Crouzon syndrome: posterior fossa volume studies in vestibular orientation.

INTRODUCTION: With the increasing possibilities of surgical treatment addressing the complex spectrum of defects in Crouzon syndrome (CS), it is of utmost importance to obtain accurate assessment of the malformation. Evaluating the volume of the posterior fossa is of great importance since many authors, considering that its volume is always decreased, favor posterior fossa enlargement as a first step in the treatment of CS. MATERIAL AND METHODS: We studied CT scans of children with CS, which were vestibular-oriented (VO) in order to conform with physiological landmarks, and we measured intracranial volume by manual segmentation; these abnormal CT scans were superposed with VO 3D-CT scanners of age-matched controls. We studied the volume index of the posterior fossa for each CS patient defined as the ratio of its volume in CS patients with the normal for age calculated from normal controls. RESULTS: We studied the imaging of 41 children with CS and 70 control. Among CS patients, the volume of the posterior fossa was increased in 10, compared with control. We found closure of the sphenoidal synchondrosis was correlated with age and with the width of the posterior fossa, but not with its length nor with the posterior fossa volume index. CONCLUSION: Segmentation on VO-CT scanner and superposition with CT scanners of normal controls is a powerful tool for the study of the impact of CS or other synostoses on volume and shape. We found that CS is more heterogeneous than previously thought, and surgical strategies should be adapted accordingly.

Brachmann-Cornelia de Lange syndrome with a papilloma of the choroid plexus: analyses of molecular genetic characteristics of the patient and the tumor. A single-case study.

PURPOSE: A 10-month-old girl with a Brachmann-Cornelia de Lange syndrome and a choroid plexus papilloma of the brain was studied at the Hospital Infantil de México Federico Gómez (HIMFG) in Mexico City. METHODS AND RESULTS: Presumptive papilloma of the third ventricle was evidenced on CT and MR images and removed. Pathological analysis confirmed its origin. A posterior radiosurgery was required due to a tumor relapse. Karyotypes (GTG bands) of the patient and her parents undertaken at HIMFG were normal. Array comparative genomic hybridization (array CGH) analyses of blood DNA of the patient and her parents carried out at BlueGnome's Laboratory in Cambridge, UK, set in evidence amplification of genes SPNS2, GGT6, SMTNL2, PELP1, MYBBP1A, and ALOX15 in chromosome 17p of the patient. Since MYBBP1A is a proto-oncogene and ALOX15 participates in the development of cancer and metastases of tumors, further fluorescent in situ hybridization (FISH) analyses of these two genes were implemented at HIMFG. Amplification of the two genes was found in the tumor of the case under study but not in an unrelated papilloma of the choroid plexus. DISCUSSION: Further analyses of the association of choroid plexus papillomas with disorders of psycho-neural development and its relationship to molecular genetic modifications at chromosome 17p are now under way at HIMFG.

Fifteen years of experience with the midfacial distraction without maxillary osteotomy protocol.

PURPOSE: Midfacial distraction for facial stenosis is minimizing the communication between cranial fossa and nasal fossa caused by the Le Fort III osteotomy during frontofacial advancement procedures. There are different types of distractors, such as internal and external devices. The aim of our study is to present a series of 22 consecutive distraction cases operated without any Le Fort osteotomy with external distraction frames. We completely avoid the gap between the skull and nose, thus avoiding related complications. PATIENTS AND METHODS: Between 1997 and 2012, we operated on 22 patients presenting syndromes associating midfacial retrusion, maxillomandibular class III malocclusion and upper airway obstruction. METHODS: We perform a fronto-orbital advancement. We do not perform any maxillary osteotomy. A vertical cut in the lateral orbital wall is done towards the inferior orbital fissure and another cut on the zygomatic arch. We realise the fixation of the frame posteriorly with a folded K-wire and anteriorly with a transmaxillary pin. Aiming overcorrection, we distract on average 1 mm a day for a mean period of 26 days and with a horizontal distraction vector. RESULTS: No deaths or life-threatening complications were reported. All midfacial retrusions were corrected without relapse. The advancement ranged between 6 and 20 mm. Several complications were notified: one sphenopetrous dislocation, one ethmoidonasal dislocation, two device disassemblages and two cases of maxillary sinusitis. Some of these complications caused an incomplete distraction result. CONCLUSIONS: Compared to other techniques, this method is safe, simple and efficient. By sparing major osteotomies, it avoids severe complications.

Trigonocephaly: Lille's surgical technique.

From the early beginning, in 1977, of our experience in the treatment of trigonocephaly and with the follow-up of 263 patients, we have been convinced that it was a condition related to the skull base. It is now proved thank to the comparison between patient's CT scan and normal template (normal skull of the same age) in vestibular orientation. We used to correct this condition by a frontal reshaping and a rotation of the superior and lateral orbital rim along an oblique axis. We call that movement: "valgisation."

Atypical scaphocephaly: a review.

BACKGROUND AND PURPOSE: Sagittal craniosynostosis (SCS) is common and easily recognized and corrected surgically. However, rare cases of SCS are more complex: these associate closure of the metopic or delayed closure of the coronal suture, uni- or bilaterally. MATERIAL AND METHODS: We reviewed the available literature on atypical sagittal craniosynostosis (ASCS). We also reviewed retrospectively our series of SCS treated since 1980 and selected cases with simultaneous closure of the metopic (leptocephaly) or delayed closure of other sutures (plagiocephaly, oxycephaly, or Crouzon syndrome). RESULTS: ASCS is rare, representing <10 % of SCS. In our series, among 447 cases of SCS followed for a mean duration of 63.7 months, we identified 22 cases of ASCS: 6 with leptocephaly, 9 with non-syndromic oxycephaly, 4 with Crouzon syndrome, and 3 with plagiocephaly. Fourteen patients required a second operation, either planned initially (severe leptocephaly) or because of brain compression. The actuarial incidence of ASCS requiring reoperation was 5.3 % of SCS at 10 years. After a mean follow-up of 113 months, morphological results in ASCS were grade 1 (no defect) in 5, grade 2 (mild defect) in 2, grade 3 (minor reoperation) in 3, and grade 4 (major reoperation) in 12; one patient had visual impairment, and two had learning difficulties. CONCLUSIONS: ACSC can be detected initially or occur with a delay in apparently standard SCS. Leptocephaly is a specific entity. Because of the implications on the management and risk for the patient, preoperative evaluation of patients with SCS with CT scanner and prolonged follow-up are necessary.

Non-syndromic oxycephaly and brachycephaly: a review.

BACKGROUND: Non-syndromic coronal synostoses oxycephaly and brachycephaly (NSCSOB) are rare. Their natural history, surgical management, and outcome are debated. MATERIAL AND METHODS: We reviewed the available literature on NSCSOB. In addition, we reviewed retrospectively our experience with cases of NSCSOB, managed and operated by our joint craniofacial team since 1984. Newborns underwent perifrontal craniectomy, while infants and older children underwent fronto-orbital advancement with frontoparietal remodeling. Cases with delayed onset of faciosynostosis were excluded. RESULTS: Some cases of NSCSOB present at birth or even prenatally with gross dysmorphism and severe brachycephaly; others present later in life with harmonious restriction of intracranial volume and are at risk for development and vision. Some NSCSOB evolve from initially unisutural synostoses. We treated 61 cases, operated aged 1.4 to 106.7 months, 30 with brachycephaly and 31 with oxycephaly. Twelve of these (19.7 %), had been treated initially for sagittal or unicoronal synostosis, and evolved into NSCSOB. Intracranial hypertension was present initially in 28 (45.9 %), with ophthalmological consequences in 13 (21.3 %) and mental retardation in 6 (9.6 %). No patient had hydrocephalus; only two had asymptomatic Chiari malformation. The mean postoperative follow-up was 113.6 months. At last control, 21 patients had developmental delay and 5 had visual impairment. CONCLUSIONS: NSCSOB are a complex entity. They have in common a high risk of severe intracranial hypertension and rare hydrodynamic complications. Genetic screening is necessary to assert their non-syndromic nature. Prolonged follow-up of all craniosynostoses is necessary because unisutural synostoses can evolve into NSCSOB, and NSCSOB can evolve into craniofaciosynostosis.

Unicoronal synostotic plagiocephaly: surgical correction: Lille's technique.

INTRODUCTION: For 35 years, we have a tight neuro-plastic surgical cooperation for the surgical correction and long-term systematic follow-up of 125 cases of unicoronal synostotic plagiocephaly. METHODS: We have tried to understand why some patients had kept an asymmetrical facial growth pattern in spite of a good fronto orbital correction. Analysis in vestibular orientation which was available from 1993 has demonstrated a discrepancy between the ocular and the vestibular verticality referential system. So we have designed a surgical procedure to try to fix that problem. RESULTS AND CONCLUSION: Preliminary results in 27 cases operated according to this procedure, for which we have pre- and post-CT scan demonstrate significative improvements of our results.

Midfacial distraction without osteotomy using a transfacial pin and external devices.

INTRODUCTION: During the 1970s, frontofacial advancement revolutionized the treatment of severe facial stenosis. Unfortunately, this method was associated with significant morbidity due to the Le Fort III osteotomy, which creates a major communication between the frontocranial dead space and the nasal fossae. Midfacial distraction improves the complication rate by diminishing the size of this gap. The aim of our study was to present an original technique that uses external distraction frames and eliminates the need for Le Fort osteotomies. This innovative technique eliminates the gap between the skull and nose, thus avoiding related complications. PATIENTS AND METHODS: Between 1997 and 2008, we operated on 17 patients presenting midfacial retrusion and maxillomandibular class III malocclusion. We performed classic fronto-orbital advancement. The only facial osteotomies are vertical cuts of both the lateral orbital wall and the zygomatic arch. The distraction device is then anchored posteriorly with a K-wire and anteriorly with a transfacial pin through the maxilla. Finally, the distraction is performed horizontally until a class II overcorrection is obtained. RESULTS: No life-threatening complications or mortalities occurred. In all cases, the midfacial retrusion was corrected without relapse. All patients with complications fully recovered. It was observed that most complications were a result of either an overly rapid activation (>1 mm/d). DISCUSSION: Midface distraction using the external transfacial pin is a simple and safe procedure that allows an efficient correction of major facial retrusion. The external transfacial pin acts directly onto the maxilla and allows distraction without Le Fort osteotomy. By eliminating major osteotomies, it reduces the number of severe complications encountered in craniofacial surgery.

Craniopharyngioma and hypothalamic obesity in children.

BACKGROUND AND PURPOSE: Obesity is a major concern in children treated for craniopharyngioma and is caused by hypothalamic damage. The role of aggressive surgical removal has been questioned, leading some authors to recommend a minimalist approach. In order to test this hypothesis, we decided to study obesity in craniopharyngioma and the factors related to it. MATERIALS AND METHODS: We reviewed retrospectively our series of pediatric craniopharyngiomas operated since 1981. The body-mass index (BMI) was calculated for each patient pre- and at several intervals postoperatively and expressed as standard deviations (SD) adjusted for age and gender. RESULTS: We operated on 45 cases, which were followed up for a mean duration of 11.0 years. Initial resection was total in 25 cases (55.6%). No patient died because of surgery or tumor progression; two died with delay presumably because of endocrine failure. At last control, 28 patients (62%) had obesity (BMI over +2SD). Hypothalamic involvement was significantly correlated with preoperative and postoperative BMI. Subtotal tumor resection was significantly associated with obesity at last control. Reoperation for tumor recurrence was associated with a significantly higher BMI. CONCLUSIONS: Our results suggest that obesity results from hypothalamic lesions caused by the tumor rather than by surgery. The postoperative weight gain appears to result from the continued impact of preoperative hypothalamic damage. The high rate of tumor recurrence in children, with the risk of additional damage to the hypothalamus, incites us to recommend total resection whenever it appears safe during initial surgery.

Neurocutaneous melanosis and the Dandy-Walker complex: an uncommon but not so insignificant association.

BACKGROUND: Neurocutaneous melanosis represents a rare congenital but nonheritable phakomatosis defined as the association of giant or multiple congenital nonmalignant melanocytic nevi with leptomeningeal melanosis or melanoma of the central nervous system. METHODS: We describe the case of an adolescent with a giant congenital bathing trunk melanocytic nevus who developed progressive intracranial hypertension due to leptomeningeal melanosis confirmed by surgical biopsy. Brain and spine magnetic resonance images showed posterior fossa malformation compatible with the Dandy-Walker complex, hydrocephalus, and extensive enhancement of posterior fossa then spine. Shunt placement, corticotherapy, and chemotherapy were attempted leading to transient relief but the boy died 12 months after the onset of primary neurological symptoms. DISCUSSION: We discuss diagnosis, pathogenesis, management, and prognosis in the light of data from the recent literature. CONCLUSION: Neurocutaneous melanosis is considered to follow from neurulation disorders which could account for associated developmental malformations as the so-called Dandy-Walker complex. Cutaneous lesions are usually recognized at birth whereas neurological manifestations develop later. Numerous neurological symptoms have been reported according to extent of leptomeningeal and parenchymal infiltration. Whether magnetic resonance imaging of the neuroaxis represents the choice radiological exam, definite diagnosis relies upon the histological data obtained by mean of biopsy. Once symptomatic, surgical and medical measures remain palliative since death occurs within 3 years.

Craniopharyngiomas in children: recurrence, reoperation and outcome.

INTRODUCTION: Craniopharyngioma bears a high rate of recurrence and morbidity in childhood. Although the outcome after recurrence and reoperation is an important parameter for the long-term evaluation of craniopharyngioma, it is poorly documented in literature. MATERIALS AND METHODS: We studied children reoperated for recurrent craniopharyngioma in our institution since the advent of computed tomography (CT) scanner. Reoperation for tumor resection was decided whenever the recurrence was solid, with the aim total resection if possible and reasonable. Stereotactic techniques were used in case of cystic recurrence, and external irradiation was used only in case of recurrent tumor not amenable to surgery. RESULTS: From September 1981 to January 2007, we performed one or more reoperations in 20 children presenting with recurrent craniopharyngiomas. The total number of reoperations was 29: resection was total in 12 cases, near-total in 8 cases, partial in 8 cases, and undocumented in 1. In addition, stereotactic procedures were performed in 11 cases, and 5 patients underwent external irradiation. DISCUSSION: No patient died after surgery or because of tumor progression; one patient died abruptly of an undiagnosed cause during external irradiation. The event-free survival after reoperation was 49.9% at 5 years and 40.0% at 10 years. At last control, after a mean follow-up of 70.4 months after the last surgery, nine patients were tumor-free and ten had stable disease. CONCLUSION: Reoperation for recurrent craniopharyngioma is an efficient method for tumor control and should be proposed whenever the recurrent tumor is solid. Morbidity results above all from tumor aggressiveness, rather than from surgical damage.

Disappearance of a middle cerebral artery aneurysm associated with Moyamoya syndrome after revascularization in a child: case report.

INTRODUCTION: Pediatric Moyamoya disease is rarely associated with intracranial aneurysms. We report a case of a 7-year-old girl with an antecedent of persistent craniopharyngeal canal, who presented with a history of choreiform movements. MATERIALS AND METHODS: A Moyamoya disease was found with an unruptured left middle cerebral artery aneurysm on her first angiography. Conservative treatment was chosen for the aneurysm and she underwent indirect revascularization by encephalosynangiosis using the multiple bur-hole technique for her Moyamoya disease. Abnormal movements were improved. Control angiogram at 6 months showed development of intracranial-extracranial anastomoses with complete resolution of the aneurysm. Aneuryms including the major arteries of the basal arterial circle occur as a by-product of the high velocity and blood flow secondary to the arterial stenosis. Blood flow modification after revascularization often lead to spontaneous regression and disappearance of these aneurysms. CONCLUSION: Therefore, a conservative treatment of these proximal aneurysms must be chosen after encephalosynangiosis.

Three-dimensional study of 31 cases of synostotic anterior plagiocephaly before and after surgical management the Lille protocol.

Synostotic anterior plagiocephaly is a rare pathological cranial malformation. Therapeutic options are rarely studied due to the rarity of the malformation and difficulties in diagnosis and care management. The objective of this study was to analyze the results obtained with the Lille protocol based on 62 CT-scans done before and after surgery in 31 patients. A specific analysis was designed for this work. Nine cephalometric measures enabled to evidence on each CT-Scan the corrections made on the fronto-orbital bandeau and the potential impact of surgery on the craniofacial structures. Results show that surgical symmetry of the fronto-orbital bandeau in the transversal plane, according to the symmetrical axis of the semicircular canals, allows a normalization of the skull's growth and morphogenesis for the surgically affected structures but also adjacent ones.

Facial diplegia revealing ventriculoperitoneal shunt failure in a patient with Crouzon syndrome. Case report.

The authors report on the case of a 15-year-old boy with Crouzon syndrome (CS) who presented with headache and facial diplegia. He had undergone several craniofacial interventions and a posterior fossa decompression for tonsillar herniation caused by the CS. A ventriculoperitoneal (VP) shunt had been inserted for hydrocephalus. Emergency computed tomography (CT) disclosed slight dilation of the ventricular cavities compared with their appearance on a baseline CT scan. Magnetic resonance imaging showed a deformed brainstem but no compression at the occipital foramen; there was no apparent explanation for the facial diplegia. The neuroophthalmological examination revealed neither papilledema nor oculomotor palsy. Electromyography confirmed incomplete peripheral facial diplegia. The patient underwent emergency shunt revision, during which complete obstruction of the ventricular catheter and severe cerebrospinal fluid hypertension were found. After surgery, cranial hypertension symptoms completely resolved and the facial diplegia improved slowly with a persistent and incomplete right superior facial palsy. Cranial 3D CT scanning reconstructions and brain magnetic resonance imaging demonstrated severe petrous bone distortion that could have been responsible for direct stretching injuries on the facial nerves at the level of the internal acoustic meatus. The present case represents the first reported occurrence of VP shunt failure as revealed by a facial palsy; the authors discuss the pathophysiology of facial palsy in intracranial hypertension.

Postmeningitis subdural fluid collection in infants: changing pattern and indications for surgery.

OBJECT: Postmeningitis subdural fluid collection (PMSFC) is a classic complication of bacterial meningitis in infants. When the diagnosis was based solely on subdural puncture (SDP), its incidence was estimated to be as high as one half of the cases of meningitis, with Haemophilus influenzae as the most common causative bacterium. Knowledge concerning the diagnostic and bacteriological characteristics of PMSFC has expanded greatly since the introduction of computerized imaging and the use of the anti-H. influenzae vaccine; however, in no recent study have the authors reappraised this clinical entity with regard to diagnosis, bacteriology, and indications for surgery. METHODS: The authors reviewed their cases of PMSFC in infants in which a diagnosis was made based on computerized tomography findings and confirmed with SDP. They treated PMSFC using placement of a subdural drain whenever the collection was either clinically eloquent or exerted a mass effect on the brain. In the 26 years preceding the study, the authors had treated 29 patients younger than 16 months of age for PMSFC. Eight patients required SDP only, 20 underwent surgical drainage, and five required craniotomy. In six cases, the fluid was grossly purulent; in the others, it was clear, xanthochromatic, or hemorrhagic. Cultures were positive for Streptococcus pneumoniae in only two cases. Although H. influenzae was the most common bacterium at the beginning of the series, Neisseria meningitidis has become more prevalent since vaccination against the former became widespread. Based on their data the authors estimate that 5% of N. meningitidis infections in infants are complicated by a significant PMSFC. CONCLUSIONS: At present, PMSFCs are most often caused by N. meningitidis. Temporary surgical drain placement is advised for all cases in which a significant mass effect is apparent on imaging.

Radical resection for craniopharyngiomas in children: surgical technique and clinical results.

Craniopharyngiomas are benign suprasellar tumors; however, their tendency to recur after resection and the risks associated with aggressive tumor resection pose a surgical dilemma. We reviewed our series of operated craniopharyngiomas and selected pediatric cases operated as first-hand cases and followed in our institution. We studied 37 cases. Resection was total in 65%. No patient died in the operative period; three patients died 6 months to 8 years after surgery of endocrine-related cause or sudden death. Tumor progression occurred in 93% of cases after subtotal resection versus 43% after total resection. Among 20 operations for recurrence, total resection was achieved in 45%. Visual deficits were often stabilized or improved after surgery, but worsened in 30% because of surgical damage or tumor recurrence. Fifty-nine percent of patients follow a normal school curriculum. All patients have some degree of pituitary hormonal replacement and 48% have obesity. Hypothalamic damage was generally associated with intraventricular extension of the tumor recurrence and re-operations, especially through combined approaches. Craniopharyngiomas in children are particularly aggressive tumors. Although the best oncological results are obtained with total resection, in some cases the functional price of surgery may be too high and alternative techniques should be proposed. The life-long management of these patients requires that the best use is made of all potential therapeutic tools.

Accidental and nonaccidental head injuries in infants: a prospective study.

OBJECT: Head injury is a major cause of morbidity and death in infants, and child abuse is among its chief causes. Retinal hemorrhages (RHs) are of paramount importance for the diagnosis of child abuse; however, their sensitivity and specificity are poorly estimated. Subdural hematoma (SDH) is a common feature; however, its incidence and causative factors are poorly documented. METHODS: To study the epidemiology of head injuries in infants, factors predisposing to SDH, value of RH for the diagnosis of child abuse, and prognostic factors, the authors prospectively collected data from cases of head injury in infants who were hospitalized at their institution over a 3-year period. One hundred fifty cases were collected, 57 of which were due to child abuse. This prospective study allows the comparison of clinical, radiological, and ophthalmological features in accidental and nonaccidental trauma in infants. CONCLUSIONS: Subdural hematomas were significantly correlated with RH and with child abuse but not with idiopathic macrocranium. The sensitivity and specificity of RH for the diagnosis of child abuse were 75 and 93.2%, respectively. Retinal hemorrhages associated with accidental trauma were always mild, and the specificity of more severe RH for the diagnosis of child abuse was 100%. The grading of RH requires the expertise of a trained neuroophthalmologist. Child abuse was also significantly associated with antecedents of perinatal illness, absence of signs of impact, and seizures on presentation. Although child abuse represented just 38% of traumas, it was the cause of 71% of deaths and 90% of severe disability in this series. Abuse and the clinical severity on presentation were two significant and independent factors conditioning outcome.

Resolution of acute acquired comitant esotropia after suboccipital decompression for Chiari I malformation.

PURPOSE: To report a case of acute comitant esotropia successfully treated with suboccipital decompression in a 9-year-old male patient with Chiari I malformation. DESIGN: Interventional case report. METHODS: A 9-year-old male with Chiari I malformation had acute onset of diplopia, headache, and comitant esotropia. RESULTS: About 9 months after suboccipital decompression, diplopia resolved and there was near orthophoria on examination 15 months after surgery. CONCLUSION: In view of our case and after a review of literature, we advocate primary suboccipital decompression to treat acute comitant esotropia in patients with Chiari I malformation. A follow-up period of at least 1 year rather than 6 months seems necessary to assess surgery effects.

MR imaging in the diagnosis of desmoplastic infantile tumor: retrospective study of six cases.

BACKGROUND AND PURPOSE: Desmoplastic infantile tumors (DITs) are rare supratentorial tumors of infancy with a favorable prognosis. Radiologic and histologic features of DIT are misleading, and DIT may be misinterpreted as a malignant lesion. We have studied the usefulness of MR imaging in the diagnosis of these tumors. METHODS: Between 1995 and 2002, six DITs were diagnosed in young children at our institution. Neuroimaging, age at diagnosis, sex, clinical presentation, symptoms duration, follow-up, and development were studied retrospectively. Contrast-enhanced CT and MR images were available. MR study included T1-, T2-, and postgadolinium T1-weighted sequences in the axial, sagittal, and coronal planes. RESULTS: These tumors were massive and predominantly cystic, with preferential frontal and parietal involvement. Typically, a DIT appears as a hypointense cystic mass with an isointense peripheral solid component on T1-weighted MR images. The peripheral solid component enhances after gadolinium administration. On T2-weighted MR images, the cystic component is hyperintense and the solid portion isointense or heterogeneous. The cystic portion is usually located deep inside the lesion, whereas its solid portion is peripheral. Meningeal enhancement and thickening adjacent to the solid portion of the tumor, calcifications, bone abnormalities adjacent to the tumor consisting of thinning and deformation were noted in 50% of our cases. Edema was usually absent or moderate. Median follow-up was 32 months, and no recurrence was noted except for one atypical case with incomplete excision, which led to the patient's death. CONCLUSION: Despite their malignant appearance, MR imaging features of DIT may help in the diagnosis and obviate unnecessary chemotherapy or radiation therapy.

Shunt revision for asymptomatic failure: surgical and clinical results.

OBJECTIVE: Shunt obstruction represents a permanent threat for patients with shunts, and its prevention and treatment are important parts of neurosurgeons' duties. Although there is little discussion regarding the need to reoperate for treatment of symptomatic shunt failure (SSF), the need to reoperate for treatment of asymptomatic shunt failure (ASF) is debated, as are the guidelines for the follow-up monitoring of patients with shunts. The goal of this study was to assess the effects of systematic follow-up monitoring and shunt revision for ASF; we reviewed our database to compare the results of shunt revision for ASF versus SSF. METHODS: We defined ASF as shunt failure diagnosed for an asymptomatic patient during a systematic consultation. In our institution, children who receive shunts for treatment of hydrocephalus are systematically monitored in an outpatient clinic, with clinical examinations and plain x-rays. Among 1,564 children with shunts, who were monitored for a mean of 10.7 years, 1106 (70.7%) required at least one shunt revision. The indication for the first revision was SSF in 609 cases and ASF in 305 cases; the indication was not specified in 192 cases. We studied the surgical outcomes after the first shunt revision and compared the results for SSF and ASF. RESULTS: After the first revision, shunt infections and subsequent shunt failure were significantly less frequent in the ASF group, compared with the SSF group. The interval between the first shunt revision and subsequent shunt failure was significantly longer in the ASF group. CONCLUSION: Our data support the practice of systematic follow-up monitoring for patients with shunts, for the early diagnosis and systematic treatment of ASF.