Effectiveness of screening for craniosynostosis with ultrasound: a retrospective review

Background

Minimizing the ionizing radiation dose to children is fundamental to pediatric radiology. The most widely accepted imaging examination for evaluating craniosynostosis is computed tomography (CT) of the head, an examination that involves ionizing radiation.

Objective

To determine if sonography of the cranial sutures is an adequate screening examination for the diagnosis of craniosynostosis in patients with abnormal skull shape.

Materials and methods

A retrospective review of all cranial suture ultrasound (US) examinations performed during the course of a 3-year period (July 2012 – September 2015) was undertaken. Results were compared with clinical follow-up and/or head CT to evaluate the accuracy of this modality as a screening tool to determine the presence or absence of craniosynostosis. Fifty-two sonographic exams were adequate for inclusion.

Results

Forty-five of the examinations did not reveal synostosis. In each of these instances, follow-up physical exam findings and/or CT imaging confirmed that no abnormal premature suture closure was present. US findings demonstrated synostosis in seven cases. CT exam or operative reports of these cases confirmed all seven findings of premature suture closure. Statistical analysis demonstrated a sensitivity of 100% (95% confidence interval [CI]: 56.1–100.0%), a specificity of 100% (95% CI: 90.2–100.0%), and a negative predictive value of 100% (95% CI: 90.2–100.0%).

Conclusion

Cranial US is a reliable screening tool to rule out craniosynostosis in patients with abnormal head shape.

     

Overview of the classification and treatment of premature craniosynostosis. On the development of encephalocele following surgery of craniostenosis

Craniosynostosis means premature closure of one or more cranial sutures. The redirection of the main growth of the brain according to the remaining open sutures results in different deformities of the skull; the intracerebral pressure may be elevated and neurological signs may be present. After reviewing the etiology, pathogenesis, classification and surgical treatment of craniosynostosis, the author describes a severe clinical complication resulting from surgical intervention.     

Evaluation and management of nonsyndromic craniosynostosis

Craniosynostosis (craniostenosis) is premature fusion of the sutures of the cranial vault. Several factors can affect the growth of the cranial vault during embryonic life and after birth, leading to different types of craniosynostosis; these can be classified on the basis of the specific sutures that are fused. Prognosis is improved by early diagnosis, and it is important to establish the correct approach to these patients on the basis of clinical and neuroradiological investigation. The first priority is to identify the type of craniosynostosis and to distinguish between the types that require surgical intervention and those that do not. We report on the different forms of nonsyndromic craniosynostosis, their clinical and neuroradiological diagnoses, and surgical strategies. CONCLUSION: The aim of this review is to provide to paediatricians a correct diagnostic approach and management of children affected from nonsyndromic craniosynostosis, for which a careful physical, ophthalmological and neurological examination is fundamental, whereas brain Computed tomography and magnetic resonance imaging are necessary for patients in which the diagnosis is uncertain or for cases of syndromic craniosynostosis.     

The utility of the plain radiograph “shunt series” in the evaluation of suspected ventriculoperitoneal shunt failure in pediatric patients

Background To our knowledge, the sensitivity of plain radiography, known as the shunt series, in diagnosing an etiology of ventriculoperitoneal (VP) shunt malfunction in children has not been previously investigated. Objective To determine the accuracy of plain radiography in diagnosing VP shunt failure in children in whom shunt malfunction is clinically suspected. Materials and methods We retrospectively reviewed the charts of 238 children who had undergone plain radiographic examination for evaluation of clinically suspected VP shunt failure over a 5-year period. The results were compared with those of CT, MRI, and nuclear cisternography. Results Just 6.72% of patients demonstrated plain radiographic signs of shunt failure. Of patients with normal plain radiographs, 43% demonstrated shunt abnormalities on CT, MRI or cisternography. Statistical analysis indicated that no more than 10.46% (P < 0.05) of plain radiographs showed signs of failure and that the sensitivity of plain radiography for the detection of VP shunt failure is no higher than 31%. Furthermore, there was poor agreement between the results of plain radiography and those of CT, MRI and cisternography. Conclusion Children with clinically suspected VP shunt failure should proceed directly to cross-sectional or nuclear imaging, as plain radiographic examinations have low sensitivity and significant false-negative rates for detecting shunt abnormalities in all-comers. Use of the shunt series should be limited to patients who specifically have suspected mechanical causes of shunt failure.     

Diagnostic accuracy of ultrasonic examination in suspected craniosynostosis among infants

The current study was performed to assess the diagnostic accuracy of ultrasound compared to CT scan as a gold standard in the diagnosis of craniosynostosis. 44 infants (17 girls) under 1 year old, clinically suspected to have craniosynostosis, were first sonographically examined by a pediatric radiologist and were later referred to another blinded pediatric radiologist to examine CT scan with 3D reconstructed images of skull. Sensitivity, specificity, positive and negative predictive values of ultrasound versus CT scan were 96.9%, 100%, 100%, and 92.3%, respectively. The high specificity of ultrasound helps to correctly rule out craniosynostosis in clinically suspected cases and thus, can prevent unnecessary exposure of healthy infants to CT scan ionizing radiation.     

Craniostenosis. Analysis of 161 cases surgically treated during the first year of life

One hundred-sixty-one cases of children operated on for craniostenosis in the first year of life at the Neurosurgical Department of the Catholic University of Rome in the period 1982-1986 are presented. The average age at diagnosis was 6 months and at operation 7.4 months. The patients considered in the present study were subdivided into two groups according to the prevalent involvement of sagittal suture or of the anterior sutural group (coronal metopic, spheno-frontal and spheno-palatine sutures). In fact, these two groups differ considerably not only in the characteristics of the pathological process of early fusion of the cranial sutures, but also in the technique required for surgical correction. Four children with premature fusion of all cranial sutures are considered separately. Congenital malformations of the bone structures or changes in ocular motility were presented in 7.9% of 63 patients with sagittal craniostenosis and in 14.7% of the group with premature fusion of the anterior cranial sutures. Surgical correction (linear craniectomies extended to the cranial base for the group with sagittal craniostenosis, craniectomies with advancement of the supraorbital margin and rotation of the bone operculum for the group with anterior craniostenosis) proved satisfactory in the great majority of cases. Postoperative morbidity was particularly low. Apart from postsurgical anaemia which required blood transfusion on the third day in 15.5% of operated patients, the only complications were transitory hyperpyretic states observed in 6.2% of cases. Only one child died for reasons related to the surgical procedure. The low postoperative mortality (0.6% of cases) and the low percentage of bone pathology recurrence (7.4%) compared with the results of other series involving older children, seem to indicate that the early surgical correction of bone malformations does not bear additional risk for the patient, permitting at the same time normal functional development of the brain and a more gradual morphological remodelling of the skull.     

Case report of optic atrophy in pansynostosis: an unusual presentation of scalp edema from hair braiding

Pansynostosis (fusion of all cranial sutures) and optic atrophy were found as incidental CT scan and ophthalmological findings in an 8-year-old who presented to the emergency room with scalp edema from tight 'cornrow' hair braiding. Cranial vault expansion was successfully performed. Ophthalmological problems have stabilized but have not reversed. Late presentation of craniosynostosis and the pathophysiology of secondary optic atrophy are discussed.     

Intraosseous haemangioma of the ilium

Intraosseous haemangioma, an uncommon benign vascular tumour, is most commonly seen in adults and tends to involve the vertebrae and the skull. Lesions of flat bones are rare and the imaging findings in these patients are non-specific. We report a unique case of intraosseous haemangioma in the ilium of a 7-year-old girl studied by US, radiography, scintigraphy, CT and MRI.     

Prenatal sonographic findings and prognosis of craniosynostosis diagnosed during the fetal and neonatal periods

The aim of the study was to explore the sonographic findings of fetuses with craniosynostosis and investigate their prognosis. We conducted a 5‐year, multicenter retrospective study and collected data on patients with craniosynostosis diagnosed in the perinatal period. Of 41 cases, 30 cases (73%) were syndromic craniosynostosis, eight cases (20%) were non‐syndromic craniosynostosis and the other three cases (7%) were secondary craniosynostosis of chromosomal deletion syndromes. The prenatal ultrasound detection rate was 61%. Half of the cases of syndromic craniosynostosis detected during the perinatal period were Pfeiffer syndrome; there were also six cases of Apert syndrome, three cases of Crouzon syndrome and other rare form of syndromic craniosynostosis (Beare‐Stevenson syndrome, Saethre‐Chotzen syndrome, cranioectodermal dysplasia, and thanatophoric dysplasia). Abnormal shape of the skull was the most common finding leading to prenatal diagnosis of craniosynostosis. Abnormal head biometry, which was the second most frequent finding, was closely correlated with deformation of the cranial shape. Three cases presented with ventriculomegaly and exophthalmos but normal cranial shape and size. The overall survival rate of infants with syndromic craniosynostosis was 79%, while all of the infants with non‐syndromic craniosynostosis survived. In conclusion, prenatal diagnosis of craniosynostosis is difficult, especially when dysmorphic change of the fetal cranium is not evident. Abnormal head biometry and ventriculomegaly could potentially be additional markers of fetal craniosynostosis and consequently increase the prenatal detection rate.     

Radiological evaluation of visceral injuries in the battered child syndrome

Diagnostic imaging plays an important role in the recognition, evaluation, and follow-up of visceral injuries in the battered child syndrome. Conventional radiography is important for the diagnosis of associated skeletal fractures, pulmonary parenchymal injury, gastric dilatation, and pneumoperitoneum. An upper gastrointestinal series is the examination of choice in suspected intramural duodenal hematoma. Ultrasonography is helpful in the diagnosis of retroperitoneal hematoma, acute traumatic pancreatitis, and pancreatic pseudocyst. Nuclear scintigraphy is valuable if injury is limited to the liver or spleen. CT is the imaging modality of choice for assessing generalized blunt abdominal trauma as well as evaluating the extent of injuries to the liver, spleen, pancreas, kidneys, and mesentery.     

Three-dimensional spiral CT of craniofacial malformations in children

Objective. To assess the value of three-dimensional CT (3D CT) in the diagnosis and management of suspected paediatric craniofacial malformations.¶Materials and methods. Twenty-eight children (12 girls, 16 boys) with a mean age of 4 years, suffering from craniofacial or cervical malformations, underwent craniofacial spiral CT. 3D reformatting was performed using an independent workstation.¶Results. 3D CT allowed the preoperative evaluation of 16 patients with craniosynostosis and the post-surgical management of 2 patients. 3D CT clearly depicted malformations of the skull base involving the petrous bone in seven patients (four cases of Goldenhar-Gorlin syndrome, one case of Treacher-Collins syndrome and two cases of Crouzon's disease). Four patients with craniofacial clefts were also evaluated. Radiological findings were confirmed by the clinical and intraoperative findings in all patients that underwent surgical treatment. Movement artefacts and “Lego effect” related to abrupt change of cranial vault border were encountered and are discussed.¶Conclusions. 3D CT of the skull can safely and reliably identify paediatric craniofacial malformations involving bone, and it should be used as morphological mapping to help the surgeon in planning surgical treatment.     

Contemporary imaging in abdominal emergencies

Imaging is often a fundamental part in the evaluation of an injured or ill child. A variety of imaging modalities (radiography, angiography/fluoroscopy, sonography, CT, magnetic resonance imaging and scintigraphy) are among the options. CT is worth focused attention because of its usefulness in a variety of emergency department settings, its increasing use, and its potential radiation risks. CT plays an important role in the evaluation of traumatic and nontraumatic abdominal emergencies in children. Therefore, the goal of this paper is to review current imaging approaches and controversies in the evaluation of common acute abdominal emergencies. Through discussion of various modalities, especially CT in evaluation of abdominal pain and trauma, the relative advantages and disadvantages including radiation risk will be reviewed.     

Skull base in trigonocephaly.

The authors present aspects of the skull base in trigonocephalic children. Different patterns on clinical anthropometric investigation, bidimensional computer tomography (CT) scan and three-dimensional (3D) CT scan were studied. We present a series of 27 cases of trigonocephaly operated on in the department of pediatric neurosurgery at the H?pital des enfants La Timone in Marseille since 1975. The skull base has been studied with CT scan in 12 patients and in 5 a 3D CT reconstruction was performed. A control series of 27 children without synostosis has been studied with the same patterns. We have analyzed in 3D, the volume of the anterior fossa related to the orbital volume, the permeability of basal sutures and the spatial orientation of petrous, sphenoid and zygomatic bones. An analysis of nasoethmoidal complex was performed in relation with hypotelorism. CT scan allows the study of the opening of orbital, nasion-pterional angles and the clinopterional angles, as well as nasion-clinoidal and bipterional distances. A computerized analysis of these data compared with normal skull base permits a new approach to these malformations and the necessity for subdivision of trigonocephaly.     

Coincidence FDG-PET in the evaluation of Langerhans' cell histiocytosis: preliminary findings

Background Bone involvement in Langerhans' cell histiocytosis (LCH) is common. Both bone scintigraphy and plain films are used to identify osseous lesions, but lack specificity for disease activity and response to therapy. FDG-PET is a sensitive technique for identifying bone lesions when histiocytes are present.Objective To describe the potential of coincidence FDG-PET (cFDG-PET) for identification of active bone lesions in LCH and to determine whether it can provide more specific information regarding lesional response to therapy than bone scintigraphy or radiography.Materials and methods The clinical data and imaging findings of three patients with osseous lesions of LCH were retrospectively reviewed.Results cFDG-PET identified all active LCH osseous lesions in these patients, differentiated active from healed lesions, and demonstrated normalization of uptake in a treated lesion earlier than bone scintigraphy and radiography.Conclusion cFDG-PET appears to have greater specificity than bone scintigraphy and radiography for the identification of active osseous lesions in LCH. It also may predict response to treatment earlier than conventional techniques. Its use in the evaluation of LCH warrants further study.Presented at the 2002 meeting of the Society for Pediatric Radiology, Philadelphia, Pennsylvania     

Skull base growth in craniosynostosis

Although considerable scientific work has been published on the role of the skull base in craniosynostosis, the changes with age throughout childhood have not been fully outlined. The realisation that little attention has been paid to the posterior skull in craniosynostosis, resulted in renewed interest in skull base growth. The availability of computer-based image analysis provides a new accurate method of study in three dimensions. Using three-dimensional visualisation techniques, 34 points of the skull base were identified on CT scans of 50 children with craniosynostosis of various types, aged from 1 month to 5 years. Several distances and angles between the various landmarks were measured in an attempt to quantify the growth of skull fossae with age. Comparisons were made with normal controls. In children with craniosynostosis, the anterior fossa was overdeveloped in the males, whereas in the females remained underdeveloped throughout the first 2 years of life. The body of the sphenoid showed moderate underdevelopment in the first 2 years in both sexes, the effect being more prominent in the males. The middle fossae showed overdevelopment in both sexes in the first 2 years of life. The posterior fossa was underdeveloped in both sexes in the first 2 years of life, the effect being more prominent in the females. Craniosynostosis seems to affect both sexes to a similar degree, but there are regional differences in the growth pattern. Better understanding of the normal growth pattern of the skull base and the effect of craniosynostosis upon it may assist our approach to surgical treatment and in particular the role of anterior and posterior skull expansive surgery.     

Clinical indicators of intracranial lesion on computed tomographic scan in children with parietal skull fracture

We conducted a review of 98 cases of pediatric traumatic parietal skull fracture in which computed tomographic (CT) scans of the head were obtained during a seven-year period. There were 69 instances of an associated intracranial lesion identified in 47 children, including parenchymal injury (23), epidural hematoma (17), subdural hematoma (11), cerebral edema (ten), and subarachnoid hemorrhage (eight). Compared with 51 other children with parietal skull fracture and normal CT scans, the clinical characteristics significantly associated with the presence of an intracranial lesion were symptoms of neurologic dysfunction (loss or altered level of consciousness and/or seizure activity), neurologic compromise on examination (altered level of consciousness and/or focal deficit), or complicated skull fracture (bilateral, diastatic, and/or depressed). Of the 47 children with an intracranial lesion on CT scan, 44 had at least one of these significant clinical characteristics (sensitivity, 93%; specificity, 50%; positive-predictive value, 62%; and negative-predictive value, 96%). Children who sustain traumatic parietal skull fracture commonly experience associated intracranial injury. Those with evidence of neurologic deficit or complicated skull fracture are at particularly high risk, and should receive cranial CT scan evaluation.     

Craniosynostosis associated with neural tube defects: is there a causal association?

The majority of primary craniosynostosis cases are sporadic. Very few articles in the literature have described cases of primary craniosynostosis associated with neural tube defects (NTDs). This co-occurrence has been seen by most authors as just a coincidence. The authors report a clinical series of 4 patients of primary craniosynostosis associated with NTDs treated at their center. Among these 4 cases, 2 had lumbosacral myelomeningocele, 1 frontoethmoidal encephalocele and 1 had occipital encephalocele. Although the co-occurrence of craniosynostosis and NTD is said to be rare, there seems to exist a justified underlying explanation for the same. The NTD causes a decrease in intracranial pressure due to egress of cerebrospinal fluid in the malformed sac that results in a deficient cerebral impulse for cranial growth that might stimulate premature sutural fusion. Thus, all patients with NTDs should be thoroughly evaluated for this association and for possible surgical management.     

Evaluation of renal parenchyma in children by DMSA scintigraphy, X-ray computed tomography and intravenous urography

The demonstration of diminished or scarred renal parenchyma in children is often the decisive factor in determining the future management of children with urinary tract malformations. Renal scintigraphy using technetium 99m-labelled dimercaptosuccinic acid (DMSA), computed tomography (CT) and intravenous urography (IU) were used to evaluate the renal parenchyma prior to ureter re-implantation in a series of 13 children. Their ages ranged from 5 months to 3 years 8 months. The indication for operation was ureteric reflux in 8 children and distal ureteric stenosis in 5. CT was performed on a Toshiba TCT-80 scanner. Renal scintigraphy was performed 3 hours after intravenous injection of DMSA. Compared with IU, DMSA imaging gave more information about the renal parenchyma in 6 children, gave equal information in 6 and less in 1. Compared with CT, DMSA imaging gave more information in 2 children, was equally informative in 8 and less so in 3. Accordingly, DMSA imaging was judged to be more sensitive than IU and as sensitive as CT. DMSA imaging can be used both for the initial evaluation and for follow-up assessment of renal parenchymal damage.     

Blunt abdominal trauma and hollow viscus injury in children: the diagnostic value of plain radiography

Although blunt abdominal trauma is common in children, the G-I tract is involved only rarely. However, this paper deals with a series of 24 cases in whom a hollow viscus was injured. We present the radiological and operative findings as well as the results of subsequent radiological workup in patients who had no surgery. Contrary to the recent trend which stresses early abdominal CT as critically important for guiding treatment we have retained a more conservative attitude. Our present protocol includes plain radiography frequently combined with sonographic screening of the abdomen. In patients with stable vital signs and when necessary, we follow up with scintigraphy. CT is reserved for complicated cases with obscure clinical manifestations which do not fit the plain radiological findings and also cases running an unexpected course.     

Evaluation of Renal Parenchyma in Children by DMSA Scientigraphy, X-ray Computed Tomography and Intravenous Urography

ABSTRACT. The demonstration of diminished or scarred renal parenchyma in children is often the decisive factor in determining the future management of children with urinary tract malformations. Renal scintigraphy using technetium 99m-labelled dimercaptosuccinic acid (DMSA), computed tomography (CT) and intravenous urography (IU) were used to evaluate the renal parenchyma prior to ureter re-implantation in a series of 13 children. Their ages ranged from 5 months to 3 years 8 months. The indication for operation was ureteric reflux in 8 children and distal ureteric stenosis in 5. CT was performed on a Toshiba TCT-80 scanner. Renal scintigraphy was performed 3 hours after intravenous injection of DMSA. Compared with IU, DMSA imaging gave more information about the renal parenchyma in 6 children, gave equal information in 6 and less in 1. Compared with CT, DMSA imaging gave more information in 2 children, was equally informative in 8 and less so in 3. Accordingly, DMSA imaging was judged to be more sensitive than IU and as sensitive as CT. DMSA imaging can be used both for the initial evaluation and for follow-up assessment of renal parenchymal damage.