisation and one had stereotactic radiosurgery of arteriovenous malformations (AVMs). The diagnosis of vascular malformation was confirmed histologically and/or radiologically in 16 (61%) patients. Of these 16 patients, 7 were AVMs, 1 thrombosed middle cerebral artery (MCA) aneurysm, 1 cavernous angioma, 6 aggregates of abnormal vessels, 1 vein of Galen aneurysm. Pre-operatively, 12 patients had magnetic resonance imaging (MRI)/magnetic resonance angiography (MRA) and 7 were positive for vascular malformation (1 false positive) with no false negatives. Digital subtraction angiography (DSA) was performed pre-operatively in 7 patients, 4 were positive with no false negatives. There were no re-bleeds at the follow-up period. Five (19%) patients died from the haemorrhage, 7 (27%) had severe neurological deficit and 14 (54%) had no neurological deficit. Residual malformation after surgery requiring additional treatment was found in 3 (16%) patients. All patients with confirmed malformations were followed-up with DSA. CONCLUSION: MRI/MRA has high sensitivity and specificity in identifying vascular malformations in children presenting acutely with spontaneous intra-cerebral haemorrhage. This may prove useful when pre-operative DSA is not promptly available. After acute clot evacuation, there is high incidence of residual malformation and such patients should be followed-up with DSA.

Diastematomyelia in children: treatment outcome and natural history of associated syringomyelia.

OBJECTIVE: To quantify the long-term outcome of children with diastematomyelia and the implication of syringomyelia in the natural history. MATERIALS: Retrospective study of 17 children (nine girls and eight boys) with diastematomyelia operated during 1989-2004. Mean age at diagnosis was 3.4 years (range 5 days-12 years), mean follow-up was 5 years; 14 had excision of spur and cord untethering, 3 had excision of spur alone. RESULTS: The bony spur was in the lumbar region in 12 and thoracic in 5 patients. Syringomyelia was present in eight (47%), associated with bony spur between L1 and 4 (p=0.088), spina bifida occulta in ten (58.8%), and spinal lipoma in one (6%). Preoperative Necker Enfants Malades (NEM) scores were 17 in eight patients, 16 in four, 15 in three, 13 in one, and 12 in one patient. Presenting neurological deficits were motor in eight, sensory deficits in three, and anal incontinence in one patient. There was improvement of NEM scores postoperatively in five patients only, but still with residual deficit. Repeat spur excision and cord untethering was performed in three patients for neurological deterioration 1-4 years after first operation. On postoperative MRI scans syringomyelia remained unchanged in all eight patients. There were one skin infection, one transient motor deterioration, and one patient with sensory deficit after surgery. CONCLUSION: Prophylactic operations were associated with the best clinical outcome. Despite improvement, all patients with established preoperative deficit still had residual neurological deficits at their last follow-up. The associated syringomyelia remained unchanged after surgery, indicating that it does not contribute to the neurological syndrome.

Short echo time 1 H magnetic resonance spectroscopy of childhood brain tumours.

AIMS: To explore short echo time (30 ms) 1 H magnetic resonance spectroscopy (MRS) in children with brain tumours and determine the contributions to the characterization of these tumours of the metabolites inositol/myoinositol and glutamate/glutamine, which are not visible at long echo times (135 or 270 ms). METHODS: Over a 12-month period 86 single-voxel MRS investigations were performed on 59 children with various brain tumours on a Siemens Symphony 1.5-T Magnetom using point-resolved spectroscopy and echo time of 30 ms. RESULTS: The procedure was well tolerated, and good-quality data were obtained. N-Acetyl aspartate (NAA)/Choline (Cho) and creatine (Cr)/Cho concentration ratios were significantly (p<0.001) lower in tumour (0.95 and 1.63, respectively) compared with non-involved brain (3.68 and 3.98, respectively) in all histological types. Inositol/Myoinositol (Inos)/Cho ratios were significantly (p<0.05) lower in untreated tumours (1.91) than in treated tumours (3.93) and in non-involved brain (3.32). Inos/Cho ratios were high in diffuse pontine gliomas and low in medulloblastomas and supratentorial primitive neuroectodermal tumours (p<0.01). Glutamate/Glutamine (Glut)/Cho ratios were high in grade 1 astrocytomas (6.4) and unbiopsied optic gliomas (9.84) but low in diffuse pontine gliomas (2.44). Lipids and macromolecules were present in most tumours but in low quantities in non-involved brain. CONCLUSION: Good-quality short echo time MRS data can be collected routinely on children with brain tumours. Inos and Glut levels show greater variability between tumour types than NAA, Cho and Cr present at long echo times, providing improved tumour characterization. Inos/Cho levels differ between untreated and treated tumours and may be useful for treatment monitoring.

Outcome of children with posterior fossa medulloblastoma: a single institution experience over the decade 1994-2003.

AIM: While the impact of radiotherapy in the management of medulloblastoma was recognised, the introduction of chemotherapy was investigated in clinical trials and shown to confer an additional advantage. We reviewed the outcome of a series of consecutive patients to assess the impact in a population-based clinical establishment. MATERIALS AND METHODS: A series of 38 children treated for medulloblastoma at Birmingham Children's Hospital between 1994 and 2003 was analysed. The effect of surgery, radiotherapy, chemotherapy and metastasis on survival was analysed. RESULTS: The overall 5-year survival rate was 61.4% for the 36 patients who had resective surgery, while 2 patients had biopsy only and died within a few months. There was no operative mortality. The incidence of hydrocephalus needing permanent shunting was higher in the first 3 years of life (p = 0.007, chi-square). The 5-year survival rate of patients with total and sub-total excision of medulloblastoma was 61.1% and 61.8%, respectively. The 5-year survival rate of patients older than 3 years was 73.4% and for patients under 3 years was 36.3% (p = 0.007, log rank). Metastases at presentation did not influence survival. All deaths occurred in the first 32 months. CONCLUSION: The contribution of chemotherapy in the improvement of the overall survival appears more evident in children younger than 3 years or presenting with metastases. The absence of significant difference in survival between patients with total or sub-total excision of medulloblastoma supports the view that total excision of medulloblastoma can be avoided when the risk for potential intra-operative damage and consequent neurological deficits is high.

Systematic review: sphincter of Oddi dysfunction--non-invasive diagnostic methods and long-term outcome after endoscopic sphincterotomy.

BACKGROUND: Sphincter of Oddi dysfunction is a benign, functional gastrointestinal disorder for which invasive endoscopic therapy with potential complications is often recommended. AIMS: To review the available evidence regarding the diagnostic accuracy of non-invasive methods that have been used to establish the diagnosis and to estimate the long-term outcome after endoscopic sphincterotomy. METHODS: A systematic review of English language articles and abstracts containing relevant terms was performed. RESULTS: Non-invasive diagnostic methods are limited by their low sensitivity and specificity, especially in patients with Type III sphincter of Oddi dysfunction. Secretin-stimulated magnetic resonance cholangiopancreatography appears to be useful in excluding other potential causes of symptoms, and morphine-provocated hepatobiliary scintigraphy also warrants further study. Approximately 85%, 69% and 37%, of patients with biliary Types I, II and III sphincter of Oddi dysfunction, respectively, experience sustained benefit after endoscopic sphincterotomy. In pancreatic sphincter of Oddi dysfunction, approximately 75% of patients report symptomatic improvement after pancreatic sphincterotomy, but the studies have been non-controlled and heterogeneous. CONCLUSIONS: Patients with suspected sphincter of Oddi dysfunction, particularly those with biliary Type III, should be carefully evaluated before considering sphincter of Oddi manometry and endoscopic sphincterotomy. Further controlled trials are needed to justify the invasive management of patients with biliary Type III and pancreatic sphincter of Oddi dysfunction.

Eosinophilic esophagitis in adults: what is the clinical significance?

Eosinophilic esophagitis is a condition that is being increasingly recognized in adults. The main presenting symptoms are dysphagia (93 %), food impaction (62 %), and heartburn (24 %). A history of allergy is obtained in 52 % of patients and peripheral eosinophilia is found in 31 % of patients with this condition. Esophageal manometric studies have provided evidence of a nonspecific motility disorder in 40 % of patients. Endoscopic findings include mucosal fragility or edema (59 %), solitary or multiple concentric rings (49 %), strictures (40 %), whitish pinpoint exudates or papules (16 %), and a small-caliber esophagus (5 %); 9 % of patients present with a normal endoscopy. Esophageal dilation in adults with eosinophilic esophagitis has limited efficacy and is associated with an increased complication rate compared with dilation in patients with benign strictures. The administration of inhaled or systemic corticosteroids results in symptomatic improvement in nearly 95 % of patients. A trial with corticosteroids before bougienage may reduce active inflammation and therefore the complication rate of the procedure.

Clinical outcome of patients with Helicobacter pylori infection: the bug, the host, or the environment?

It is well established that only a minority of patients with Helicobacter pylori infection develop severe inflammation leading to peptic ulcer or gastric cancer. Recent evidence suggests that the virulence factors of the organism do not seem crucial in the progression of inflammation towards a more severe disease. It seems probable that other host derived and environmental factors are more significant in determining clinical outcome but additional studies are needed to clarify the underlying mechanisms involved in the pathogenesis of infection.