Background The embryology of the bony craniovertebral junction (CVJ) is reviewed with the purpose of explaining the genesis and unusual configurations of the numerous congenital malformations in this ...region. Functionally, the bony CVJ can be divided into a central pillar consisting of the basiocciput and dental pivot and a two-tiered ring revolving round the central pivot, comprising the foramen magnum rim and occipital condyles above and the atlantal ring below. Embryologically, the central pillar and the surrounding rings descend from different primordia, and accordingly, developmental anomalies at the CVJ can also be segregated into those affecting the central pillar and those affecting the surrounding rings, respectively. Discussion A logical classification of this seemingly unwieldy group of malformations is thus possible based on their ontogenetic lineage, morbid anatomy, and clinical relevance. Representative examples of the main constituents of this classification scheme are given, and their surgical treatments are selectively discussed.
We present 2 studies that investigate the use of emoticons in clarifying message intent. We examine sarcasm in particular, which can be especially hard to interpret correctly in written ...communication. In both studies, participants were required to make the intentions of their messages clear. In the first, they clarified the meaning of existing sentences without altering the wording; in the second, they produced their own sentences. Results provided clear evidence that tongue and wink emoticons are the principal indicators of sarcastic intent, and that ellipsis is associated more with criticism, rather than with sarcasm. These findings highlight the significant role emoticons play in clarifying message intention, compensating for the absence of nonverbal cues in written communication.
Torticollis is encountered often in the paediatric setting and should be considered a presenting symptom, rather than a diagnosis. Aetiologies of torticollis are numerous, and the nomenclature ...describing underlying diagnosis can be confusing. Furthermore, children with torticollis typically present in the first instance to primary or secondary care rather than to the subspecialist. These factors can contribute to erroneous treatment of this patient-group which could be time critical in some instances. In this review, we discuss the common causes for torticollis and propose a simple clinical assessment tool and early management scheme that will assist in the differential-diagnosis and treatment pathway of this challenging condition.
Conclusion
: Torticollis can be the initial presentation of various conditions. The diagnosis and management tools provided in this article can aid in guiding paediatricians as to the correct initial management, imaging, and specialist referral.
What is Known:
• Torticollis in childhood is a very common presenting symptom with numerous aetiologies.
• Management is complex, requires multiple clinical and imaging examinations, and is usually performed by non-specialized professionals.
What is New:
• A new, simple clinical-assessment tool under the acronym PINCH designed to aid paediatric general practitioners in diagnosing correctly the aetiology of torticollis.
• A practical management scheme to aid in the treatment pathway of children with torticollis.
Aims
As awareness and frequency of tethered spinal cord (TSC) related to occult spinal dysraphism (OSD) has increased with magnetic resonance imaging (MRI), variability exists in its evaluation and ...management. Due to no published level I data, we summarize the current International Children's Continence Society (ICCS) recommendations for diagnosis and treatment of OSD.
Methods
Guidelines were formulated based on analysis of pertinent literature and consensus among authors. This document was vetted by the multidisciplinary members of the ICCS via its website before submission for peer review publication.
Results
The more frequent diagnosis of OSD is associated with increased operative intervention. Spinal cord untethering (SCU) has a highly variable risk profile, largely dependent on the specific form of OSD. Progressive neurological deterioration attributed to “tethered cord” may occur, with or without surgery, in selected forms of OSD whereas other cohorts do well.
Conclusion
Infants with classic cutaneous markers of OSD, with progressive neurologic, skeletal, and/or urologic findings, present no diagnostic or therapeutic dilemma: they routinely undergo MRI and SCU. Conversely, in asymptomatic patients or those with fixed, minor abnormalities, the risk profile of these OSD cohorts should be carefully considered before SCU is performed. Irrespective of whether or not SCU is performed, patients at risk for progression should be followed carefully throughout childhood and adolescence by a multidisciplinary team.
Purpose
Whilst rigid fixation for craniovertebral instability is the gold standard, in very young, small children conventional management may have to be modified. We present a single-centre ...experience of craniocervical fixation in children under 5 years.
Methods
A retrospective review of cases that had undergone atlantoaxial (AA) or occipitocervical (OC) fixation aged under 5 years. Fusion was assessed using computerised tomography or flexion extension X-rays.
Results
Twenty-six children (median age 2.3, range 0.8–4.9 years, 19 under the age of 3) underwent OC (
n
= 19) or AA (
n
= 7) fusion between 1999 and 2016. Pathology comprised 17 congenital, five trauma, two tumour and two post-infection cases. Twenty-one patients underwent sublaminar cable fixation with calvarial, autologous bone graft and halo-body orthosis immobilisation. An occipital plate and rods to sublaminar wire construct were used in four cases. A rigid instrumented fixation with occipital plate and C2 pedicle screws was utilised in one case. Follow-up was for a median of 2.8 years (range 0.03–16.3 years). Initial fusion rate was 91%, reaching 100% following two re-operations. Ninety-two per cent of patients were neurologically stable or improved following surgery. Twenty-one patients had a good overall outcome. Two patients had post-operative neurological deteriorations, and four died due to non-procedure related causes. Pin site morbidity secondary to halo use occurred in five cases.
Conclusion
High fusion rates with good outcomes are achievable using semi-rigid fixation in the under 5-year-olds. Full thickness, autologous calvarial bone graft secured with wire cables and halo external orthosis offers a safe and effective alternative technique when traditional screw instrumentation is not feasible.
Graphic abstract
These slides can be retrieved under Electronic Supplementary Material.
Intracardiac migration is a rare complication of ventriculoperitoneal shunt insertion. Only 15 cases have been reported, 7 of which were paediatric cases, treated with techniques including ...interventional radiography, open thoracotomies and direct extraction through the initial shunt incision. The authors report the youngest case of intracardiac shunt migration complicated by significant coiling and knotting within the cardiac chambers and pulmonary vasculature. Migration likely began when the SVC was pierced during initial shunt placement and progressed due to negative intrathoracic pressure. Extrusion was achieved combining thoracoscopic endoscopy, interventional fluoroscopy screening and a posterolateral neck incision with uncoiling of the shunt via a Seldinger guide wire. This offered a minimally invasive solution with rapid post-operative recovery.
The term Chiari I malformation (CIM) is imbedded in the paediatric neurosurgical lexicon; however, the diagnostic criteria for this entity are imprecise, its pathophysiology variable, and the ...treatment options diverse. Until recently, CIM has been considered to be a discrete congenital malformation requiring a uniform approach to treatment. Increasingly, it is recognised that this is an oversimplification and that a more critical, etiologically based approach to the evaluation of children with this diagnosis is essential, not only to select those children who might be suitable for surgical treatment (and, of course those who might be better served by conservative management) but also to determine the most appropriate surgical strategy. Whilst good outcomes can be anticipated in the majority of children with CIM following foramen magnum decompression, treatment failures and complication rates are not insignificant. Arguably, poor or suboptimal outcomes following treatment for CIM reflect, not only a failure of surgical technique, but incorrect patient selection and failure to acknowledge the diverse pathophysiology underlying the phenomenon of CIM. The investigation of the child with ‘hindbrain herniation’ should be aimed at better understanding the mechanisms underlying the herniation so that these may be addressed by an appropriate choice of treatment.
Objective
Encephaloceles are considered to result from defects in the developing skull through which meninges, and potentially brain tissue, herniate. The pathological mechanism underlying this ...process is incompletely understood. We aimed to describe the location of encephaloceles through the generation of a group atlas to determine whether they occur at random sites or clusters within distinct anatomical regions.
Methods
Patients diagnosed with cranial encephaloceles or meningoceles were identified from a prospectively maintained database between 1984 and 2021. Images were transformed to atlas space using non-linear registration. The bone defect, encephalocele and herniated brain contents were manually segmented allowing for a 3-dimensional heat map of encephalocele locations to be generated. The centroids of the bone defects were clustered utilising a K-mean clustering machine learning algorithm in which the elbow method was used to identify the optimal number of clusters.
Results
Of the 124 patients identified, 55 had volumetric imaging in the form of MRI (48/55) or CT (7/55) that could be used for atlas generation. Median encephalocele volume was 14,704 (IQR 3655–86,746) mm
3
and the median surface area of the skull defect was 679 (IQR 374–765) mm
2
. Brain herniation into the encephalocele was found in 45% (25/55) with a median volume of 7433 (IQR 3123–14,237) mm
3
. Application of the elbow method revealed 3 discrete clusters: (1) anterior skull base (22%; 12/55), (2) parieto-occipital junction (45%; 25/55) and (3) peri-torcular (33%; 18/55). Cluster analysis revealed no correlation between the location of the encephalocele with gender (
χ
2
(2,
n
= 91) = 3.86,
p
= 0.15). Compared to expected population frequencies, encephaloceles were relatively more common in Black, Asian and Other compared to White ethnicities. A falcine sinus was identified in 51% (28/55) of cases. Falcine sinuses were more common (
χ
2
(2,
n
= 55) = 6.09,
p
= 0.05) whilst brain herniation was less common (
χ
2
(2,
n
= 55) = .16.24,
p
< 0.0003) in the parieto-occipital location.
Conclusion
This analysis revealed three predominant clusters for the location of encephaloceles, with the parieto-occipital junction being the most common. The stereotypic location of encephaloceles into anatomically distinct clusters and the coexistence of distinct venous malformations at certain sites suggests that their location is not random and raises the possibility of distinct pathogenic mechanisms unique to each of these regions.
Dermal sinus tracts (DSTs) are congenital lesions that connect the cutaneous ectoderm with the underlying neuroectodermal tissues. They are typically midline, solitary lesions. Multiple, and ...atypically located DSTs have been only rarely described. We present two cases of multiple and laterally located DSTs. The first presented with bacterial meningitis and two tracts in the right buttock, one of which entered the spinal canal through the S3 neural foramen. The second child had three midline lumbar DSTs, one subcutaneous dermoid cyst and one intradural epidermoid cyst. Complete surgical excision was achieved in both cases with good late follow up. Multiple or atypically located DSTs appear to carry the same risks of infection as the more common, midline, single tracts. Complete surgical excision is recommended to avoid the risks of neurological deterioration, in particular due to infection. Incomplete disjunction is the proposed developmental anomaly for DSTs, however the location of the cases presented here requires an alternative explanation.
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