Three-dimensional quantification of soft tissue changes and its relationship to skeletal changes after Le Fort III, monobloc, and facial bipartition in syndromic craniosynostosis.

To determine the effect of midface surgery on soft tissue changes and their relationship to hard tissue changes in patients with syndromic craniosynostosis. A retrospective analysis of patients who had undergone Le Fort III (LFIII), monobloc (MB), or facial bipartition (FB) was conducted. A 3D soft tissue mesh was generated from the preoperative scan and registered to the postoperative scan, after which the advancement was visualised. A total of 68 patients were included: 28 had undergone LFIII, 27 MB, and 13 FB. The included diagnoses were Apert (n = 23), Crouzon (n = 34), and craniofrontonasal syndrome (n = 11). After LFIII, most soft tissue advancement was seen around subnasale and pronasale (mean 15.1 ± 5.9 mm and 14.7 ± 5.7 mm, at age 7-12 years). After MB, a greater hard tissue than soft tissue advancement was seen for most landmarks, showing a high positive correlation. In patients undergoing FB without distraction (n = 10), mean preoperative inter-canthal distance was 48.9 mm, this reduced by 6.9 mm postoperatively. This study provides a comprehensive overview of the outcomes after midface surgery using 3D quantification for a better understanding of the soft tissue changes and their relationship to hard tissue changes.

Skeletal changes after midface surgery in patients with craniofacial deformities: a three-dimensional quantification method.

To determine the skeletal changes after midface surgery in patients with syndromic craniosynostosis who underwent Le Fort III (LFIII), monobloc (MB), or facial bipartition (FB). This was a retrospective study including 75 patients: 33 treated by LFIII, 29 by MB, and 13 by FB. Twenty-five had a diagnosis of Apert, 39 Crouzon, and 11 craniofrontonasal syndrome. A three-dimensional mesh was created from the preoperative scan and registered to the postoperative scan to visualise the advancement. LFIII at age 7-12 years effectuated a higher mean advancement in the maxillary (15.5 mm) and zygomatic (7.6 mm) regions when compared to ≥13 years (10.2 mm and 5.5 mm). After MB, mean advancement of the fronto-orbital region was higher at <7 years (16.4 mm), and similarly lower at ages 7-12 (13.8 mm) and ≥13 (12.5 mm). The mean preoperative inter-dacryon distance (34.4 ± 4.4 mm) was reduced by 8.7 ± 4.2 mm after FB without distraction (n = 10). More advancement was seen when midface surgery was performed at a younger age, due to more severe cases and a desire for overcorrection. The highest mean advancement was observed in the fronto-orbital region. Antero-inferior rotational movement was seen after all three techniques.

Intracranial complications of sinogenic and otogenic infections in children: an ESPN survey on their occurrence in the pre-COVID and post-COVID era.

BACKGROUND: COVID-19 pandemic is thought to have changed the epidemiology of some pediatric neurosurgical disease: among them are the intracranial complications of sinusitis and otitis (ICSO). According to some studies on a limited number of cases, both streptococci-related sinusitis and ICSO would have increased immediately after the pandemic, although the reason is not clear yet (seasonal changes versus pandemic-related effects). The goal of the present survey of the European Society for Pediatric Neurosurgery (ESPN) was to collect a large number of cases from different European countries encompassing the pre-COVID (2017-2019), COVID (2020-2021), and post-COVID period (2022-June 2023) looking for possible epidemiological and/or clinical changes. MATERIAL AND METHODS: An English language questionnaire was sent to ESPN members about year of the event, patient's age and gender, presence of immune-deficit or other favoring risk factors, COVID infection, signs and symptoms at onset, site of primary infection, type of intracranial complication, identified germ, type and number of surgical operations, type and duration of medical treatment, clinical and radiological outcome, duration of the follow-up. RESULTS: Two hundred fifty-four cases were collected by 30 centers coming from 14 different European countries. There was a statistically significant difference between the post-COVID period (129 children, 86 cases/year, 50.7% of the whole series) and the COVID (40 children, 20 cases/year, 15.7%) or the pre-COVID period (85 children, 28.3 cases/year, 33.5%). Other significant differences concerned the presence of predisposing factors/concurrent diseases (higher in the pre-COVID period) and previous COVID infection (higher in the post-COVID period). No relevant differences occurred as far as demographic, microbiological, clinical, radiological, outcome, morbidity, and mortality data were concerned. Paranasal sinuses and middle ear/mastoid were the most involved primary site of infection (71% and 27%, respectively), while extradural or subdural empyema and brain abscess were the most common ICSO (73% and 17%, respectively). Surgery was required in 95% of cases (neurosurgical and ENT procedure in 71% and 62% of cases, respectively) while antibiotics in 99% of cases. After a 12.4-month follow-up, a full clinical and radiological recovery was obtained in 85% and 84% of cases, respectively. The mortality rate was 2.7%. CONCLUSIONS: These results suggest that the occurrence of ICSO was significantly increased after the pandemic. Such an increase seems to be related to the indirect effects of the pandemic (e.g., immunity debt) rather than to a direct effect of COVID infection or to seasonal fluctuations. ICSO remain challenging diseases but the pandemic did not affect the management strategies nor their prognosis. The epidemiological change of sinusitis/otitis and ICSO should alert about the appropriate follow-up of children with sinusitis/otitis.

Quantitative detection and follow-up of intracranial hypertension in craniosynostosis: an optical coherence tomography study.

BACKGROUND/PURPOSE: To evaluate in craniosynostosis: 1) the diagnostic accuracy of fundoscopy and optical coherence tomography (OCT) to detect intracranial hypertension (ICH); 2) the time course of retinal thickness after treatment of ICH; and 3) the relation between high hyperopia (HH) and fundoscopy/OCT scan findings. METHODS: Syndromic, multisuture, unicoronal, unilambdoid and sagittal synostosis patients visiting our national center were included in this longitudinal cohort study and formed a consecutive series. Retinal layers on OCT, OCT fundus image and fundoscopy were evaluated. ICH was scored according to presence of abnormal intracranial pressures, hydrocephalus, progressive cerebellar tonsillar herniation or fingerprinting and growth arrest. Diagnostic accuracy of OCT, fundoscopy and fundus image, the time course of retinal thickness after ICH and interference of HH were analyzed using linear mixed models. RESULTS: 577 OCT scans in 307 patients were included. ICH was found in 7.2%. Combining total retinal thickness (TRT), OCT fundus image and fundoscopy resulted in a sensitivity of 76% and 81% specificity to detect signs of ICH. TRT was increased in patients who have had signs of ICH versus patients who never had signs of ICH (ß+44.9 µm in patients who have had ICH, 95%CI 9.0-80.8,P=0.01). TRT decreased to normal in the years after surgery (ß -3.6 µm/year, 95%CI -7.2 - -0.05, P=0.047). There were greater odds of having increased TRT in patients with HH (OR 2.9, 95%CI 1.1-7.6,P=0.03). CONCLUSIONS: The correlation between TRT, OCT fundus image, fundoscopy and particularly for the combination of these parameters with ICP surrogate markers is fair. Increased TRT in the presence of a clinical suspicion of ICH warrants further screening.

Neurological deficits are present in syndromic craniosynostosis patients with and without tonsillar herniation.

BACKGROUND: Children with syndromic craniosynostosis (sCS) have a higher incidence of cerebellar tonsillar herniation (TH) than the general population. In the general population, TH ≥ 5 mm below the foramen magnum is associated with typical neurological deficits but, in sCS, we do not know whether this degree of TH is required before such deficits occur. OBJECTIVE: This prospective cohort study aimed to determine the association between findings on neurological assessment and cerebellar tonsillar position. METHODS: Magnetic resonance imaging (MRI) was used to determine TH ≥ 5 mm and the presence of syringomyelia. In regard to the outcome of neurological deficits, these were categorized according to: A, cerebellar function; B, cranial nerve abnormalities; and C, sensory or motor dysfunction. RESULTS: Twenty of 63 patients with sCS (32% [95% confidence interval 21-45%]) had TH ≥ 5 mm and/or syringomyelia. There was no significant difference in proportion between individual forms of sCS: 16/34 Crouzon, 2/11 Muenke, 2/12 Apert, and 0/7 Saethre-Chotzen patients. Neurological deficits were prevalent (73% [95% confidence interval 60-83%]), and as frequent in patients with TH ≥ 5 mm and/or syringomyelia as those without. Surgery occurred in 3 patients overall, and only in Crouzon patients. CONCLUSION: Determining the effect of TH ≥ 5 mm on neurologic functioning in sCS patients is used to better determine when surgical intervention is warranted. However, we have found that neurological deficits are prevalent in sCS patients, irrespective of cerebellar tonsillar position, suggesting that such findings are developmental and, in part, syndrome-specific central nervous system features.

Abnormal transcranial Doppler cerebral blood flow velocity and blood pressure profiles in children with syndromic craniosynostosis and papilledema.

OBJECTIVE: Children with syndromic craniosynostosis are at risk of intracranial hypertension. This study aims to examine patient profiles of transcranial Doppler (TCD) cerebral blood flow velocity (CBFv) and systemic blood pressure (BP) in subjects with and without papilledema at the time of surgery, and subsequent effect of cranial vault expansion. METHODS: Prospective study of patients treated at a national referral center. Patients underwent TCD of the middle cerebral artery 1 day before and 3 weeks after surgery. Measurements included mean CBFv, peak systolic velocity, and end diastolic velocity; age-corrected resistive index (RI) was calculated. Systemic BP was recorded. Papilledema was used to indicate intracranial hypertension. RESULTS: Twelve patients (mean age 3.1 years, range 0.4-9.5) underwent TCD; 6 subjects had papilledema. Pre-operatively, patients with papilledema, in comparison to those without, had higher TCD values, RI, and BP (all p = 0.04); post-operatively, the distinction regarding BP remained (p = 0.04). There is a significant effect of time following vault surgery with a decrease in RI (p < 0.01). CONCLUSION: Patients with syndromic craniosynostosis who have papilledema have a different TCD profile with raised BP. Vault surgery results in increased CBFv and decrease in RI, however the associated systemic BP response to intracranial hypertension remained at short-term follow-up.

Spring-assisted posterior vault expansion in multisuture craniosynostosis.

PURPOSE: Patients with Apert and Crouzon syndromes and craniofrontonasal dysplasia need a vault expansion within the first year of life to treat or prevent the development of raised intracranial pressure. Many craniofacial units perform a conventional posterior vault expansion as initial surgery; an alternative to this technique is the spring-assisted posterior vault expansion. The purpose of this study was to demonstrate the efficacy of spring-assisted posterior vault expansion and to compare this technique with the conventional method. METHODS: A retrospective study was conducted among all consecutive patients who received a posterior vault expansion between 2006 and 2011. Patients treated with springs were compared with patients treated with the conventional technique for blood loss, duration of surgery, postoperative hospital admittance, increase in skull circumference and anterior-posterior length, and complications. RESULTS: Of the 31 included patients, 15 were treated with springs, and 16, with the conventional technique. Patients treated with springs had a significantly larger increase in skull circumference and anterior-posterior length, and not significant changes in blood loss compared with the conventional group. Complications in the conventional group were the following: minor dural tear in three patients, problematic wound healing in one patient, and insufficient expansion in one patient. Spring-related complications included skin perforation in two patients, a minor dural tear in two patients, and leakage of cerebrospinal fluid after an unnoticed dural tear during spring placement in one patient. CONCLUSION: Spring-assisted posterior vault expansion has some advantages over the conventional technique and is, therefore, the preferred technique in our center.

Endoscopic third ventriculocisternostomy in hydrocephalic children under 2 years of age: appropriate or not? A single-center retrospective cohort study.

PURPOSE: Treating hydrocephalus can be difficult in children under the age of 2 years because a high amount of uncertainty exists as to which treatment to perform. In this retrospective cohort study, we analyzed children under the age of 2 years with hydrocephalus undergoing an endoscopic third ventriculocisternostomy (ETV) with respect to ETV outcome. METHODS: In 59 consecutive patients under the age of 2 years, an ETV was performed between 1999 and 2010 at the Erasmus MC, Sophia Children's Hospital. Demographics, etiology of hydrocephalus, and radiological data were extracted retrospectively from the patients' medical records and operative reports and related to outcome. ETV Success Score (ETVSS) was used to retrospectively calculate the probability of success related to the actual outcome. RESULTS: In this series, 42.4 % of patients had a successful ETV. The only statistically significant finding concerned age. The failed ETV patients appeared to be younger (0.52 ± 0.60 vs. 0.86 ± 0.56 year, p = 0.005), and when using a cutoff age of 6 months only, five out of 32 infants had a successful ETV (p = 0.002). Of the children with an arachnoid cyst, 57.1 % were treated successfully with an ETV. Of the five patients with a high probability of ETV success, four (80 %) were indeed successfully treated with ETV (p = 0.049). CONCLUSIONS: Our data confirm the overall ineffectiveness of an ETV in children under the age of 6 months. Nevertheless, using the ETVSS is recommended to aid in the decision-making process even in patients under the age of 6 months.

Long-term functional outcome in 167 patients with syndromic craniosynostosis; defining a syndrome-specific risk profile.

OBJECTIVE: Little is known about the long-term prevalence of elevated intracranial pressure (ICP), obstructive sleep apnoea (OSA), level of education, language and motor skills, impaired sight and hearing in craniosynostosis syndromes. The objective of this study was to define the prevalence per syndrome of elevated ICP, OSA, impaired sight and impaired hearing. METHODS: A retrospective study was undertaken on 167 consecutive patients diagnosed with Apert, Crouzon, Pfeiffer, Muenke or Saethre-Chotzen syndrome, aged 1-25 years and treated between 1983 and 2008. The mean age at time of referral and review was 1 years and 2 months and 10 years and 3 months, respectively. RESULTS: Patients with Apert and Crouzon/Pfeiffer syndromes had the highest prevalence of elevated ICP (33% and 53%, respectively) and OSA (31% and 27%, respectively), while Saethre-Chotzen syndrome was also associated with a fair risk for elevated ICP (21%). The prevalence of impaired sight (61%) and hearing (56%) was high in all syndromes. CONCLUSION: Based on these data, a syndrome-specific risk profile with suggestions for screening and treatment is presented.

Brucellosis, an uncommon and frequently delayed diagnosis.

In the Netherlands, brucellosis is uncommon. Diagnosis is difficult and frequently delayed. We present three patients with back pain and/or arthralgia caused by brucellosis. We emphasise the importance of considering brucellosis in patients returning from a stay in a rural area of an endemic country, who present with osteoarticular symptoms and signs of chronic inflammation. Clues to the diagnosis come from a thorough medical history.

Supratentorial low grade astrocytoma: prognostic factors, dedifferentiation, and the issue of early versus late surgery.

BACKGROUND: A retrospective study of patients with low grade astrocytoma was carried out because the best management of such patients remains controversial. Prognostic factors were identified by multivariate analysis. Special attention was paid to the effect of extent and timing of surgery. METHODS: Ninety patients with low grade astrocytoma were studied. Seventy two patients had resective surgery, 15 had a diagnostic biopsy only, and three patients had resective surgery after initial biopsy. RESULTS: Significant prognostic factors for survival were age, preoperative neurological condition, epilepsy as the single sign, extent of surgery, and histology. The extent of surgery was highly significant on univariate analysis (p=0.002); however, after correction for age and preoperative symptoms this was considerably reduced (p=0.04). A subgroup of 30 patients with epilepsy as their single presenting symptom was identified. Thirteen of these patients were treated immediately after diagnosis, whereas the other 17 patients were initially followed up and treated only after clinical or radiological progression. Survival in both groups was identical (63% survival rate after five years) and much better than survival for the whole group (27% survival rate after five years). Malignant dedifferentiation was observed in 25 (70%) of 36 patients who were reoperated, after a median period of 37 months. This period was 41 months for the subgroup of patients with epilepsy only and 28 months for the remaining patients. CONCLUSIONS: Due to the retrospective nature of the study only restricted conclusions can be drawn. Low grade glioma with epilepsy as the single symptom has a much better prognosis than if accompanied by other symptoms. This prognosis is not influenced by the timing of surgery. It seems, therefore, safe to defer surgery until clinical or radiological progression in low grade glioma with epilepsy only.