Physiological Changes and Clinical Implications of Syndromic Craniosynostosis

Syndromic craniosynostosis has severe cranial stenosis and deformity, combined with hypoplastic maxillary bone and other developmental skeletal lesions. Among these various lesions, upper air way obstruction by hypoplastic maxillary bone could be the first life-threatening condition after birth. Aggressive cranial vault expansion for severely deformed cranial vaults due to multiple synostoses is necessary even in infancy, to normalize the intracranial pressure. Fronto-orbital advancement (FOA) is recommended for patients with hypoplastic anterior part of cranium induced by bicoronal and/or metopic synostoses, and posterior cranial vault expansion is recommended for those with flattening of the posterior part of the cranium by lambdoid synostosis. Although sufficient spontaneous reshaping of the cranium can be expected by expansive cranioplasty, keeping the cranial bone flap expanded sufficiently is often difficult when the initial expansion is performed during infancy. So far distraction osteogenesis (DO) is the only method to make it possible and to provide low rates of re-expansion of the cranial vault. DO is quite beneficial for both FOA and posterior cranial vault expansion, compared with the conventional methods. Associated hydrocephalus and chronic tonsillar herniation due to lambdoid synostosis can be surgically treatable. Abnormal venous drainages from the intracranial space and air way obstruction should be always considered at any surgical procedures. Neurosurgeons have to know well about the managements not only of the deformed cranial vault and the associated brain lesions but also of other multiple skeletal lesions associated with syndromic craniosynostosis, to improve treatment outcome.

Distraction Osteogenesis Update: Introduction of Multidirectional Cranial Distraction Osteogenesis

In this review, we discuss in detail our current procedure for treating craniosynostosis using multidirectional cranial distraction osteogenesis (MCDO). The MCDO method allows all phenotypes of skull deformity to be reshaped by distraction osteogenesis, except in patients who are 5 months of age or younger and patients with posterior cranial vault problems. We report the results of clinical data of 36 children with craniosynostosis who underwent MCDO between 2005 and 2014 in our institute. This method has the following benefits, such as a high flexibility of reshaping, shorter treatment period and less invasive secondary intervention. We also discuss the other distraction osteogenesis techniques that are used to treat craniosynostosis and compare them with MCDO. The preferred procedure for correction of craniosynostosis may depend on the patient's age, the extent of deformity, and the extent of correction achievable by surgery. We can arrange the combinations of various methods according to the advantage and disadvantage of each technique.

Effectiveness of Helmet Cranial Remodeling in Older Infants with Positional Plagiocephaly

BACKGROUND: Management of positional plagiocephaly by wearing a cranial molding helmet has become a matter of growing medical interest. Some research studies reported that starting helmet therapy early (age 5 to 6 months) is important and leads to a significantly better outcome in a shorter treatment time. The aim of the present study was to evaluate the effectiveness of cranial remodeling treatment with wearing helmet for older infants (> or =18 months). METHODS: We conducted a retrospective study of 27 infants with positional plagiocephaly without synostosis, who were started from 2008 to 2012. Every child underwent a computerized tomography (CT) before starting helmet therapy to exclude synostosis of the cranial sutures and had CT performed once again after satisfactory completion of therapy. Anthropometric measurements were taken on using spreading calipers in every child. The treatment effect was compared using cranial vault asymmetry (CVA) and the cranial vault asymmetry index (CVAI), which were obtained from diagonal measurements before and after therapy. RESULTS: The discrepancy of CVA and CVAI of all the patients significantly decreased after cranial molding helmet treatment in older infants (> or =18 months) 7.6 mm from 15.6 mm to 8 mm and 4.51% from 9.42% to 4.91%. Six patients had confirmed successful outcome, and all subjects were good compliance patients. The treatment lasted an average of 16.4 months, was well tolerated, and had no complication. Additionally, the rate of the successful treatment (final CVA < or =5 mm) significantly decreased when the wearing time per was shorter. CONCLUSION: This study showed that treatment by cranial remodeling orthosis was effective if the patient could wear the helmet longer and treatment duration was somewhat longer than in younger patients, well tolerated in older infants and had no morbidity. This therapeutic option is available and indicated in these older infants before other cranial remodeling surgery.

Effectiveness of Helmet Cranial Remodeling in Older Infants with Positional Plagiocephaly

BACKGROUND: Management of positional plagiocephaly by wearing a cranial molding helmet has become a matter of growing medical interest. Some research studies reported that starting helmet therapy early (age 5 to 6 months) is important and leads to a significantly better outcome in a shorter treatment time. The aim of the present study was to evaluate the effectiveness of cranial remodeling treatment with wearing helmet for older infants (> or =18 months). METHODS: We conducted a retrospective study of 27 infants with positional plagiocephaly without synostosis, who were started from 2008 to 2012. Every child underwent a computerized tomography (CT) before starting helmet therapy to exclude synostosis of the cranial sutures and had CT performed once again after satisfactory completion of therapy. Anthropometric measurements were taken on using spreading calipers in every child. The treatment effect was compared using cranial vault asymmetry (CVA) and the cranial vault asymmetry index (CVAI), which were obtained from diagonal measurements before and after therapy. RESULTS: The discrepancy of CVA and CVAI of all the patients significantly decreased after cranial molding helmet treatment in older infants (> or =18 months) 7.6 mm from 15.6 mm to 8 mm and 4.51% from 9.42% to 4.91%. Six patients had confirmed successful outcome, and all subjects were good compliance patients. The treatment lasted an average of 16.4 months, was well tolerated, and had no complication. Additionally, the rate of the successful treatment (final CVA < or =5 mm) significantly decreased when the wearing time per was shorter. CONCLUSION: This study showed that treatment by cranial remodeling orthosis was effective if the patient could wear the helmet longer and treatment duration was somewhat longer than in younger patients, well tolerated in older infants and had no morbidity. This therapeutic option is available and indicated in these older infants before other cranial remodeling surgery.

Primary Malignant Lymphoma of the Cranial Vault with Extra- and Intracranial Extension

Bone involvement is a common finding in many types of lymphomas, particularly in advanced stages. However, cranial vault affliction has been regarded as an exceedingly rare presentation. Here, we report the case of a patient with cranial vault lymphoma who presented with a scalp mass. An 81-year-old woman presented with a gradually growing and non-painful frontal scalp mass that she noticed one month before admission. It was a flatly elevated, round mass measuring about 6x4x4 cm. Computed tomography and magnetic resonance imaging of the brain revealed a contrast-enhancing intracranial extradural mass at the counter-location of the scalp mass. The superior sagittal sinus was involved at the tumor site. Cerebral angiography showed that the tumor feeding vessels originated from the bilateral external carotid arteries. An operation was performed and the tumors were removed together with the involved bone. The pathologic diagnosis was malignant diffuse large B-cell type lymphoma. The patient was transferred to the Hemato-Oncology department for chemotherapy. Primary lymphoma of the cranial vault with scalp mass is very rare but it should be considered in the differential diagnosis of scalp masses. Although the results of reported cases are variable, the combination of surgery, radiation, and chemotherapy appears to offer favorable outcomes.

Metástasis de calota / Calvarial metastasis

Objective. To describe five cases of cranial vault metastasis. Materials and methods. Clinical records and images of five patients with cranial vault metastasis were reviewed at the “Hospital de Clinicas” and “Hospital Español” from Buenos Aires, between January 2009 to June 2010. Results. Case 1, 63 years old female, complains for headache with aleatory response to medical therapy; MRI: right occipital bone lession; Pathological Finding: breast carcinoma metastasis. Case 2, 70 years old female, complains for palpable lession of the scalp; MRI: right fronto-parietal intraxial lession and right parietal cranial vault lession; Pathological Finding: endometrial carcinoma metastasis (primary, diagnosed 4 months before). Case 3, 76 years old female, with palpable lession in the scalp; MRI: left parietal cranial vault lesion. Pathological Finding: kidney carcinoma metastasis (primary, diagnosed in 2008). Case 4, 50 years old female and Case 5, 78 years old male, both complains for bone pain; serological findings and marrow bone biopsy compatible with multiplemyeloma; screening of the long bones and cranial vault demonstrate evidence of bone infiltration. Conclusion. Metastasis at the cranial vault often presents with headache or pain upon inspection of a palpable scalp lession. Images of the CNS involves CT scan (delimitate lession and differentiates litic from blastic metastasis) and MRI (shows the degree of dural and brain infiltration). Prevalence of cranial vault metastasis is greater than brain metastasis, but this underdiagnosis is due that the first are frequently course without symptoms.

Modified model of sub-critical size cranial defect in the rabbit / Modelo modificado de defecto craneal de tamaño subcrítico de conejo

In medical sciences, any experimental animal model should be reproducible and adequate to the purpose of simulated human physiological response. In bone injury response research, when bone substitutes are in use, it is of primary importance that studied defects fail to heal unless treated with the tissue engineering therapy under study. This failure defines the concept of "critical size defect" (CSD) wich has different limits according to the animal species used and the location of the defect. Although this is a basic concept, when the aim of the study is to investigate the interface Bone-Biomaterial, it is of primary importance to obtain as much contact area as possible. In order to do so, we propose a modified surgical approach to the classical bi-parietal round sub-critical defect model in rabbit vault.

En las ciencias médicas, cualquier modelo animal de experimentación debe ser reproducible y ajustado al propósito de simular la respuesta fisiológica humana. En la investigación de la respuesta a la lesión ósea, cuando son utilizados sustitutos óseos, es de gran importancia el estudio de defectos que no logran sanar, a menos que sean tratados con terapia de ingeniería de tejidos. Este fracaso define el concepto de "defecto de tamaño crítico" (CDS) el cual tiene límites diferentes según la especie animal utilizada y la ubicación del defecto. Aunque se trata de un concepto básico, cuando el objetivo del estudio es investigar la interfaz hueso-biomaterial, es de primordial importancia obtener la máxima superficie de contacto que sea posible. Para ello, se propone una modificación en el enfoque quirúrgico del modelo clásico de defecto de tamaño subcrítico biparietal en la bóveda de conejo.

Chronic subdural empyema and cranial vault osteomyelitis due to Salmonella paratyphi A.

Intracranial infections, especially subdural empyema, due to salmonella are rare. Subdural empyema caused by Salmonella paratyphi A has been documented only once earlier in the literature. Hence, we report a case of subdural empyema and osteomyelitis of cranial vault due to S. paratyphi A. A 42- year-old male presented with headache and purulent discharge from right parietal burr hole wound site. Patient gave a history of head injury two years ago. He underwent burr hole evacuation of chronic subdural haematoma, excision of outer membrane and right parietal craniectomy. The cultures grew S. paratyphi A. Recovery was uneventful following surgical intervention and antibiotic therapy.

Reconstrucción de la bóveda craneana con implante de poli-éter-éter-cetona diseñado por computadora: informe de un caso / Cranial vault reconstruction using computer-designed polyetheretherketone (PEEK) implant: case report

Introducción: La reconstrucción de los huesos del cráneo es un procedimiento complejo y representa un reto para el grupo médico tratante; generalmente se realiza en pacientes con pérdida de la bóveda craneana secundaria a procesos infecciosos crónicos no controlados o por osteorradionecrosis, lo que implica mayor posibilidad de fracaso o rechazo de los materiales utilizados para la reparación del defecto. La selección del material para sustituir la bóveda craneana es difícil debido a la diversidad de productos existentes; el material debe ser inerte, ligero, fácil de colocar y adaptable al defecto, con lo cual se ofrece mejores resultados estéticos y funcionales. Con el diseño por computadora del implante es posible ofrecer un implante individual y específico para cada defecto y paciente. Caso clínico: Mujer con diagnóstico de estesioneuroblastoma tratada con resección craneofacial anterior y radioterapia; como complicación presentó osteomielitis y osteorradionecrosis y, consecuentemente, pérdida de la bóveda craneana en la región frontal; este defecto fue reconstruido con un implante de poli-éter-éter-cetona diseñado por computadora con base en el defecto evaluado por tomografía computarizada. Se muestran los resultados obtenidos. Conclusiones: El implante diseñado por computadora realizado con poli-éter-éter-cetona es una alternativa segura, fácil de usar y con gran adaptabilidad a los defectos de la bóveda craneana.

BACKGROUND: Reconstruction of the bones of the skull is a complex procedure and represents a challenge for the surgical team. It is generally performed in patients who have loss of the cranial vault secondary to chronic infection or uncontrolled osteoradionecrosis, indicating a greater chance of failure or rejection of the materials used for repair of the defect. Selection of material to replace the cranial vault is complex due to the diversity of existing products. The ideal material is inert, lightweight, easy to fit and adaptable to the defect, offering the best aesthetic and functional results. Computer design of the implant makes this process easier by providing an implant specific to each individual patient and defect. CLINICAL CASE: We report the case of a patient who was diagnosed with esthesioneuroblastoma and was treated with anterior craniofacial resection and radiotherapy. Osteomyelitis and osteoradionecrosis were consequent complications with loss of the cranial vault in the frontal region. The defect was reconstructed with a polyetheretherketone (PEEK) computer-designed implant based on the defect evaluated by computed tomography. Results obtained are shown below. CONCLUSIONS: The PEEK computer-designed implant is a safe and easy to use alternative with great adaptability to cranial vault defects.

Primary Mlignant non-Hodgkin's Lymphoma of Cranial Vault

Primary non-Hodgkin's lymphoma of the cranial vault is very rare. A 55-year-old woman presented with a right parietal subcutaneous scalp lump. Computed tomography(CT) scan showed a right parietal cranial vault lesion with scalp swelling and extradural and intradural involvement. Tumor removal, including portion of involved skull, followed by c ranioplasty with resin were performed. Histologic study revealed the features of a T-cell non-Hodgkin's lymphoma(high grade, large cell, diffuse, immunoblastic type) and there was no systemic involvement. The chemotherapy and radiotherapy were instituted after surgery.

The Clinical Analysis of 22 Cases of Encephalocele

An encepholocele is defined as a herniation of cranial contents through a defect in the skull. Encephaloceles are classified accroding to their contents and location. Encephalocele is a useful general term to refer to common features of the various forms of anomaly, but considerable differences exist in the pathology, treatment and prognosis of encephaloceles at each anatomical location. Improved neuroimaging facilities, especially MRI, make it easy not only to detect the encephalocele including its contents and location, but also to get preoperative informations. We have reviewed a total of 22 patients with encephalocele whom we have experienced from 1986, July to 1990, June. Twelve were at occipital location, six at cranial vault, three at cranial base, one at frontoethmoidal location. The size of cranium bifidum and herniating sac of the cranial vault and occipital location is larger than that of frontobasal location. The incidence of associated hydrocephalus is in order of occipital, cranial vault, frontobasal form, and its prognosis is also better in frontobasal form than in occipital of cranial vault form.