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.

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.

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.

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.

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.

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.

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.

Somatostatin and its analogues in peptic ulcer bleeding: facts and pathophysiological aspects.

Peptic ulcer bleeding remains a common medical emergency and despite recent advances in management is still associated with high mortality. Endoscopic treatment remains the cornerstone for the effective management of high-risk patients. Recent evidence suggests that potent antisecretory drugs that inhibit gastric acid secretion, such as proton pump inhibitors, may be of help alone or in combination with endotherapy in the management of peptic ulcer bleeding. Somatostatin appears to offer a distinct advantage over antisecretory drugs, as it inhibits both acid and pepsin secretion and combines these effects with a reduction in gastroduodenal mucosal blood flow which seems to be important in the pathophysiology of peptic ulcer bleeding. Additionally, the inhibition of pepsin secretion might induce a decreased proteolytic activity preventing the dissolution of freshly formed clots at the site of bleeding. Despite its theoretical advantages, there has been very little evidence in the recent past in setting of randomised, controlled, clinical trials. In reviewing the available data, we found that the efficacy of somatostatin and its analogue octreotide are different in the control of peptic ulcer bleeding and this might be due to the different distribution of its receptors through the GI tract. Further studies are needed to define the exact role, if any, of somatostatin and its analogues, in high-risk patients with peptic ulcer bleeding and this might be a rather interesting area for future research.

Endoscopic ultrasonography in the diagnosis and management of portal hypertension. Where are we next?

Endoscopic ultrasonography has recently emerged as an accurate, non-invasive and reproducible alternative means of providing data for patients with portal hypertension. It is well established that endoscopic ultrasonography is more sensitive than endoscopy in the diagnosis of gastric varices. Dilated venous abnormalities outside the gastrooesophageal lumen, which cannot be diagnosed by endoscopy, are readily visible with endoscopic ultrasonography or miniature probes. Endoscopic ultrasonography is also useful to predict the risk of variceal recurrence and thus the risk of rebleeding after endotherapy which cannot be reliably predicted using endoscopy alone. The introduction of echo endoscopes equipped with Doppler facilities has allowed the sonographic visualisation of the vessels and the evaluation of vascular blood flow along with possible morphologic and haemodynamic changes after endoscopic or pharmacological therapy. However, despite its theoretical advantages, relative evidence suggests that in the clinical setting of portal hypertension, endoscopic ultrasonography remains an investigational tool with limited clinical applications.

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.

A critical appraisal of drainage in syringomyelia.

The use of drains in the treatment of syringomyelia has a simple and immediate appeal and has been practiced widely since the report of Abbe and Coley over 100 years ago. Good short-term results have been claimed in the past, but long-term outcome is largely unknown. An experience in Birmingham, England is reviewed in which 73 patients who had had some form of syrinx drainage procedure performed were subsequently followed up. In these cases, a total of 56 syringopleural and 14 syringosubarachnoid shunts had been inserted. Ten years after the operations, only 53.5% and 50% of the patients, respectively, continued to remain clinically stable. A 15.7% complication rate was recorded, including fatal hemorrhage, infection, and displacement of the drain from the pleural and syrinx cavities. At second operation or necropsy, at least 5% of shunts were discovered to be blocked. The effect of other drainage procedures that do not use artificial tubing, such as syringotomy and terminal ventriculostomy, was analyzed but found not to offer any substantial benefit. These results indicate that drainage procedures are not an effective solution to remedying the progressive, destructive nature of syringomyelia. It is suggested that, rather than attempting to drain the syrinx cavity, disabling the filling mechanism of the syrinx is more appropriate. Most forms of syringomyelia have a blockage at the level of the foramen magnum or in the subarachnoid space of the spine. Surgical measures that aim to reconstruct the continuity of the subarachnoid space at the site of the block are strongly recommended. Lowering the overall pressure of the cerebrospinal fluid is advocated when reestablishment of the pathways proves impossible. Syrinx drainage as an adjuvant to more physiological surgery may have a place in the treatment of syringomyelia. If two procedures are done at the same time, however, it is difficult to ascribe with certainty a success or failure, and it is suggested that the drainage procedure be reserved for a later attempt if the elective first operation fails.

Management and outcome of posttraumatic syringomyelia.

Traumatic paraplegia is the most common cause of nonhindbrain-related syringomyelia. Fifty-seven patients with a mean age of 34.3 years at presentation were treated at the Midland Centre for Neurosurgery and Neurology between 1973 and 1993. A variety of treatment strategies have been used over the years, including syringosubarachnoid and syringopleural shunts, spinal cord transection, and pedicled omental graft transposition. More recently decompressive laminectomy, subarachnoid space reconstruction and formation of surgical meningocele have been used. A total of 81 operations were performed in these patients, 69 of them at the Syringomyelia Clinic. Combinations of strategies were often chosen; the use of one strategy such as drainage did not preclude another such as transection or augmentation of the cerebrospinal fluid pathways. The overall postoperative complication rate was 12%. Problems specific to the operation type included dislodged, blocked, and infected drains (10 patients). Acute gastric dilation was seen following pedicled omental graft (one patient). At 6 years only 49% of the drains inserted still functioned. A higher than expected rate of cervical spondylotic myelopathy has been noted. Two patients developed Charcot's joints. Thirty-six patients were asked to score themselves with regard to limb function and performance of daily living activities and 30% reported improvement, particularly ion arm function. Since the use of magnetic resonance imaging has become widespread, it has become apparent that decompressive laminectomy with subarachnoid space reconstruction is effective in controlling the syrinx cavity. In complete paraplegia, spinal cord transection is an effective alternative. Pedicled omental grafting was associated with poor outcome and an increased complication rate and has been abandoned.

Sylvian aqueduct syndrome and global rostral midbrain dysfunction associated with shunt malfunction.

OBJECT: This study is a retrospective analysis of clinical data obtained in 28 patients affected by obstructive hydrocephalus who presented with signs of midbrain dysfunction during episodes of shunt malfunction. METHODS: All patients presented with an upward gaze palsy, sometimes associated with other signs of oculomotor dysfunction. In seven cases the ocular signs remained isolated and resolved rapidly after shunt revision. In 21 cases the ocular signs were variably associated with other clinical manifestations such as pyramidal and extrapyramidal deficits, memory disturbances, mutism, or alterations in consciousness. Resolution of these symptoms after shunt revision was usually slow. In four cases a transient paradoxical aggravation was observed at the time of shunt revision. In 11 cases ventriculocistemostomy allowed resolution of the symptoms and withdrawal of the shunt. Simultaneous supratentorial and infratentorial intracranial pressure recordings performed in seven of the patients showed a pressure gradient between the supratentorial and infratentorial compartments, with a higher supratentorial pressure before shunt revision. Inversion of this pressure gradient was observed after shunt revision and resolution of the gradient was observed in one case after third ventriculostomy. In six recent cases, a focal midbrain hyperintensity was evidenced on T2-weighted magnetic resonance imaging sequences at the time of shunt malfunction. This rapidly resolved after the patient underwent third ventriculostomy. CONCLUSIONS: It is probable that in obstructive hydrocephalus, at the time of shunt malfunction, the development of a transtentorial pressure gradient could initially induce a functional impairment of the upper midbrain, inducing upward gaze palsy. The persistence of the gradient could lead to a global dysfunction of the upper midbrain. Third ventriculostomy contributes to equalization of cerebrospinal fluid pressure across the tentorium by restoring free communication between the infratentorial and supratentorial compartments, resulting in resolution of the patient's clinical symptoms.

Mechanical dysfunction of ventriculoperitoneal shunts caused by calcification of the silicone rubber catheter.

OBJECT: The authors studied new and calcified shunt catheters to identify the prevalence of failures caused by aging materials in the shunt. Complications associated with these devices have various origins. Among late complications, fracture or migration of the system is related to the subcutaneous adhesion of the distal tubing in a growing child. A review of a cohort of 64 children who underwent shunt placement in 1980 with barium-impregnated distal catheters showed that 10 of these patients underwent reoperation for complications related to aging of the shunt material. This group represents 15% of the whole series and 30% of those children who were followed for more than 3 years. The true impact of aging of materials on shunt function is probably underestimated. METHODS: The authors performed physical, chemical, and mechanical analyses of the retrieved aged catheters and also of new catheters, resulting in the following findings: 1) calcifications were observed only on the external surface of the catheter, predominantly in its subcutaneous segment at the level of the neck and anterior chest wall; 2) calcifications contained particles of free silicon and barium sulfate, signifying fragmentation of the polymer; 3) the microstructure of the silicone polymer was modified: microfractures and alteration of the polymeric network were observed; 4) silanol groups were observed on the external surface of the catheter; and 5) the mechanical properties of the silicone rubber were degraded, and the aged catheters were more brittle than the new ones, with ruptures at elongations and fracture energy much lower than that seen in new catheters. Furthermore, in vitro testing with a metastable solution of simulated body fluid demonstrated the critical impact of pH variations in liquid media and surface degradation of the catheters on the precipitation of hydroxylapatite crystals. CONCLUSIONS: Although most shunt complications can be addressed by better patient management and surgical technique, late complications appear to be partly related to aging of the material. Distal tubing calcifications have been observed in barium-impregnated catheters. The industry recently responded to these observations by introducing plain silicone-coated shunt tubing; further evaluation will show what improvement can be expected.

Intracranial volume change in childhood.

OBJECT: The goal of this study was to construct a model of normal changes in intracranial volume occurring throughout childhood from age 7 days to 15 years. METHODS: Using the technique of segmentation on magnetic resonance images obtained in healthy children, intracranial volume was measured and plotted against age. CONCLUSIONS: Intracranial volume in the first few months of life is on average 900 cm3 in males and 600 cm3 in females. By the age of 15 years, it increases up to 1500 cm3 in males and 1300 cm3 in females, increased by factors of 1.6 and 2.1, respectively. By the time the child reaches 2 years of age, intracranial volume has reached 77% (1150 cm3 in males and 1000 cm3 in females) and, by 5 years, 90% (1350 cm3 in males and 1200 cm3 in females) of the volume observed at age 15 years. The change in intracranial volume that occurs with age is not linear, but there seems to be a segmental pattern. Three main periods can be distinguished, each lasting approximately 5 years (0-5, 5-10, and 10-15 years), during which the growth of intracranial volume is linear. Throughout childhood, males have higher intracranial volumes than females, with a similar growth pattern.

Intracranial volume change in craniosynostosis.

OBJECT: There is still controversy regarding the optimum time to perform surgery for craniosynostosis. Some recommend surgery soon after birth and others delay until the age of 12 months. Intracranial pressure has been measured in an attempt to provide a scientific rationale, but many questions remain unanswered. To date, little attention has been given to intracranial volume and its changes during the first few years of life in children with craniosynostosis. The authors' goal was to focus on intracranial volume during this period and to compare measurements obtained in patients with craniosynostosis with measurements obtained in healthy individuals. METHODS: Using the technique of segmentation, the intracranial volume of 84 children with various forms of craniosynostosis was measured on preoperative computerized tomography scans. The change in average volume that occurs with increasing age was calculated and compared with a model of normal intracranial volume growth. The age at presentation for children with craniosynostosis was 1 to 39 months; 76% of the patients were younger than 12 months. In eight patients in whom only one cranial expansion procedure was performed, postoperative intracranial volumes were measured as well. Several interesting observations emerged. 1) There was little difference in head growth between boys and girls with craniosynostosis during the first few months of life. After the age of 12 months, however, the difference in intracranial volume normally seen between the two genders was observed in the craniosynostosis group as well. 2) Excluding children with complex pansynostosis, who have smaller heads, children with all other types of craniosynostosis have similar head growth after the 1st year of life, with no difference between the number of and type of suture affected. Children with Apert's syndrome develop greater than normal intracranial volumes after the 1st year of life. 3) Although children with craniosynostosis are born with a smaller intracranial volume, by the age of 6 months volume has reached normal levels, and from that point on volume follows the pattern of normal head growth. 4) Children who presented after the age of 6 months and later developed recurrent craniosynostosis after initial successful treatment had a small intracranial volume at their initial presentation. 5) Of the patients whose postoperative intracranial volumes were measured, all but one had preoperative volumes at or above normal values, and their postoperative volumes were considerably higher than normal for their age. These children all followed a growth curve parallel to that of healthy children but at higher volume value. One patient with a smaller-than-normal initial intracranial volume was surgically treated at a very young age and, despite cranial expansion surgery, postoperative volume did not reach normal levels. It is postulated that this was due to the fact that the operation was performed at a time when craniosynostosis was still active. CONCLUSIONS: The results of this study indicate that the underlying mechanism leading to craniosynostosis and constriction of head volume "exhausts" its effect during the first few months of life. Measurement of intracranial volume in clinical practice could be used to "fine tune" the optimum time for surgery. In late-presenting children, this may be useful in predicting possible recurrence.