Transillumination of an Anterior Fontanelle Dermoid Cyst.

Dermoid cysts are slowly growing benign lesions of ectodermal tissue that often occur in the anterior fontanelle. Clinicians often rely on a negative transillumination test to begin the process of correctly diagnosing a dermoid cyst. However, here the authors present a case of a 7-month-old girl who presents with a transilluminating dermoid cyst.

Pediatric ventriculoperitoneal shunt: a comparative study between anterior fontanel ultrasound-guided versus conventional cranial end insertion.

PURPOSE: Ventriculoperitoneal (V-P) shunt is one of the most common neurosurgical procedures in pediatrics for the treatment of hydrocephalus. Shunt failure is one of the common mechanical complications which lead to major morbidities. This study aims to compare between cranial part insertions of the V-P shunts guided by trans-anterior fontanel ultrasound versus conventional insertion. METHODS: A prospective comparative randomized study was conducted on 60 pediatric patients aged ≤ 2 years who suffered hydrocephalus and allocated into 2 groups. In the first group (n = 30), the cranial parts of the ventriculoperitoneal shunts were inserted guided by trans-anterior fontanel ultrasound, and in the second group (n = 30), the insertions were by the conventional method. The follow-up duration of the patients was 3 months. RESULTS: Proximal part obstruction of the V-P shunt was found in 3 cases of the conventional group during follow-up with statistical insignificance (p = 0.237) while adequate proximal part location recorded statistical significance (p = 0.0005) in favor of ultrasound-guided group. CONCLUSION: The use of the anterior fontanel ultrasound guide during ventriculoperitoneal shunt insertion is a feasible, safe, and effective technique for the placement of ventricular catheters in pediatric patients with a patent anterior fontanel.

Anterior fontanelle closure and diagnosis of non-syndromic craniosynostosis: a comparative study using computed tomography.

OBJECTIVE: Suspicion of early anterior fontanel (AF) closure is a common reason for referral to a pediatric neurosurgeon because of the suspected increased risk of developing craniosynostosis (CS) in spite of the absence of evidence in the literature. The aim of this study was to analyze the association between AF closure and the diagnosis of non-syndromic CS in Brazilian children. METHODS: An observational and case-cohort study was conducted to compare the incidence of closed AF between healthy children (group 1) and children diagnosed with non-syndromic CS (group 2) at a pediatric neurosurgery referral center. The accuracies of completely closed AF and diagnosis of CS were assessed. RESULTS: High-resolution three-dimensional reconstruction computed tomography scans were obtained for 140 children aged < 13 months, of whom 62.9% were boys and 37.1% were girls (p < 0.001). The most common types of non-syndromic CS were trigonocephaly (34, 48%) and scaphocephaly (25, 35.7%). Closed fontanel (27, 38.6%) was observed in both groups, and a sensitivity of 36.1%, specificity of 72%, the positive predictive value of 59%, and negative predictive value of 51% were observed in the patients diagnosed with CS when AF closure occurred before the age of 6 months. CONCLUSION: The results of this comparative study of AF closure and CS diagnosis suggest that early AF closure does not imply a diagnosis of CS. Pediatricians should be aware of the risk of misdiagnosis of CS in cases with a widely open AF in spite of the presence of CS.

Diagnosis and Management of Suture-Related Concerns of the Infant Skull.

The cranial fontanelles and sutures have several benign variations, including most cases of "early" or "late" closure of the anterior fontanelle, bathrocephaly, overriding sutures, and benign metopic ridging. However, recognizing true craniosynostosis and referring the patient to a craniofacial specialist in a timely fashion are imperative, as minimally invasive options can be offered to most patients younger than 6 months of age. Gaining comfort with the physical examination of an infant with an abnormal head shape is best achieved through experience and pattern recognition and will frequently facilitate an accurate diagnosis without the need for ionizing radiation.

Neurodevelopmental risk evaluation of premature closure of the anterior fontanelle.

PURPOSE: To evaluate neurological development of completely healthy children with anterior fontanelle premature closure via Denver Developmental Screening Test II and to compare the results with control group. METHOD AND RESULTS: The records of 140 patients applied to Mersin University Pediatric Neurology Outpatient Clinic between 2011 and 2019 with the complaint of premature closure of the anterior fontanelle were retrospectively reviewed. Patients with microcephaly, craniosynostosis, infection, sequelae of hypoxia-ischemia, metabolic disorders, intracranial hemorrhage, epilepsy, endocrine problems, and dysmorphic features were excluded from the study. Sixty-six completely healthy children with anterior fontanelle premature closure were included in the study. Denver Developmental Screening Test II was performed by the same developmental specialist to the children with premature closure of the anterior fontanelle as well as to the healthy control group. For each child included in the case and the control group, 90% of the values for each development area were calculated and recorded. Then, the results were compared. Denver II Developmental Screening Test (p < 0.001) and gross motor subtest (p < 0.001) results showed statistically significant retardation in the case group compared with the control group. CONCLUSIONS: The study was the first study in the literature on the gross motor development of children with premature closure of anterior fontanelle, and it has been found significantly undeveloped compared with the control group, and it has been concluded that similar patients should be evaluated from this view point in pediatric neurology department.

Temporal mapping of the closure of the anterior fontanelle and contiguous sutures using computed tomography, in silico models of modern infants.

The aim of this study is to quantify and statistically model the age-related decline in the fibrous connective tissue interface of the anterior fontanelle in modern Australian infants, using three-dimensional, semi-automated computed-assisted design protocols. Non-linear regression with variance models, using power functions, combined with quantile regression of the 5th and 95th population percentiles, were utilised to assess absolute anterior fontanelle surface area (AFSA) as a function of age, using multi-slice cranial computed tomography scans obtained from 256 infants aged < 30 months (males: n = 126, females: n = 109) from Brisbane children's hospitals. Normalised AFSA (NFSA), standardised for variation in cephalic size, followed a progressive decline from birth, the greatest velocity change occurring between the 3-6 and 6-9 month cohorts. Growth of the neurocranium is the most significant within the first 8 months postpartum, with a mean increase of 19.03 mm in maximum cranial length and 10.04 mm in breadth. Directionality of fontanelle closure, quantified using spline curves refutes fundamental assumptions that the anterior fontanelle is consistent with a quadrilateral, and contiguous sutures exhibit constant velocity of closure. The present study provides normative values for fontanelle size and diameters as well as new predictive non-linear models for age substantiation, screening of developmental abnormalities and indicators of suspected child maltreatment in modern infants aged birth to 30 months.

Normal values of the resistivity index of the pericallosal artery with and without compression of the anterior fontanelle.

BACKGROUND: Resistivity index (RI) of the pericallosal artery as is commonly measured during head ultrasound (US) examination in neonates. Some studies have shown that RI measured with gentle compression of the fontanelle provides additional information in cases of neonatal brain anomalies. OBJECTIVE: The purpose of this study was to establish normal RI values with and without compression in a large population of neonates with normal cranial ultrasound as a function of gestational age. MATERIALS AND METHODS: The authors of this retrospective study reviewed the RI of 323 infants with normal gray-scale cranial US and with a gestational age ranging 26-42 weeks. We conducted the exams both with and without compression of the anterior fontanelle and we studied changes in RI depending on gestational age, gender and type of delivery. RESULTS: Infants with a gestational age of more than 35 weeks tended to have a lower RI (P=0.011). The compression of the anterior fontanelle emphasized the change in RI with increasing gestational age, with higher gestational ages having a lower RI (P<0.001). The results concerning the percentage change between baseline RI and RI with compression showed that infants with higher gestational ages have a smaller percentage change in RI (P=0.002). CONCLUSION: We established the normal values for RI from 26 weeks to 42 weeks of gestation. The results of the study show the importance of taking the gestational age into consideration when evaluating the RI.

Closure of the anterior and posterior fontanelle in the New Zealand population: A computed tomography study.

AIM: Significant ethnic variation has been demonstrated in the closure of the anterior fontanelle (AF); however, to date, this has not been investigated in the Maori/Pasifika population. METHODS: The computed tomography scans of 163 individuals (116 Maori/Pasifika and 47 New Zealand (NZ) European) aged between birth and 4 years were retrospectively analysed to investigate the surface area (SA) and time of closure of the anterior and posterior fontanelles in New Zealand. RESULTS: The Maori/Pasifika group showed clinical AF closure (SA < 114 mm2 ) rates of 25% at 4-6 months, increasing to 47% at 10-12 months and 80% at 13-18 months. The posterior fontanelle was clinically unfused in 17% of the Maori/Pasifika group aged <1 month and in 7% of the 1-3-month-old group. No cases of posterior fontanelle non-fusion were identified in the NZ European population. CONCLUSION: This study establishes normal values for AF size and closure frequency for the first time in the paediatric Maori/Pasifika population.

Identifying infant hydrocephalus in the emergency department with transfontanellar POCUS.

Hydrocephalus carries significant morbidity in the infant population. Although clinical symptoms are often nonspecific, hydrocephalus is easily identified using transfontanellar sonography. In this review, we provide the emergency physician with a succinct overview of infant hydrocephalus and the point-of-care ultrasound (POCUS) technique for identification of this pathology.

Neuroimaging findings using transfontanellar ultrasound in newborns with microcephaly: a possible association with congenital Zika virus infection.

OBJECTIVE: The objective of this study is to determine the main neuroimaging findings of microcephalic newborns with possible Zika virus (ZIKV) intrauterine infection using transfontanellar cranial ultrasound. METHODS: We performed a retrospective study to describe the main neuroimaging findings in newborns with microcephaly and possible association with congenital ZIKV infection. Microcephaly was defined in the postnatal period using transfontanellar cranial examination which was performed using both two- (2D) and three-dimensional (3D) ultrasound. RESULTS: One hundred and fifty newborns with microcephaly were identified during the study period. The mean ± (standard deviation - SD) of cephalic perimeter was 28.5 ± 4.2 cm (range, 25-38 cm). Transfontanellar neuroimaging patterns detected cerebral calcifications, neuronal migrational abnormalities, dysgenesis of the corpus callosum, and cerebellar atrophy in 34.9%, 31.1%, 26%, and 16.2%, respectively. Hydrocephalus was seen in 28% of overall newborns. A history of maculopapular rash was present in almost half of the mothers (46.1%). CONCLUSION: Neuroimaging patterns by means of transfontanellar ultrasound are accurate and diagnostic investigations of brain pathology in newborns affected by microcephaly and possible intrauterine ZIKV infection.

Estimation of elevated intracranial pressure in infants with hydroce-phalus by using transcranial Doppler velocimetry with fontanel compression.

For infants with acute progressive hydrocephalus, invasive drainage of cerebrospinal fluid (CSF) is performed until a ventriculo-peritoneal shunt can be inserted. Surrogate markers of intracranial pressure (ICP) may help optimise the timing of invasive procedures. To assess whether RI with/without fontanel compression helps distinguish between infants with normal (<5 cmH2O), mild (5-11 cmH2O), and moderate (>11 cmH2O) ICP elevation, 74 ICP measures before/after CSF removal and 148 related Doppler measures of the middle cerebral artery were assessed. Higher RI was associated with fontanel compression, elevated ICP, and their interaction (all p < 0.001). Without compression, differences in RI were observed between normal and moderate (p < 0.001) and between mild and moderate ICP elevation (p = 0.033). With compression, differences in RI were observed for all pairwise comparisons among normal, mild, and moderate ICP elevation (all p < 0.001). Without compression, areas under the receiver-operating characteristic curve for prediction of mild and moderate ICP elevation were 0.664 (95% confidence interval (CI), 0.538-0.791; p = 0.020) and 0.727 (95% CI, 0.582-0.872; p = 0.004), respectively, which improved to 0.806 (95% CI, 0.703-0.910; p < 0.001) and 0.814 (95% CI, 0.707-0.921; p < 0.001), respectively, with compression. RI with fontanel compression provides improved discrimination of infants with absent, mild, and moderate ICP elevation.

Potential Role of Transfontanelle Ultrasound for Infants Undergoing Modified Blalock-Taussig Shunt.

OBJECTIVE: Transfontanelle ultrasound is a noninvasive method for assessing cerebral blood flow in neonates and infants. The authors applied this technique as a point-of-care tool, before and after modified Blalock-Taussig shunt procedure, to evaluate cerebral perfusion. DESIGN: Retrospective, observational study. SETTING: Tertiary care children's hospital. PARTICIPANTS: Ten infants undergoing modified Blalock-Taussig shunt procedure. OBSERVATION: Transfontanelle ultrasound examinations with modified resistive index were analyzed before and after the modified Blalock-Taussig shunt procedure. MEASUREMENTS AND MAIN RESULTS: Two infants died and 2 patients required a revision procedure due to shunt thrombosis. Baseline-modified resistive index and regional cerebral oxygenation were comparable between the right and left hemisphere. However, after the procedure, the modified resistive index decreased at both sides of the internal carotid arteries compared with baseline values (p value right side = 0.012, left side = 0.036) and was greater at the ipsilateral internal carotid arteries with the shunt (p = 0.012, mean difference = 0.07, 95% confidence interval [CI] 0.02-0.12). Four infants with the large patent ductus arteriosus presented diastolic reverse flows at both internal carotid arteries at baseline. However, the diastolic reverse flow disappeared after the procedure. An infant who developed diastolic reverse flow after the procedure, died. CONCLUSIONS: Transfontanelle ultrasound is a feasible tool for assessing the pattern of shunt flow and cerebral perfusion before and after the modified Blalock-Taussig shunt procedure. The transfontanelle ultrasound examinations may have potential role in assessing "over-shunting," but it needs more studies.

Osseous Convexity at the Anterior Fontanelle: A Presentation of Metopic Fusion?

BACKGROUND: Craniosynostosis, or a premature fusion of 1 or more cranial vault sutures, results in characteristic head shape deformities. In previous reports, an osseous prominence at the anterior fontanelle has been suggestive of adjacent suture fusion and local elevation in intracranial pressure (ICP). This prominence has been termed the "volcano" sign, and has been described in the anterior fusion of the sagittal suture and serves as an indication for surgery. METHODS: Two patients presented for head shape evaluation with mild metopic ridging and anterior fontanellar osseous convexities consistent with the volcano sign. Low-dose computed tomography imaging was performed in both patients due to concern for underlying craniosynostosis with elevated locoregional ICP. RESULTS: In both patients, imaging was significant for a localized, superior forehead metopic fusion, as well as a bony, convex prominence at the site of the ossified anterior fontanelle. There were no other clinical or radiologic signs or symptoms to suggest elevated ICP. Surgery was not indicated in either patient. CONCLUSIONS: Here the authors present 2 patients with osseous convexities at the site of the closed anterior fontanelle without signs or symptoms of elevated ICP, or classic signs of metopic synostosis. The authors hypothesize that this pattern may be due to a form of mechanically induced premature fusion of a normal metopic suture that is focused superiorly at the bregma, with minimal resultant restriction of overall skull growth. This is in contrast to metopic synostosis, which primarily has a sutural pathology and leads to characteristic findings of hypotelorism and trigonocephaly.

The relationship between transorbital ultrasound measurement of the optic nerve sheath diameter (ONSD) and invasively measured ICP in children. : Part II: age-related ONSD cut-off values and patency of the anterior fontanelle.

PURPOSE: To analyse the diagnostic accuracy of age-related optic nerve sheath diameter (ONSD) cut-off values in children for detecting raised intracranial pressure (ICP) and to assess the benefit of using patency of the anterior fontanelle in describing a different set of cut-off values. METHODS: The ONSD measurement was performed prior to invasive ICP measurement in children under general anesthesia. The diagnostic accuracy of the ONSD measurement was compared to ICP at thresholds of 20, 15, 10, and 5 mmHg. This was further analysed in children above and below the age of 1 year, with a subgroup analysis of age at 4 years, and assessment of the anterior fontanelle (AF) as a reliable physiological marker in part II of this study. RESULTS: Data from 174 children were analysed. In children ≤1 year old, the ONSD measurement with the best diagnostic accuracy for detecting ICP ≥ 20 mmHg was 5.16 mm, compared to 5.75 mm in children >1 year old (p < 0.001). In addition, patency of the anterior fontanelle (AF) was found to be a useful clinical marker for defining different ONSD cut-off values at ICP thresholds of 20, 15, 10 and 5 mmHg. CONCLUSION: Transorbital ultrasound measurement of the ONSD is a reliable non-invasive marker of ICP particularly at higher thresholds of 20 and 15 mmHg. Patency of the AF is a useful clinical marker for defining different ONSD cut-off values in children.

Anterior Fontanelle Wormian Bone With Exomphalos Major and Dysmorphic Facial Features: A Previously Unseen Association?

Wormian bones are independent ossification centers found within cranial sutures or fontanelles. Though common in adult populations, their presence in children can be associated with several conditions such as osteogenesis imperfecta, hypothyroidism, pyknodysostosis, cleidocranial dysostosis, rickets, and acrocallosal syndrome. These conditions encompass a large range of clinical features but there has only been 1 other reported patient of exomphalos occurring concurrently with these ossicles. The authors present the case of a child with an anterior fontanellar Wormian bone, dysmorphic facial features, and exomphalos major born to unaffected parents. The pattern of features seen in this child did not closely match any condition commonly associated with Wormian bones. The only other reported case of both Wormian bone and exomphalos was in a child with acrocallosal syndrome who presented with more severe dysmorphic features than seen here. It is possible that this patient represents a previously unknown association between acrocallosal syndrome and exomphalos or a less severe variant of the condition. Conversely, this patient may possibly illustrate a newly discovered association between Wormian bones, facial dysmorphism, and midline abdominal defects.

Introduction of percutaneous-tunneled transfontanellar external ventricular drainage in the management of hydrocephalus in extremely low-birth-weight infants.

OBJECTIVE Hydrocephalus treatment in extremely low-birth-weight (ELBW) infants still represents a challenge for the pediatric neurosurgeon, particularly when the patient weighs far less than 1000 g. In such cases, the benefits in terms of neurological outcome following early treatment do not always outweigh the surgical risks, especially considering the great difference in the surgical risk before patient weight increases. To assess the efficacy and reliability of a percutaneous-tunneled, transfontanellar external ventricular drain (PTTEVD) in ELBW infants, the authors started a new protocol for the early surgical treatment of hydrocephalus. METHODS Ten cases of posthemorrhagic hydrocephalus (PHH) in ELBW infants (5 cases < 700 g, range for all cases 550-1000 g) were treated with a PTTEVD that was implanted at bedside as the first measure in a stepwise approach. RESULTS The average duration of the procedure was 7 minutes, and there was no blood loss. The drain remained in place for an average of 24 days (range 8-45 days). In all cases early control of the hydrocephalus was achieved. One patient had a single episode of CSF leakage (due to insufficient CSF removal). In another patient Enterococcus in the CSF sample was detected the day after abdominal surgery with ileostomy (infection resolved with intrathecal vancomycin). One patient died of Streptococcus sepsis, a systemic infection existing prior to drain placement that never resolved. One patient had Pseudomonas aeruginosa sepsis prior to drain insertion; a PTTEVD was implanted, the infection resolved, and the hydrocephalus was treated in the same way as with a traditional EVD, while the advantages of a quick, minimally invasive, bedside procedure were maintained. Once a patient reached 1 kg in weight, when necessary, a ventriculoperitoneal shunt was implanted and the PTTEVD was removed. CONCLUSIONS The introduction of PTTEVD placement in our standard protocol for the management of PHH has proved to be a wise option for small patients.

Cystic Congenital Scalp Inclusion Dermoid: A Case Report.

Dermoid cysts are developmental tumours that develop from germ cells displaced between the 3rd and 5th week of embryogenesis. Although dermoid cysts are known to be the most common scalp swellings; cystic congenital inclusion dermoid of the anterior fontanelle is a very rare scalp swelling. It is a benign, slow-growing, non-tender, soft swelling which is covered with intact skin. Ruling out intracranial extension is necessary. Computed Tomography is the investigation of choice for its diagnosis. We describe such a rare case of cystic congenital inclusion dermoid of the anterior fontanelle where the cyst was completely excised.