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      Approaches to ventriculoperitoneal shunt scalp erosion: countersinking into the calvarium. Illustrative case


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          Ventriculoperitoneal shunting (VPS) is a standard procedure for the treatment of hydrocephalus, and the management of its complications is common in the practice of pediatric neurosurgery. Shunt exposure, though a rare complication, can occur because of thin, fragile skin, a young patient age, protuberant hardware, poor scalp perfusion, and a multitude of other patient factors.


          The authors report a complex case of VPS erosion through the scalp in a young female with Pfeiffer syndrome treated with external ventricular drainage, empirical antibiotics, and reinternalization with countersinking of replaced shunt hardware into the calvarium to prevent internal skin pressure points, reduce wound tension, and allow wound healing.


          Recessing the shunt hardware, or countersinking the implant, into the calvarium is a simple technique often used in functional neurosurgical implantation surgeries, providing a safe surgical strategy to optimize wound healing in select cases in which the skin flap is unfavorable.

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          Risk factors for shunt malfunction in pediatric hydrocephalus: a multicenter prospective cohort study.

          OBJECT The rate of CSF shunt failure remains unacceptably high. The Hydrocephalus Clinical Research Network (HCRN) conducted a comprehensive prospective observational study of hydrocephalus management, the aim of which was to isolate specific risk factors for shunt failure. METHODS The study followed all first-time shunt insertions in children younger than 19 years at 6 HCRN centers. The HCRN Investigator Committee selected, a priori, 21 variables to be examined, including clinical, radiographic, and shunt design variables. Shunt failure was defined as shunt revision, subsequent endoscopic third ventriculostomy, or shunt infection. Important a priori-defined risk factors as well as those significant in univariate analyses were then tested for independence using multivariate Cox proportional hazard modeling. RESULTS A total of 1036 children underwent initial CSF shunt placement between April 2008 and December 2011. Of these, 344 patients experienced shunt failure, including 265 malfunctions and 79 infections. The mean and median length of follow-up for the entire cohort was 400 days and 264 days, respectively. The Cox model found that age younger than 6 months at first shunt placement (HR 1.6 [95% CI 1.1-2.1]), a cardiac comorbidity (HR 1.4 [95% CI 1.0-2.1]), and endoscopic placement (HR 1.9 [95% CI 1.2-2.9]) were independently associated with reduced shunt survival. The following had no independent associations with shunt survival: etiology, payer, center, valve design, valve programmability, the use of ultrasound or stereotactic guidance, and surgeon experience and volume. CONCLUSIONS This is the largest prospective study reported on children with CSF shunts for hydrocephalus. It confirms that a young age and the use of the endoscope are risk factors for first shunt failure and that valve type has no impact. A new risk factor-an existing cardiac comorbidity-was also associated with shunt failure.
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            Pfeiffer syndrome

            Pfeiffer syndrome is a rare autosomal dominantly inherited disorder that associates craniosynostosis, broad and deviated thumbs and big toes, and partial syndactyly on hands and feet. Hydrocephaly may be found occasionally, along with severe ocular proptosis, ankylosed elbows, abnormal viscera, and slow development. Based on the severity of the phenotype, Pfeiffer syndrome is divided into three clinical subtypes. Type 1 "classic" Pfeiffer syndrome involves individuals with mild manifestations including brachycephaly, midface hypoplasia and finger and toe abnormalities; it is associated with normal intelligence and generally good outcome. Type 2 consists of cloverleaf skull, extreme proptosis, finger and toe abnormalities, elbow ankylosis or synostosis, developmental delay and neurological complications. Type 3 is similar to type 2 but without a cloverleaf skull. Clinical overlap between the three types may occur. Pfeiffer syndrome affects about 1 in 100,000 individuals. The disorder can be caused by mutations in the fibroblast growth factor receptor genes FGFR-1 or FGFR-2. Pfeiffer syndrome can be diagnosed prenatally by sonography showing craniosynostosis, hypertelorism with proptosis, and broad thumb, or molecularly if it concerns a recurrence and the causative mutation was found. Molecular genetic testing is important to confirm the diagnosis. Management includes multiple-staged surgery of craniosynostosis. Midfacial surgery is performed to reduce the exophthalmos and the midfacial hypoplasia.
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              A multi-institutional, 5-year analysis of initial and multiple ventricular shunt revisions in children.

              To evaluate risk factors and predictors of cerebrospinal ventricular shunt revisions in children. A retrospective, longitudinal cohort of 1307 children ages 0 to 18 years undergoing initial ventricular shunt placement in the year 2000, with follow-up through 2005, from 32 freestanding children's hospitals within the Pediatric Health Information Systems database was studied. Rates of ventricular shunt revision were compared with patient demographic, clinical, and hospital characteristics with use of bivariate and multivariate regression accounting for hospital clustering. Thirty-seven percent of children required at least one shunt revision within 5 years of initial shunt placement; 20% of children required two or more revisions. Institutional rates of first shunt revision ranged from 20 to 70% of initial shunts placed among the 32 hospitals in the cohort. Hospitals where one to 20 initial shunt placements per year experienced the highest initial shunt revision rate (42%). Hospitals performing over 83 initial shunt placements per year experienced the lowest revision rate (22%). We found that children undergoing shunt placement in the Midwest were more likely to experience multiple shunt revisions (odds ratio, 1.25; 95% confidence interval, 1.06-1.47) after controlling for hospital volume, shunt type, age, and diagnosis associated with initial shunt placement. Higher hospital volume of initial shunt placement was associated with lower revision rates. Substantial hospital variation in the rates of ventricular shunt revision exists among children's hospitals. Future prospective studies are needed to examine the reasons for the variability in shunt revision rates among hospitals, including differences in specific processes of care.

                Author and article information

                J Neurosurg Case Lessons
                J Neurosurg Case Lessons
                J Neurosurg Case Lessons
                Journal of Neurosurgery: Case Lessons
                American Association of Neurological Surgeons
                27 November 2023
                27 November 2023
                : 6
                : 22
                : CASE23310
                [1 ]Department of Neurological Surgery, University of Illinois Chicago, Chicago, Illinois
                [2 ]Department of Neurosurgery, Division of Neurological Surgery, Ann & Robert H. Lurie Children’s Hospital, Northwestern University Feinberg School of Medicine, Chicago, Illinois
                [3 ]Division of Pediatric Neurosurgery, Departments of Neurosurgery and Public Health Sciences, University of Rochester School of Medicine and Dentistry, Rochester, New York; and
                [4 ]Division of Plastic & Reconstructive Surgery, Ann & Robert H. Lurie Children’s Hospital, Northwestern University Feinberg School of Medicine, Chicago, Illinois
                Author notes
                Correspondence Tord D. Alden: Ann & Robert H. Lurie Children’s Hospital, Chicago, IL. talden@ 123456luriechildrens.org .

                INCLUDE WHEN CITING Published November 27, 2023; DOI: 10.3171/CASE23310.

                Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

                © 2023 The authors

                CC BY-NC-ND 4.0 ( http://creativecommons.org/licenses/by-nc-nd/4.0/)

                : 12 June 2023
                : 20 July 2023
                Page count
                Figures: 4, Tables: 0, References: 14, Pages: 4
                Congenital, Congenital
                Craniofacial, Craniofacial
                Deformity, Deformity
                Hydrocephalus, Hydrocephalus
                Pediatric, Pediatric
                Technique, Technique
                Surgical-Technique, Surgical Technique
                Case Lesson

                hydrocephalus,scalp erosion,wound healing,ventriculoperitoneal shunt,countersink,recess,vps = ventriculoperitoneal shunt


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