Reassessing the association: Evaluation of a polyalanine deletion variant of RUNX2 in non-syndromic sagittal and metopic craniosynostosis.

The RUNT-related transcription factor RUNX2 plays a critical role in osteoblast differentiation, and alterations to gene dosage cause distinct craniofacial anomalies. Uniquely amongst the RUNT-related family, vertebrate RUNX2 encodes a polyglutamine/polyalanine repeat (Gln23-Glu-Ala17 in humans), with the length of the polyalanine component completely conserved in great apes. Surprisingly, a frequent 6-amino acid deletion polymorphism, p.(Ala84_Ala89)del, occurs in humans (termed 11A allele), and a previous association study (Cuellar et al. Bone 137:115395;2020) reported that the 11A variant was significantly more frequent in non-syndromic sagittal craniosynostosis (nsSag; allele frequency [AF] = 0.156; 95% confidence interval [CI] 0.126-0.189) compared to non-syndromic metopic craniosynostosis (nsMet; AF = 0.068; 95% CI 0.045-0.098). However, the gnomAD v.2.1.1 control population used by Cuellar et al. did not display Hardy-Weinberg equilibrium, hampering interpretation. To re-examine this association, we genotyped the RUNX2 11A polymorphism in 225 individuals with sporadic nsSag as parent-child trios and 164 singletons with sporadic nsMet, restricting our analysis to individuals of European ancestry. We compared observed allele frequencies to the non-transmitted alleles in the parent-child trios, and to the genome sequencing data from gnomAD v.4, which display Hardy-Weinberg equilibrium. Observed AFs (and 95% CI) were 0.076 (0.053-0.104) in nsSag and 0.082 (0.055-0.118) in nsMet, compared with 0.062 (0.042-0.089) in non-transmitted parental alleles and 0.065 (0.063-0.067) in gnomAD v.4.0.0 non-Finnish European control genomes. In summary, we observed a non-significant excess, compared to gnomAD data, of 11A alleles in both nsSag (relative risk 1.18, 95% CI 0.83-1.67) and nsMet (relative risk 1.29, 95% CI 0.87-1.92), but we did not replicate the much higher excess of RUNX2 11A alleles in nsSag previously reported (p = 0.0001).

Speech and Language Development, Hearing, and Feeding in Patients With Genetically Confirmed Crouzon Syndrome With Acanthosis Nigricans: A 36-Year Longitudinal Retrospective Review of Patients at the Oxford Craniofacial Unit.

OBJECTIVE: Crouzon syndrome with acanthosis nigricans (CAN) is caused by the specific mutation c.1172C>A (p.Ala391Glu) in the fibroblast growth factor receptor 3 gene, and has an estimated prevalence of 1:1,000,000 births. Most cases occur de novo; however, autosomal dominant inheritance may occur. The clinical presentation typically includes craniosynostosis, midface and maxillary hypoplasia, choanal atresia/stenosis, hydrocephalus, and intracranial hypertension. Patients develop acanthosis nigricans, a hyperkeratotic skin disorder. The authors present the first known study to investigate the speech, language, hearing, and feeding of patients with CAN. METHODS: A retrospective case-note review of patients with a genetically confirmed diagnosis of CAN attending the Oxford Craniofacial Unit during a 36-year period (1987-2023) was undertaken. RESULTS: Participants were 6 patients with genetically-confirmed CAN (5 females, 1 male), all cases arose de novo. All patients had craniosynostosis (n = 5/6 multisuture synostosis, n = 1/6 left unicoronal synostosis). Hydrocephalus was managed through ventriculoperitoneal shunt in 67% (n = 4/6) of patients, and 67% (n = 4/6) had a Chiari 1 malformation. Patients had a complex, multifactorial feeding history complicated by choanal atresia/stenosis (100%; n = 6/6), and significant midface hypoplasia. All patients required airway management through tracheostomy (83%; n = 5/6); and/or continuous positive airway pressure (67%; n = 4/6). All patients underwent adenotonsillectomy (100%; n = 6/6). Initial failure to thrive, low weight, and/or height were seen in 100% (n = 6/6) patients; 80% (n = 4/5) had reflux; 100% (n = 6/6) had nasogastric, or percutaneous endoscopic gastrostomy based feeding during their treatment journey. All patients had hearing loss (100%; n = 6/6). Early communication difficulties were common: receptive language disorder (50%; n = 3/6); expressive language disorder (50%; n = 3/6); and speech sound disorder in 50% (n = 3/6)-necessitating the use of Makaton in 80% of patients (n = 3/5). CONCLUSIONS: Patients with CAN experience significant respiratory, neurological, and structural obstacles to hearing, speech, language, and feeding. The authors present a recommended pathway for management to support patients in these domains.

Social Media and Website Use: The Experiences of Parents and Carers Accessing Care at the Oxford Craniofacial Unit.

INTRODUCTION: Historically, medical professionals have been the providers of specialist information about rare medical conditions. Now, increasingly, patients and the public are using the internet to access and generate information about medical diagnoses. The global nature of the internet allows patients to connect across geographical borders, and to obtain and share information that would have been previously inaccessible to them. This research investigated the use of website and social media by parents of children with craniosynostosis. METHODS: A cross-sectional survey-based design was employed. Participants were parents of children with craniosynostosis attending multidisciplinary craniofacial clinics within the Oxford Craniofacial Unit. A questionnaire was administered which assessed social media and website use adapted from the questionnaire created by Khouri and colleagues (2016) and Huggons and colleagues (2019). The surveys were administered over an 18-month period (November 2020 to May 2022). RESULTS: The final sample comprised 82 parents [70 mothers; 10 fathers; 1 sister/carer and 1 parent (mother/father unspecified)]. The children were aged 11 months-16 years of age (average age 6 y and 3 mo). Children had a variety of diagnoses: 31 sagittal, 19 metopic, 14 syndromic craniosynostosis, 8 unicoronal, 6 multisuture, 3 bicoronal, and 1 unilambdoid.Results showed that 93% (n=76/82) of parents used the internet to find out more about craniosynostosis, with 72% (n=59/82) of parents specifically using social media to find out more about craniosynostosis. The social media platforms used included: Facebook 64% (n=53/82), Instagram 24% (n=20/82), Blogs 12% (n=10/82), Twitter 4% (n= 4/82), Tik Tok 2% (n=2/82), and Snapchat 0.01% (n=1/82).Parents reported that Facebook was the most helpful source of information about craniosynostosis (52%; n=43/82). Parents indicated the key timepoints they used social media included: when their child received a diagnosis (70%; n=58/82), before their child's surgery (34%; n=28/82), before their first craniofacial clinic appointment (83%; n=25/30), and when child was older (17%; n=14/84). Forty percent (n=33/82) of parents said that a diagnosis of craniosynostosis made no difference to their social media use, whereas 34% (n=28/82) of parents used social media more, and 20% (n=16/82) used it less. CONCLUSION: Results highlight that parents use social media and other websites to access information relating to craniosynostosis. Future research should examine whether parental use of social media changes across their child's lifespan and evaluate the quality of this information.

Craniofacial Surgery-Related Hashtag Utilisation on Instagram.

BACKGROUND: Patients, parents, and carers are increasingly using social media to access and contribute to health information. There are currently 3.484 billion active social media users. Instagram is a primarily visual social media platform for sharing photographs and videos with currently over one billion monthly active users. Limited research has investigated the use of Instagram in healthcare contexts including plastic surgery, and in the treatment of specific conditions, such as fibromyalgia and scoliosis. This study aimed to investigate the use of Instagram in relation to craniofacial surgery. METHODS: The authors investigated the use of 12 Instagram craniofacial surgery-related hashtags. Quantitative and qualitative data were collected for each of the "top" nine posts associated with each hashtag. Duplicate posts, posts not in English and those not relevant to craniofacial surgery were excluded.Thematic analysis was then used to qualitatively evaluate the captions and comments of each of the nine "top" posts associated with each hashtag. RESULTS: A total number of 151,738 posts were identified using the 12 hashtags queried in the present study, with #craniofacial being the most popular hashtag (87% of posts). Parents (n = 25, 33%) and surgeons (n = 25, 33%) were responsible for the majority of posts. A heterogenous group were responsible for the remaining 34% of posts.Results indicated that parents and surgeons posted significantly different images (chi-squared with Yates correlation is 25.0519, P  < 0.00001). Parents' posts were predominantly images of their child/patients (n = 24) compared to posts about patients by surgeons (n = 6). Posts by surgeons were predominantly images of professionals (n = 14) compared to images of professionals posted by parents (n = 1).Results of qualitative analysis of captions of posts indicated a significant difference in themes that emerged between parents and surgeons; with surgeons largely using Instagram for information giving, and parents for sharing of experiences (chi-square (n = 108) = 40.83, P  < 0.00001).There was a significant difference (two-tailed t test, P  = 0.002) in engagement (measured by the number of likes on posts) on posts by parents (mean = 3778) compared to posts by surgeons (mean = 135.4). CONCLUSIONS: Results indicate that craniofacial-surgery related posts on Instagram provide a medium to allow individuals and families with rare disorders to connect and share their experiences. The use of Instagram to share information about craniofacial abnormalities via Instagram has the potential to be used by healthcare professionals to educate and support patients and families.

Randomized Pilot Study: Anal Inserts Versus Percutaneous Tibial Nerve Stimulation in Patients With Fecal Incontinence.

BACKGROUND: Anal inserts and percutaneous tibial nerve stimulation may be offered to those with fecal incontinence in whom other conservative treatments have failed. OBJECTIVE: We aimed to compare anal inserts and percutaneous tibial nerve stimulation. DESIGN: This was an investigator-blinded randomized pilot study. SETTINGS: The study was conducted at a large tertiary care hospital. PATIENTS: Adult patients with passive or mixed fecal incontinence were recruited. INTERVENTIONS: Patients were randomly assigned to receive either the anal inserts or weekly percutaneous tibial nerve stimulation for a period of 3 months. MAIN OUTCOME MEASURES: The primary end point was a 50% reduction of episodes of fecal incontinence per week as calculated by a prospectively completed 2-week bowel diary. Secondary end points were St Mark's incontinence score, International Consultation on Incontinence Questionnaire-Bowel scores (for bowel pattern, bowel control, and quality of life), use of antidiarrheal agents, estimates of comfort and acceptability. RESULTS: Fifty patients were recruited: 25 were randomly assigned to anal inserts and 25 were randomly assigned to percutaneous tibial nerve stimulation. All completed treatment. A significant improvement of scores in the 2-week bowel diary, the St Mark's scores and the International Consultation on Incontinence Questionnaire-Bowel scores, was seen in both groups after 3 months of treatment. A reduction of ≥50% fecal incontinence episodes was reached by 76% (n = 19/25) by the anal insert group, compared with 48% (n = 12/25) of those in the percutaneous tibial nerve stimulation group (p = 0.04). The St Mark's fecal incontinence scores and the International Consultation on Incontinence Questionnaire-Bowel scores for bowel pattern, bowel control, and quality of life (p = 0.01) suggest similar improvement for each group. LIMITATIONS: A realistic sample size calculation could not be performed because of the paucity of objective prospective studies assessing the effect of the insert device and percutaneous tibial nerve stimulation. CONCLUSIONS: Both anal insert and percutaneous tibial nerve stimulation improved the symptoms of fecal incontinence after 3 months of treatment. The insert device appeared to be more effective than percutaneous tibial nerve stimulation. Larger studies are needed to investigate this further. See Video Abstract at http://links.lww.com/DCR/B460. TRIAL REGISTRATION NUMBER: Clinicaltrials.gov No. NCT04273009. ESTUDIO PILOTO ALEATORIZADO DE INSERCIONES ANALES CONTRA LA ESTIMULACIN PERCUTNEA DEL NERVIO TIBIAL EN PACIENTES CON INCONTINENCIA FECAL: ANTECEDENTES:Las inserciones anales y la estimulación percutánea del nervio tibial (PTNS) se pueden ofrecer a las personas con incontinencia fecal que han fallado en otros tratamientos conservadores.OBJETIVO:Nuestro objetivo fue comparar inserciones anales y estimulación percutánea del nervio tibial.DISEÑO:Este fue un estudio piloto aleatorio ciego para investigadores.AJUSTE:El estudio se realizó en un hospital de atención terciaria.PACIENTES:Se reclutaron pacientes adultos con incontinencia fecal pasiva o mixta.INTERVENCIONES:Los pacientes fueron asignados al azar para recibir inserciones anales o estimulación del nervio tibial percutáneo semanal durante un período de tres meses.PRINCIPALES MEDIDAS DE RESULTADO:El principal resultado fue una reducción del 50% de los episodios de incontinencia fecal por semana, según lo calculado mediante un diario intestinal de dos semanas completado de forma prospectiva. Los criterios de valoración secundarios fueron la puntuación de incontinencia de St Mark, las puntuaciones del ICIQ-B (para patrón intestinal, control intestinal y calidad de vida), uso de agentes antidiarreicos, estimaciones de comodidad y aceptabilidad.RESULTADOS:Se reclutaron 50 pacientes: 25 fueron asignados al azar a inserciones anales y 25 a PTNS. Todo el tratamiento completado. Se observó una mejora significativa de las puntuaciones en el diario intestinal de dos semanas, la puntuación de St Mark y la puntuación del ICIQ-B en ambos grupos después de 3 meses de tratamiento. Se alcanzó una reducción de ≥ 50% de los episodios de incontinencia fecal en un 76% (n = 19/25) en el grupo de inserción anal, en comparación con el 48% (n = 12/25) de los del grupo de estimulación percutánea del nervio tibial (p = 0,04). Las puntuaciones de incontinencia fecal de St Mark, las puntuaciones del ICIQ-B para el patrón intestinal, el control intestinal y la calidad de vida (p = 0,01) sugieren una mejora similar para cada grupo.LIMITACIONES:No se pudo realizar un cálculo realista del tamaño de la muestra debido a la escasez de estudios prospectivos objetivos que evaluaran el efecto del dispositivo de inserción y la estimulación percutánea del nervio tibial.CONCLUSIONES:Tanto la inserción anal como la estimulación percutánea del nervio tibial mejoraron los síntomas de incontinencia fecal después de 3 meses de tratamiento. El dispositivo de inserción parecia ser más efectivo que la estimulación percutánea del nervio tibial. Se necesitan estudios más amplios para investigar esto más a fondo. Consulte Video Resumen en http://links.lww.com/DCR/B460.NÚMERO DE REGISTRO DE PRUEBA:Clinicaltrials.gov No. NCT04273009.

Multisuture Craniosynostosis and Papilledema in Pycnodysostosis: A Paradox?

Pycnodysostosis (PYCD) is a rare autosomal-recessive skeletal disorder that typically presents with osteosclerosis of the majority of the postcranial skeleton and osteolysis of the calvarium, manifesting as persistent open cranial fontanelles and widely spaced cranial sutures. Craniosynsostosis in PYCD is a somewhat paradoxical feature, and has only been rarely reported. The authors present a unique case of a 6-year-old girl with PYCD, multisuture craniosynostosis involving the coronal and sagittal sutures, severe obstructive sleep apnoea, and raised intracranial pressure presenting as papilledema. She underwent a frontofacial monobloc distraction advancement which successfully corrected her papilledema and obstructive sleep apnoea.Pycnodysostosis is caused by a loss of function mutation in the CTSK gene that codes for the lysosomal cysteine protease, cathepsin K (CTSK). Loss of CTSK impairs the ability of osteoclasts to degrade bone extracellular matrix. Differences in osteoclast phenotype and extracellular matrix composition between membranous and cartilaginous bone may explain the clinical features of PYCD. Animal model studies suggest that craniosynostosis may arise due to variations in patient genetic background.

Evaluation of 153 Asymptomatic Subjects Using the Anopress Portable Anal Manometry Device.

BACKGROUND/AIMS: The Anopress device is a new portable manometry system. The aim of this study is to formulate normative data using this new device by recording the anorectal function of asymptomatic subjects. Patient comfort was also assessed. METHODS: Anorectal function was assessed in asymptomatic volunteers using the Anopress. All volunteers were examined in a standardized way in accordance with the study protocol. Normative values for the Anopress were obtained from the recorded data and patient comfort was assessed using a visual analogue scale. RESULTS: We recruited 153 healthy volunteers. Eighty were female (23 parous; median age 39.5 [interquartile range {IQR}, 28.75-53.00]) and 73 were male (median age 40.5 [IQR, 29.00-52.25]). For the female cohort, the following normal range (2.5-97.5 percentile) values were recorded across the whole anal canal: resting pressure 40.0-103.0 mmHg; squeeze increment 35.0-140.6 mmHg; endurance 1.3-9.0 seconds; involuntary squeeze 41.1-120.8 mmHg; and strain pressure 22.1-77.9 mmHg. Similarly, the following male normal range (2.5-97.5 percentile) values were recorded across the whole anal canal: resting pressure 38.3-99.6 mmHg; squeeze increment 42.5-154.8 mmHg; involuntary squeeze 40.0-123.6 mmHg; endurance 2.0-10.0 seconds; and strain pressure 11.0-72.1 mmHg. The median visual analogue scale scores for discomfort during the measurement was 0.0 (IQR, 0.00-0.00). CONCLUSIONS: Normative values for the Anopress device have been calculated by this study. The Anopress appears to be a safe and well tolerated way of measuring pressures from the entire anal canal. Further comparisons with other standard and commonly used manometry tests are, however, required to verify its reliability.

Pilot study of immunological factors in non-inflammatory bowel disease enterocutaneous fistulas.

BACKGROUND: Tumour necrosis factor alpha (TNF-α) is a cytokine elevated in inflammatory bowel disease enterocutaneous fistula (IBD ECF). Dendritic cells are antigen presenting cells that orchestrate the immune responses and regulate the production of cytokines by immune cells including T cells. No study to date has assessed the level of TNF-α or the presence of dendritic cells in non-IBD ECF. The aim of this study was to assess the inflammatory activity, with a particular emphasis on TNF-α in non-IBD ECF when compared with control small bowel tissue. METHODS: Tissue biopsies were obtained from ECF at operation from non-IBD patients and from terminal ileum in normal colonoscopy control patients. After overnight culture, accumulation of intracellular TNF-α was measured by flow cytometry in cells treated with monensin to assess the on-going cytokine production. Data were acquired using FACS Canto II. Unpaired Student's t-test was used to compare variables between groups and p < 0.05 was regarded as significant. RESULTS: The on-going production of TNF-α from dendritic cells (p = 0.0007), putative monocyte and B cell populations (p = 0.04) and CD3+ T cells (p = 0.04) was significantly higher in non-IBD ECF tissue than that from control tissue. CONCLUSIONS: This study reveals results which provide evidence for the potential use of anti-TNF-α agents in the treatment of non-IBD ECF. A pilot study to evaluate this treatment as an alternative option in an already surgically challenging group of patients is planned. Positive findings would be a major medical advance with a new use for anti-TNF-α agents.

The Significance of Squamosal Suture Synostosis.

The squamosal suture is one of the lateral minor skull sutures, separating the parietal and squamous temporal bones. While the phenotypic appearances and sequelae of synostosis of the major cranial vault sutures are well documented, little is reported concerning synostosis of the squamosal suture (SQS). The aim of this study was to determine the frequency of squamosal suture synostosis, and to document the significance of this entity.A retrospective review of the diagnostic imaging for all new pediatric patients (aged ≤16 years) referred to the Oxford Craniofacial Unit between January 2008 and February 2013 was completed to identify patients with SQS. Computed tomography (CT) imaging was available in 422 patients and the axial and three-dimensional reconstructed images reviewed.Squamosal suture synostosis was confirmed in 38 patients (9%). It was present in conjunction with major suture synostosis in 33 patients and in isolation in 5. The incidence increased with age. It was more common in patients with syndromic craniosynostosis (18%) and associated syndromic conditions (36%) than in those with isolated major suture synostosis (6%). It was found to occur with coronal, lambdoid, and sagittal synostosis, but was most frequent with multisuture fusion patterns. Squamosal suture synostosis was not associated with a consistent calvarial deformity either in isolation or when associated with a major suture fusion. No patient underwent surgery specifically to correct SQS.In conclusion, contrary to previous reports, squamosal suture synostosis is a relatively frequent finding in the general case mix of a typical craniofacial unit, but is of limited clinical significance.

Transcutaneous Sacral Electrical Stimulation for Chronic Functional Constipation.

BACKGROUND: Transcutaneous sacral nerve stimulation is reported to improve symptoms of fecal incontinence. Chronic constipation may also respond to stimulation, but this is poorly reported in the literature. OBJECTIVE: The study assessed the efficacy of transcutaneous electrical stimulation directly over the sacral nerve roots in chronic constipation. PATIENTS: Chronic functional constipation was established in all patients using the Rome III criteria. SETTING: The therapy was self-administered at home. DESIGN/INTERVENTION: A pilot study was conducted of transcutaneous sacral stimulation given over a 4-week period for 12 hours a day. MAIN OUTCOME MEASURES: Patients were assessed using the Patient Assessment of Constipation Symptoms, the Patient Assessment of Constipation Quality of Life, and the Cleveland constipation tool. A Global Rating of Change measure and a 1-week bowel diary was kept for the final week and compared with baseline. RESULTS: Of the 20 patients recruited (16 female, median age 38.5 years), 80% (16) completed the trial. Five (31%) patients reported at least a point reduction in the Patient Assessment of Constipation Symptoms score, 4 (25%) deteriorated, and 7 (44%) improved by less than one point. Median (interquartile range) Patient Assessment of Constipation Symptoms scores were 2.33 (2.34) at baseline and 2.08 (2.58) at follow-up (p = 0.074). Median scores for the Patient Assessment of Constipation Quality of Life and Cleveland systems were 3.00 (1.64) and 17.15 (18) at baseline and 2.22 (3.04) and 15.31 (12) at follow-up (p = 0.096 and 0.111). One-third of patients reported a positive Global Rating of Change measure, although 68% required concurrent laxatives during the trial. LIMITATIONS: This is a pilot study and is limited by its small sample size. CONCLUSIONS: Continuous transcutaneous sacral stimulation in the short term appears to be ineffective for chronic constipation. Larger well-powered studies with intermittent stimulation regimens are required to investigate this further.

Long-term morphological outcomes in nonsyndromic sagittal craniosynostosis: a comparison of 2 techniques.

Correction of scaphocephaly is one of the principle goals of surgery in sagittal craniosynostosis. Reported relapse in head shape after surgery and continued head growth into late adolescence underscores the need for long-term outcomes to be considered when comparing between different surgical approaches in this condition; yet there are relatively few reports of results to 5 years and beyond in the literature. Therefore, a retrospective review was performed of the anthropometric data of 224 patients with sagittal craniosynostosis who underwent primary surgery between 1994 and 2012. During this period, patients underwent either a modified strip craniectomy (MSC) or calvarial remodeling (CR) procedure. Sixty-two patients were treated by MSC and followed up for a mean of 44 months. One hundred sixty-two patients had CR, with follow-up for a mean of 45 months. Overall, 90 patients were seen up to 5 years, and 47 patients to 9 years or more after surgery. The cephalic index (CI) of MSC-treated patients improved from a mean of 67.0 to 72.7, with 31% achieving a CI greater than 75 at one year. Calvarial remodeling was significantly more effective at correcting the scaphocephalic deformity. Patients treated with CR improved from a mean CI of 66.7 to 76.1. Sixty-two percent of the patients achieved a CI greater than 75. In both groups, outcomes were stable throughout follow-up with no significant relapse up to 14 years after surgery.

The incidence of raised intracranial pressure in nonsyndromic sagittal craniosynostosis following primary surgery.

OBJECT Raised intracranial pressure (ICP) is recognized to occur in patients with nonsyndromic isolated sagittal craniosynostosis (SC) prior to surgery. However, the incidence of raised ICP following primary surgery is rarely reported and there appears to be a widely held assumption that corrective surgery for SC prevents the later development of intracranial hypertension. This study reports the incidence of postoperative raised ICP in a large cohort of patients with SC treated by 1 of 2 surgical procedures in a single craniofacial unit. METHODS A retrospective review was performed of all patients with SC who underwent either a modified strip craniectomy (MSC) or calvarial remodeling (CR) procedure under the care of the Oxford Craniofacial Unit between 1995 and 2010 and who were followed up for more than 2 years. The influence of patient age at surgery, year of surgery, sex, procedure type, and the presence of raised ICP preoperatively were analyzed. RESULTS Two hundred seventeen children had primary surgery for SC and were followed up for a mean of 86 months. The overall rate of raised ICP following surgery was 6.9%, occurring at a mean of 51 months after the primary surgical procedure. Raised ICP was significantly more common in those patients treated by MSC (13 of 89 patients, 14.6%) than CR (2 of 128 patients, 1.6%). Also, raised ICP was more common in patients under 1 year of age, the majority of whom were treated by MCS. No other factor was found to have a significant effect. CONCLUSIONS Postoperative raised ICP was found in more than 1 in 20 children treated for nonsyndromic SC in this series. It was significantly influenced by the primary surgical procedure and age at primary surgery. Careful long-term follow-up is essential if children who develop raised ICP following surgery are not to be overlooked.