Relationship between cranial mechanics and dysmorphic dentofacial characteristics: a cross-sectional study.

AIMS: The aim of the present study was to evaluate if cranial dysfunctions felt by osteopaths could correlate with sagittal dysmorphologies diagnosed by orthodontists, using cephalometric traces in the sagittal plane. Metholology: One hundred and six children between 6 and 12 years old (42 boys and 64 girls) were tested by an osteopath to determine if the cranial movement felt was considered to be eased in flexion or extension. To test reproducibility intra-operator, 27 randomly selected subjects were tested twice, at a one-month interval by the same osteopath before the start of their orthodontic treatment. These tests were then correlated with a cephalometric analysis of the sagittal plane to determine what type of dysmorphology existed, if any, as well as the angle of the spheno-occipital synchondrosis (SOS). RESULTS: Practitioners systematically found more cranial movement in extension for all the bones in patients in skeletal class II than in the others. Similarly, they systematically found more cranial movement in flexion in patients in skeletal class III than in the other skeletal classes. However, there was no significant difference found in SOS angulation between skeletal classes I, II, and III. DISCUSSION: This study tends to confirm the correlation, described previously by orthodontists, between the mobility of the bones of the cranial vault and dysmorphic dentofacial characteristics in the sagittal plane. Anomalies during development could lead to the typical cranial characteristics of flexion or extension. As such, these situations could be related to skeletal classes III and II respectively.

Cryoablation for the treatment of occipital neuralgia.

BACKGROUND: Treatment of occipital neuralgia (ON) can be complex, though many treatment options exist. Cryoablation (CA) is an interventional modality that has been used successfully in chronic neuropathic conditions and is one such option. OBJECTIVE: To study and evaluate the efficacy and safety of cryoablation for treatment of ON. STUDY DESIGN: Retrospective evaluation. SETTING: Academic university-based pain management center. METHODS: All patients received local anesthetic injections for ON. Patients with greater than or equal to 50% relief and less than 2 week duration of relief were treated with CA. RESULTS: Thirty-eight patients with an average age of 49.6 years were included. Of the 38 patients, 20 were treated for unilateral greater ON, 10 for unilateral greater and lesser ON, and 8 for bilateral greater ON. There were 10 men and 28 women, with an average age of 45.2 years and 51.1 years, respectively. The average relief for all local anesthetic injections was 71.2%, 58.3% for patients who reported 50 - 74% relief (Group 1) and 82.75% for patients who reported greater than 75% relief (Group 2). The average improvement of pain relief with CA was 57.9% with an average duration of 6.1 months overall. Group 1 reported an average of 45.2% relief for an average of 4.1 months with CA. In comparison, Group 2 reported an average of 70.5% relief for 8.1 months. The percentage of relief (P = 0.007) and duration of relief (P = 0.0006) was significantly improved in those reporting at least 75% relief of pain with local anesthetic injections (Group 2 vs Group 1). Though no significance in improvement from CA was found in men, significance was seen in women with at least 75% benefit with local anesthetic injections in terms of duration (P = 0.03) and percentage (P = 0.001) of pain relief with CA. The average pain score prior to CA was 8 (0 - 10 visual analog scale, VAS), this improved to 4.2, improvement of 3.8 following CA at 6 months (P = 0.03). Of the 38 patients, 3 (7.8%) adverse effects were seen. Two patients reported post procedure neuritis and one was monitored for procedure-related hematoma. LIMITATIONS: Study limitations include the retrospective nature of the study. Additionally, only the percentage of relief, pain score, and duration of relief were collected. CONCLUSIONS: CA is safe, and should be considered in patients with ON. KEY WORDS: Cryoablation, cryoanalgesia, occipital neuralgia, treatment, adverse effects.

The effects of tissue-non-specific alkaline phosphatase gene therapy on craniosynostosis and craniofacial morphology in the FGFR2C342Y/+ mouse model of Crouzon craniosynostosis.

OBJECTIVES: Craniosynostosis, the premature fusion of cranial bones, has traditionally been described as a disease of increased bone mineralization. However, multiple mouse models of craniosynostosis display craniosynostosis simultaneously with diminished cranial bone volume and/or density. We propose an alternative hypothesis that craniosynostosis results from abnormal tissue mineralization through the downregulation of tissue-non-specific alkaline phosphatase (TNAP) enzyme downstream of activating mutations in FGFRs. MATERIAL AND METHODS: Neonatal Crouzon (FGFRC342Y/+) and wild-type (FGFR+/+) mice were injected with lentivirus to deliver a recombinant form of TNAP. Mice were sacrificed at 4 weeks postnatal. Serum was collected to test for alkaline phosphatase (AP), phosphorus, and calcium levels. Craniofacial bone fusion and morphology were assessed by micro-computed tomography. RESULTS: Injection with the TNAP lentivirus significantly increased serum AP levels (increased serum AP levels are indicative of efficient transduction and production of the recombinant protein), but results were variable and dependent upon viral lot and the litter of mice injected. Morphological analysis revealed craniofacial form differences for inferior surface (p=0.023) and cranial height (p=0.014) regions between TNAP lentivirus-injected and vehicle-injected Crouzon mice. With each unit increase in AP level, the odds of lambdoid suture fusion decreased by 84.2% and these results came close to statistical significance (p=0.068). CONCLUSION: These results suggest that TNAP deficiency may mediate FGFR2-associated craniosynostosis. Future studies should incorporate injection of recombinant TNAP protein, to avoid potential side effects and variable efficacy of lentiviral gene delivery.

Occipital nerve stimulation for refractory occipital pain after occipitocervical fusion: expanding indications.

BACKGROUND: Occipital nerve stimulation is being used for various pain syndromes. Here, we expand its use for the treatment of refractory occipital pain after occipitocervical fusion. CASE DESCRIPTION: We describe a case of occipital neuralgia in a 60-year-old man following posterior occipitocervical fusion. The maximum pain intensity was rated 9/10 on the visual analogue scale (VAS). Since pain proved to be refractory to analgetic medication, two quadripolar electrodes (Resume II, Medtronic) were implanted in the occipital region to stimulate the occipital nerve bilaterally. The patient experienced a dramatic response during test stimulation for 10 days with externalized electrodes, and a pacemaker (Synergy, Medtronic) was connected to the electrodes. While on chronic stimulation (bipolar 6 V, 210 mus, 130 Hz) improvement of pain was maintained, reflected by a decrease in the VAS score to 1/10 at 12 months of follow-up. CONCLUSION: Occipital nerve stimulation for medical refractory occipital neuralgia after occipitocervical fusion is an effective method expanding the indications for its use.

Iatrogenic pyogenic osteomyelitis of C-1 and C-2 treated with transoral decompression and delayed posterior occipitocervical arthrodesis. Case report.

The authors describe a case of osteomyelitis of the craniocervical junction caused by iatrogenic infection of the spine during corticosteroid injection therapy. This 58-year-old diabetic man presented with acute exacerbation of neck pain that had began 4 months prior to admission. He did not experience the associated fever, chills, or sweats, but he did notice transient weakness in the right upper extremity. A computed tomography (CT) scan of the cervical spine demonstrated a destructive process involving the odontoid and the left occipitocervical and atlantoaxial joints that was not present on a CT obtained 2 months earlier, just before trigger-point and left-sided C1-2 facet joint corticosteroid injections. A diagnosis of staphylococcal osteomyelitis was made, and initial treatment with external immobilization and appropriate antibiotic therapy failed to control radiographically demonstrated and clinical progression. The patient was successfully treated using staged anterior decompression and posterior instrumented fusion with prolonged antibiotic therapy. To the authors' knowledge this case is the first reported instance of iatrogenic pyogenic osteomyelitis of the craniocervical junction successfully treated with anterior decompression and delayed posterior arthrodesis.

Thyroid follicular carcinoma metastasized to the lung, skull, and brain 12 years after initial treatment for thyroid gland--case report.

A 65-year-old woman presented with multiple metastases from thyroid follicular carcinoma to the lung, skull, and brain. The skull and brain tumors had been successfully treated by surgery, thyroxine supplementation, and radiosurgery until she died of sudden intracerebral hemorrhage which had no connection with tumor treatment. The lung tumor was treated by conventional irradiation and radioactive ablation. Well-differentiated thyroid carcinoma is a slowly progressive tumor. Follicular carcinoma is thought to have the most optimistic prognosis even with metastases to the lymph nodes and lung. Radioactive ablation using iodine-131 is widely used to treat the primary and/or metastatic lesion. However, the prognosis for patients with brain metastases is poor. Intracranial metastasis of this tumor is rare, but has a mean posttreatment survival of around 12 months. Surgical excision of the metastatic intracranial lesion may be the only effective treatment.

Brachycephaly and external pressure.

Browsing through some literatures, I happened to find out some records on the rite of imitation of the child's head to brachycephaly. In 1976, two skulls showing extraordinary forms were excavated out of an ancient tomb at Yean village of Daedong township in Kimhae County in Kyungnam Province, the Republic of Korea. The deformation in Yean village is judged to be an intentional frontal flattening. The frontal bone of deformed individuals is remarkably flattened by a primary deforming force. The parietal bone expands laterally and superoposteriorly as a major compensatory growth. The lambdoid or occipital flattening induced by the counterforce is light and unstable. In the facial skeleton, the maxilla shifts downward as a minor compensatory growth. These findings might be substantially correspondent to the custom in the southern area of Korea in the 4th century A.D. paragraphed by Han chuan of Wei-dynasty record, History of the Three Kingdoms. It described that the people gave a pressure onto the child's forehead with stones in order to mold it for preference. Flatheaded meant "free" among the Chinook and Salish tribes of American Indians. Babies of slaves were denied the year-long beauty treatment--a board tied to the infants' padded skull--that permanently flattened the foreheads of tribal members. Referring to these records, I presume that brachycephaly can be induced by direct extrinsic pressure at least until the soft infant's head gets hard.

Occipital lesions: a possible cost of cradleboards.

This examination of a Mimbres-Mogollon pueblo skeletal sample reveals a surprising percentage of individuals with occipital lesions. Each lesion is located in the approximate center of the squama immediately superior to the external occipital protuberance. Notably, no child over the age of 1 year exhibits a lesion that would have been active at the time of death, but a number of older children and adults exhibit evidence of healed lesions in this same area on the occipital. The restricted nature of these lesions, in terms of both their locations and ages of those actively affected, suggests that the use of cradleboards may have been at least a contributing, if not initiatory, factor in their creation. Specifically, this study suggests that the pressure and friction of an infant's head against a cradleboard may have 1) produced ischemic ulcers, 2) produced the conditions favorable for bacterial infections such as impetigo or carbuncles, or 3) complicated the treatment of other infections appearing on the back of the scalp.

Cranial imaging in autosomal recessive osteopetrosis. Part II. Skull base and brain.

The authors reviewed cranial imaging studies (radiographs, computed tomographic scans, and magnetic resonance [MR] images) in 13 infants and children with the autosomal recessive form of osteopetrosis to characterize patterns of skull base, brain, and cranial nerve involvement at presentation and with progression of disease. Marked sclerosis and deposition of osteopetrotic bone was noted along the anterior (but not posterior) occipitomastoid suture (n = 8), at the basioccipital-exoccipital synchondrosis (n = 9), and along the sphenooccipital synchondrosis (n = 8). Endobones, presumably representing unresorbed primitive ossification centers, were seen in the sphenoidal body and basioccipital bone in 11 of the 13 patients. Marked cupping at the basioccipital-exoccipital synchondrosis was observed in three. Neurologic deficits included blindness (n = 11), conductive hearing loss (n = 11), and facial nerve palsies (n = 4). Delayed myelination was seen with MR imaging in two of five retarded infants, including one with a documented coexisting neuronal storage defect. Prominent extracerebral cerebrospinal fluid spaces were present over the frontal lobes in five of the eight developmentally normal patients, representing either subclinical parenchymal disease or a phenomenon related to discordant growth rates between skull and brain.

Cribra orbitalia in the aborigines of Hawaii and Australia.

Cranii of 53 Hawaiian aboriginal infants and children, and 45 from Austrailian aboriginal children were inspected. Cribra orbitalia was present in 22.8% of the former and 26.6% of the latter; osteoporotic pitting (symmetrical osteoporosis; porotic hyperostosis) was also present in the latter. The frequency compares favorably with that found in pre-Columbian North American Pueblo Indians, 24.7%. It is associated with a widespread skeletal involvement suggestive of an active bone marrow. The findings support the concept that cribra orbitalia is related to symmetrical osteoporosis and that it may be associated with a blood disorder.