Slit Ventricle Syndrome Research Articles

Cranial expansion and aqueductoplasty for combined isolated fourth ventricle and slit-ventricle syndrome: a surgical alternative.

An isolated fourth ventricle (IFV) is a rare entity observed in shunted patients and its treatment is still uncertain. Endoscopic aqueductoplasty has shown good results for restoring CSF flux between the third and fourth ventricles. However, it needs some grade of ventricular dilation to be performed. Some patients affected by IFV show slit-ventricle morphology in CT/MRI. Usually, the rise of opening pressure or the shunt externalization gets enough ventricular dilation. However, the lack of intracranial compliance in some patients makes these options unsuitable and high-ICP symptoms are developed without ventricular dilation. We present a two cases series affected by IFV with no ventricular dilation in radiological exams. ICP sensors were implanted, observing high-ICP and establishing the diagnosis of craniocerebral disproportion. A two-stage surgical plan based on a dynamic cranial expansion followed by a supratentorial endoscopic aqueductoplasty was performed. A physical and mathematical model explaining our approach was also provided. Chess-table cranial expansion technique was performed in both patients. Six/seven days after the first surgery, respectively, ventricular dilation was observed in CT. Endoscopic precoronal aqueductoplasty was then performed. No postoperative complications were described. IFV symptoms improved in both patients. Eighteen and 12 months after the two-stage surgical plan, the patients remain symptom-free and void of flow is still observed between the third and the fourth ventricles in MRI. The two-stage approach was a suitable option for the treatment of these complex patients affected by both craniocerebral disproportion and isolated fourth ventricle.

Novel Endoscopic Technique for Inserting a Sheath into a Slit Ventricle: The Water-Slide Technique

The efficacy and safety of endoscopic procedures for slit ventricle syndrome (SVS) have been presented in recent studies. However, inserting a sheath into a target ventricle is essential in the beginning of endoscopic procedures for SVS, and this maneuver is challenging owing to the quite narrow ventricular space. We report a novel flexible endoscopic technique, the water-slide technique, that can effectively guide a sheath into a slit ventricle without the use of other adjunctive devices, such as a neuronavigation system and an ultrasound machine. Ten endoscopic procedures using the water-slide technique were performed in 9 patients with SVS. All patients had undergone ventriculoperitoneal shunt placement. Using the technique, the sheath was first placed on the flexible endoscope. The endoscope was then inserted into the lateral ventricle along the tract around the pre-existing ventricular catheter with irrigation of artificial cerebrospinal fluid via the working channel of the endoscope. After the endoscope reached the ventricle, the sheath was inserted into the ventricle along the endoscope. The endoscope was then used as a stylet. The endoscopic procedure was performed afterward. Continuous irrigation of artificial cerebrospinal fluid via the working channel is important when using this technique. In all cases, the sheath was successfully introduced to the slit ventricle without the use of adjunctive devices. There were no postoperative complications correlated with the maneuver. This novel endoscopic technique is effective and safe for inserting a sheath into a target ventricle in the management of SVS.

ЭНДОСКОПИЯ ДЛЯ ДОСТИЖЕНИЯ ШУНТНЕЗАВИСИМОГО СОСТОЯНИЯ ПРИ ЛЕЧЕНИИ СИНДРОМА ЩЕЛЕВИДНЫХ ЖЕЛУДОЧКОВ

Objective: Objective: To demonstrate the results of neuroendoscopic operations for achievement of shunt-independent condition in treatment of slit ventricle syndrome. Methods: Forty-three cases were considered: adult patients aged 18 years and over – 5 (11.63%), and children aged 0-17 years – 38 (88.37%). Pediatric patients were divided into the following age groups: under 1 year – 6 (13.95%); 1-2 years – 7 (16.28%); 2-17 years – 25 (58.14%). The interval between operations was an average of 2 years 6 months (from 1 week to 16 years), and the period of observation after surgery – an average of 41.8 months (from 1 week to 100 months). Results: In children under 1 year, the efficiency was 83.3%; 1-2 years old – 71.4%; 2-17-years old – 92%; 60% of adult patients aged 18 and over. The effectiveness of operations performed using standard rigid endoscopes was 72.1%, and using a semi-rigid needle-shaped endoscope – 95.2%. In total, the overall efficiency of the ETV, aqueductoplasty, choroid plexus coagulation, cystocysternostomy, septostomy, ventricular stenting, revision and reconstruction of VPS, plasty and stenting of Foramen of Magendie and Lyushka, independently from the age of the patients, type of endoscopes was 83%. In 68.3% of cases shunt-independent state was achieved. Conclusions: Endoscopic methods have shown their high effectiveness in the treatment of patients with slit ventricle syndrome and other complications of hydrocephalus. Using a more advanced modern endoscopic technique, such as a semi-rigid needle endoscope in our case, allows to restore CSF circulation even in the most distal sections of the interpeduncular and prepontine cisterns, as well as safely remove and install a ventricular catheter with minimal trauma to surrounding structures and achieve a shunt-independent state. Keywords: Slit ventricle syndrome, endoscopic ventriculocysternostomy, obstructive hydrocephalus, semi-rigid needle-shaped neuroendoscope, aqueductoplasty, septostomy.

Management of paediatric hydrocephalous with Miethke fixed pressure gravitational valves. The Alder Hey Children's Hospital experience.

The management of paediatric hydrocephalous remains challenging with the complication and revision rates being consistent in the literature. We hypothesise that the use of a fixed pressure gravitational valve for all de novo shunt insertions decreases the rate of functional revisions and that by implementing the routine use of gravitational valves in children, we would see a reduction in over-drainage and slit ventricle syndrome. Retrospective data collection in a single centre, between February 2010 and August 2018. All patients undergoing fixed pressure gravitational Miethke valve insertion were included. We collected data on patients' demographics, reason for shunt insertion, type of valve and time to and reason for first revision. Data analysis was done with SPSS. A total of 235 patients were included in our study (124 males, 111 females), aged from 0 to 18.6years (median 0.28). A total of 99 shunt revisions were documented, 30 of which secondary to ventricular catheter malfunction and 28 secondary to infection. The overall mechanical valve survival rates were 88.5%, 86.4% and 85.5% at 1, 2 and 5years, respectively. Shunt revision due to over-drainage was documented in only 3 cases (1.3%). Our results are in agreement with existing literature regarding shunt failures secondary to all extrinsic factors to the valve (infection and mechanical failure). We have shown that the use of a Miethke fixed pressure valve for all de novo shunt insertions in paediatric hydrocephalus decreases the need for functional revisions with valve survival rates being superior to the ones described for other types.

Proximal Migration of Lumboperitoneal Shunt Catheter Up To Cervical Spine

Lumboperitoneal (LP) shunt had been in use as an internal cerebrospinal fluid (CSF) diversion for a variety of different indications, namely benign intracranial hypertension, communicating hydrocephalus, slit ventricle syndrome and CSF fistulas. Generally considered to be a simple surgical procedure but certain potential complications are associated with this technique, including chronic subdural hematoma, subarachnoid haemorrhage, acquired Chiari malformations and migration of the shunt tubing.1, 2 Proximal intrathecal migration of LP shunt catheter tip, high up in the cervical spine is a rare reported complication.3, 4, 5, 6, 7, 8 If doubtful on X-rays, Computed Topography (CT) scan confirms the diagnosis. Although multi factorial, this proximal migration is usually because of a faulty technique when anchoring sutures are loosened or cut. Measures employed to avoid this potential complication include appropriate technique for securing the LP shunt tube at the proximal lumbar and the distal peritoneal insertion sites with suture collars, thus enabling fixation of the tube. We report a case of an obese middle age female who developed proximal migration of the lumbar tube up to cervical spine, after having undergone LP shunt for pseudotumor cerebri.

S1-15 SESSION 1: NEUROSURGERY CRANIAL VAULT EXPANSION WITH THE SPLIT BONE FLAP TECHNIQUE IN SHUNT RELATED CRANIOSYNOSTOSIS

Introduction: Shunt-related craniosynostosis causing craniocerebral disproportion represents a particular complication of the treatment of hydrocephalus. When the modification of the shunt opening pressure, does not improve the symptomatology, surgery for correction of craniocerebral disproportion is indicated. We present the results and advantages of the split bi-frontal bone technique that is a modification of the previous used frontal bone advancement technique. Methods: We report our series of 8 patients treated with this technique that consists in a splitting bi-frontal bone flap that is repositioned and left floating posteriorly. Splitting the bon flat permit to increase the cranial volume and to reduce the intracranial pressure. All the patients benefitted from a pre and post-operative cerebral blood flow CT. Results: All patients were relieved from there chronic headaches. This technique increases the volume of the skull favoring the expansion of the brain and allows good esthetical results. No peri-operative complications were reported. The study of cerebral blood flow confirmed the increase of the cerebral blood perfusion and the improvement of the regional cerebral blood flow in borderline regions. All patients in scholar age presented an improvement of their performance Conclusion: The bi-frontal split technique can be used for the treatment of slit ventricle syndrome for its efficacy, the low rate of complications and the good aesthetical results both in children and adults.

Continuous Intracranial Pressure Monitoring in 24 Microcephalic Children with Developmental Delay: A Significant Portion of Microcephalic Children with Developmental Delay Have High Intracranial Pressure

The new direct gradual cranial expansion surgical technique has been used to treat children with postshunt microcephaly and slit ventricle syndrome. To evaluate the feasibility of this new surgical treatment, we studied intracranial pressure (ICP) in microcephalic children with developmental delay. Mean ICP, age, sex, head size, and developmental assessments were compared in 24 microcephalic children with developmental delay who had had continuous ICP monitoring. Children studied included 9 boys and 15 girls with a mean age of 4.9 ± 2.0 years. Mean ICP was 18.7 ± 8.6 mm Hg. Children with high ICP had significantly lower age and higher B wave ratios than children with low ICP. There were no statistically significant differences in developmental scores and head sizes between children with high ICP and children with low ICP. In multiple linear regression analysis, we observed significantly increased risk of mean ICP elevation by B wave ratio and developmental score and decreased risk of mean ICP elevation by age, but not significantly increased risk of mean ICP elevation by head circumferences (z score). Our findings suggest that a portion of microcephalic children with developmental delay have high ICP that cannot be expected from head sizes, and high ICP has decreasing tendency with age.

Clinical characteristics of slit ventricle syndrome after ventriculoperitoneal shunt in children

目的 探讨发生于脑室-腹腔分流术后裂隙脑室综合征(slit ventricle syndrome，SVS)的临床特征，提高儿科医生对SVS的认识。 方法 采用回顾性分析的方法对SVS患儿临床资料进行分析。 结果 7例患儿中原发病分别为先天性脑积水3例，先天性颞极蛛网膜囊肿1例，脑出血1例，颅内感染2例。手术时间为生后3个月～4岁。手术至诊断SVS时间为1.3～11.5年，所有患儿均出现不同程度的头痛，初期多是间断性头痛，严重时出现剧烈头痛伴呕吐，甚至抽搐、视力障碍。 结论 对于进行脑室-腹腔分流术后或者蛛网膜囊肿切除术后患儿出现不明原因的头痛、呕吐时应想到有SVS的可能性。

Chronic Debilitating Headache in Adults Caused by Craniocerebral Disproportion: Treatment by Cranial Vault Expansion.

Craniocerebral disproportion is rarely considered as a cause for chronic, debilitating headache in adults. Children reported with this disorder typically suffer from headaches and lethargy for many years and have multisutural synostosis. The terms craniocerebral disproportion, craniostenosis, and slit-ventricle syndrome are used inconsistently as diagnostic designations. Three adults with craniocerebral disproportion who had been treated in infancy for two different pathologies are reported. All benefited greatly from cranial vault expansion.

Endoscopy in the treatment of slit ventricle syndrome.

The present study aimed to investigate the efficacy of endoscopy in the treatment of post-shunt placement for slit ventricle syndrome (SVS). Endoscopic surgery was performed on 18 patients with SVS between October 2004 and December 2012. Sex, age, causes of the hydrocephalus, ventricular size and imaging data were collected and analyzed. All patients were divided into two groups according to ventricular size and underwent endoscopic surgeries, including endoscopic third ventriculostomy (ETV), endoscopic aqueductoplasty and cystocisternostomy. All treated patients were observed postoperatively for a period of 2 to 3 weeks, and outpatient follow-up was subsequently scheduled for >12 months. Clinical results, including catheter adherence, shunt removal and complications, were analyzed during the follow-up period. The success rate of endoscopic surgery was indicated to be 82.7%. Syndromes caused by aqueductal stenosis in 15 patients who underwent ETV were relieved; however, syndromes in the 3 patients with cerebral cysticercosis, suprasellar arachnoid cysts, pinea larea glioma and communicating hydrocephalus, respectively, were not relieved and underwent shunt placement again. Brain parenchyma, choroid plexus and ependymal tissue were the predominant causes for catheter obstruction and the obstruction rate was indicated to be 77.8% (14/18). Complications, such as pseudobulbar paralysis, infection and intraventricular hemorrhage arose in 3 patients. The present study indicates that endoscopic treatments are effective and ETV may be considered as a recommended option in the treatment of post-shunt placement SVS in hydrocephalus patients.

Shunt-dependent hydrocephalus: management style among members of the American Society of Pediatric Neurosurgeons.

OBJECTIVE The authors conducted a survey to evaluate differences in the understanding and management of shunt-dependent hydrocephalus among members of the American Society of Pediatric Neurosurgeons (ASPN). METHODS Surveys were sent to all 204 active ASPN members in September 2014. One hundred thirty responses were received, representing a 64% response rate. Respondents were asked 13 multiple-choice and free-response questions regarding 4 fundamental problems encountered in shunted-hydrocephalus management: shunt malfunction, chronic cerebrospinal fluid (CSF) overdrainage, chronic headaches, and slit ventricle syndrome (SVS). RESULTS Respondents agreed that shunt malfunction occurs most often as the result of ventricular catheter obstruction. Despite contrary evidence in the literature, most respondents (66%) also believed that choroid plexus is the tissue most often found in obstructed proximal catheters. However, free-text responses revealed that the respondents' understanding of the underlying pathophysiology of shunt obstruction was highly variable and included growth, migration, or adherence of choroid plexus, CSF debris, catheter position, inflammatory processes, and CSF overdrainage. Most respondents considered chronic CSF overdrainage to be a rare complication of shunting in their practice and reported wide variation in treatment protocols. Moreover, despite a lack of evidence in the literature, most respondents attributed chronic headaches in shunt patients to medical reasons (for example, migraines, tension). Accordingly, most respondents managed headaches with reassurance and/or referral to pain clinics. Lastly, there were variable opinions on the etiology of slit ventricle syndrome (SVS), which included early shunting, chronic overdrainage, and/or loss of brain compliance. Beyond shunt revision, respondents reported divergent SVS treatment preferences. CONCLUSIONS The survey shows that there is wide variability in the understanding and management of shunt-dependent hydrocephalus and its complications. Such discrepancies appear to be derived partly from inconsistent familiarity with existing literature but especially from a paucity of high-quality publications.

Diagnostic Difficulties in Slit Ventricle Syndrome

Slit ventricle syndrome is a rare complication of the treatment of hydrocephalus in infancy. Symptoms usually appear several years after the placing of the shunt system. They can cause diagnostic difficulties. The diagnosis is usually wrong or delayed. The authors describe a patient with SVS, the course of hospitalization and examinations performed.

Slit ventricle syndrome and subdural haemorrhage due to suspected overshunting in a dog with hydrocephalus internus

A 1.5-year-old Chihuahua was presented with clinical signs consistent with multifocal localisation. MRI revealed hydrocephalus internus. Following placement of a ventriculoperitoneal (VP) shunt with a low-pressure valve, improvement was noted....

Aqueductal stenosis in pediatric patients: An outcome analysis

Ventriculo-peritoneal (VP) shunt and endoscopic third ventriculostomy (ETV), both are well-established therapeutic modalities for the management of hydrocephalus. However, the treatment of choice is still controversial due to non-uniformity of the pre-existing data. It was a single institute retrospective cum prospective study enrolling 68 pediatric patients of congenital aqueductal stenosis (neonate to 18-year-old, mean age 48.2 ± 67.9 mo). The present study dealt with a uniform population of congenital aqueductal stenosis excluding secondary causes like post-infectious, post-hemorrhagic and tumor-related aqueductal stenosis. There were 54 patients in the VP shunt group and 14 in the ETV group. In the VP shunt group, there was no intra-operative or post-operative mortality and none had cerebrospinal fluid leak. Technical failure rate (shunt malfunction within 5 days of surgery) was 1.8% after primary shunting procedure and 4.5% after shunt revisions. Among 38 patients witha minimum available follow-up of 6m o (mean 34.5± 29.3 mo), the overall incidence of shunt obstruction after primary VP shunt was 36.8% out of which 21.1% blocked within a year. The shunt infection rate was 5.3%, which presented clinically within 3 mo of surgery. Two (5.3%) patients developed symptomatic slit ventricles and 13.2% patients had new-onset seizures. In the ETV group, there was one (7.1%) technical failure where the stoma could not be made due to severe bleeding. Two (14.2%) patients had intra-operative bleeding and the post-operative mortality was 7.1% (cause of death: ventriculitis). Follow-up ranging from 6-52 mo (mean 15.4 ± 13.6 mo) was available in 10 patients. Stoma blocked in 50% patients and all of them presented by around 1 mo period. Although precise statistical comparison could not be done due to small and disparate number of cases in the two groups, the data is still valuable in the Indian context. After ETV, there was no long-term morbidity whereas 61% patients of VP shunt continued to have some or other problem in the form of shunt obstruction, infection, new-onset seizures or slit ventricle syndrome. We conclude that depending on the expertise, ETV should be offered as the treatment of choice in congenital aqueductal stenosis because of its less frequent association with long-term morbidities.

Cranial vault expansion with the split bone flap technique in shunt-related craniosynostosis

Shunt-related craniosynostosis causing craniocerebral disproportion represents a particular complication of the treatment of hydrocephalus. When the modification of the shunt opening pressure does not improve the symptomatology, surgery for correction of craniocerebral disproportion is indicated. We present the results and advantages of the split bi-frontal bone technique that is a modification of the previous used frontal bone advancement technique. We retrospectively reviewed 5 patients with iatrogenic craniosynostosis treated in our institution from 1995 to 2012. A splitting bi-frontal bone flap that is left floating posteriorly was performed to increase the cranial volume. All patients were cured. This technique increases the volume of the skull favoring the expansion of the brain and allows good cosmetical results. No perioperative complications were reported. The fact that the brain has a more appropriate volume allows the disappearance of the clinical symptomatology related to the split ventricle syndrome. The advantage is the respect of the fronto-orbital shape in children and adults with the same results of the classic frontal advancement in terms of volume expansion. The study of cerebral blood flow confirms the increase of the cerebral blood perfusion and the improvement of the regional cerebral blood flow in borderline regions. We preconize, for the treatment of slit ventricle syndrome, the bi-frontal split technique in children and adults for its efficacy, the low rate of complications and the respect of aesthetical constraints.

Kyphectomy and Pedicular Screw Fixation with Posterior-Only Approach in Pediatric Patients with Myelomeningocele

Purpose: Defective posterior spinal arch and paraspinal musculature lead to progressive kyphosis in patients with myelomeningocele. Kyphosis decreases the patients' functional status and quality of life. To correct or prevent further deterioration, different surgical techniques have been introduced. Our aim is to present our clinical experience in kyphectomy and pedicle screw fixation with a posterior-only approach in pediatric patients with myelomeningocele and to discuss the technique with a review of the literature. Materials and Methods: Four patients with lumbar and 2 patients with thoracolumbar kyphosis (female:male ratio = 1:5) secondary to myelomeningocele were operated between January 2009 and October 2012. The median age was 5.5 years (range = 3-10 years). The criteria of the patient selection for the procedure were progression of kyphosis angle, impaired truncal balance and cosmetic deformity. In this retrospective study, we performed chart reviews for demographic and clinical data. We measured the pre- and postoperative kyphosis angles by using the Cobb method on lateral x-rays. Results: The mean preoperative kyphosis angle was 114.3° (range = 91-136°). The mean operative time was 171.7 min (range = 110-220 min). The mean intraoperative blood loss was 450 cc (range = 300-700 cc). The postoperative mean kyphosis angle was 28.2° (range = 13-33°). Five patients had skin breakdown. After osteofusion was established, those 5 patients' instrumentations were explanted. No acute or immediate postoperative complications occurred. Other complications were pneumonia and urinary tract infection. In the long term, 2 patients died due to pneumonia and slit-ventricle syndrome, respectively. Conclusions: Kyphectomy and pedicle screw instrumentation with the posterior-only approach dramatically reduces the kyphosis angle that develops in patients with myelomeningocele. The method itself is less time-consuming and leads to less intraoperative blood loss compared to other methods used for this patient population. Skin breakdown is the most common short-term complication.

Posterior cranial vault distraction osteogenesis: evolution of technique.

The rapid growth of the brain in the first few years of life drives the expansion of the cranial vault. This expansion occurs primarily at the cranial sutures; premature fusion of these results in growth restriction perpendicular to the axis of the suture. The result of this is physical deformation of the cranial and facial skeleton, as well as the distortion of the underling brain and its physiology. These patients can present with symptoms of raised intracranial pressure, neurodevelopmental delay, as well as the morphological features of craniosynostosis. Acquired conditions such as the slit ventricle syndrome may also result in cephalocranial disproportion with these clinical features. Traditional vault remodeling surgery is able to correct the physical abnormalities as well as correcting cephalocranial disproportion. Its limitations include the degree of scalp expansion achievable as well as resulting defects in the bone. The use of distraction osteogenesis of the cranial vault permits a controlled expansion in a predetermined vector in a gradual manner. When used in the calvarium, this combines the benefits of tissue expansion on the scalp, as well as stimulating the production of new bone, reducing the defects resulting from expansion. In this review, the authors describe some of the surgical considerations important to the use of this technique. This includes the relevant anatomy and technical aspects illustrated with the use of clinical cases. Finally, they present a summary of their experience and discuss the complications associated with cranial vault distraction osteogenesis.

Shunt dependency after cyst-peritoneal shunting for the arachnoid cyst of the temporal lobe: report of thirteen cases and literature review

Objective To investigate the mechanism,diagnosis,and treatment of shunt dependency syndrome in patients with temporal lobe arachnoid cysts who were initially treated with cystoperitoneal (CP) shunting.Methods Thirteen patients with temporal lobe arachnoid cysts who had initially been with CP shunts placement and had developed shunt dependency syndrome were treated by the authors at Tiantan Hospital between April 2010 and January 2012.The clinical manifestation,neuroimaging findings,intracranial pressure (ICP) data,treatment methods,and therapeutic results were reviewed retrospectively.Results The study included 10 males and 3 females.The mean age at the time of development of shunt dependency was 12.3 years (range 5.5-24.0years).In most patients,neuroimaging findings showed a collapsed cyst (the cyst appeared almost unchanged in only one patient) and normal or small ventricles (only one patient had enlarged ventricles).Three patients underwent simple replacement of the shunt,four underwent ventriculoperitoneal shunt placement,and the other six underwent lumboperitoneal shunt placement.All patients experienced resolution of their symptoms postoperatively.The mean duration of follow-up was 20 months.Conclusions Shunt dependency syndrome was a rare but serious complication of cystoperitoneal (CP) shunting for arachnoid cysts.The condition was similar to the slit ventricle syndrome,but also had some differences.ICP monitoring could confirm the diagnosis when there was no significant radiological findings. Key words: Arachnoid cyst ; Cyst-peritoneal shunting; Shunt dependency ; Mechanism

The truth and coherence behind the concept of overdrainage of cerebrospinal fluid in hydrocephalic patients

Overdrainage, siphoning, and slit-ventricle syndrome are well-documented complications of shunting in hydrocephalic patients. Despite the prevalence of these conditions, their mechanisms are still not fully understood. In this paper, the authors trace the concept of overdrainage and the related phenomena of siphoning and slit-ventricle syndrome. To provide a historical overview of overdrainage and to reignite discussion of a topic that has been settled. A medical literature search and review were performed via Google Scholar. Of 565 publications, 3 primary papers were identified and a timeline was developed demonstrating the convergence of the aforementioned concepts. From the primary papers, 25 relevant publications were selected and further analyzed searching for hypothesis, evidence, and conclusions. Overdrainage, siphoning, and slit-ventricle syndrome are associated concepts that have converged into a pathophysiological theory where siphoning of CSF leads to overdrainage, which is then hypothesized to cause slit-ventricle syndrome in a small subset of patients. Our data suggests that while there have been numerous reports regarding overdrainage and its consequences, the evidence is not as robust as currently presumed and this subject requires prospective exploration.

Slit ventricle syndrome and early-onset secondary craniosynostosis in an infant.

Patient: Female, 14 monthsFinal Diagnosis: Slit ventricle syndromeSymptoms: Hydrocephalus • lethargy and seizure • vomitingMedication: —Clinical Procedure: —Specialty: Pediatrics and NeonatologyObjective:Challenging differential diagnosisBackground:Shunt surgery is a common solution for hydrocephalus in infancy. Slit ventricle syndrome and secondary craniosynostosis are late-onset complications after shunt placement; these 2 conditions occasionally occur together.Case Report:We report a case of early-onset secondary craniosynostosis with slit ventricle syndrome after shunt surgery in an infant, which led to a catastrophic increase in intracranial pressure (ICP). A 4-month-old girl with a Dandy-Walker malformation underwent a ventriculoperitoneal shunt procedure. Her head circumference (HC) gradually decreased to approximately the 5th percentile for her age group after shunt surgery. Seven months later, she developed increased ICP symptoms and underwent a shunt revision with a diagnosis of shunt malfunction. Her symptoms were temporarily relieved, but she repeatedly visited the emergency room (ER) for the same symptoms and finally collapsed, with an abrupt increase in ICP, 3 months later. Further evaluation revealed the emergence of sagittal synostosis at 7 months after initial shunt surgery. After reviewing all clinical data, slit ventricle syndrome combined with secondary craniosynostosis was diagnosed. Emergent cranial expansion surgery with shunt revision was performed, and the increased ICP signs subsided in the following days.Conclusions:Clinical suspicion and long-term HC monitoring are important in the diagnosis of slit ventricle syndrome and secondary craniosynostosis after shunt surgery, even in infants and young children.