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The Journal of International Advanced... Oct 2023This study aimed to discuss 3 cases of congenital cholesteatoma located posterior to the sigmoid sinus, with no/minimal involvement of mastoid, and compare them with... (Review)
Review
BACKGROUND
This study aimed to discuss 3 cases of congenital cholesteatoma located posterior to the sigmoid sinus, with no/minimal involvement of mastoid, and compare them with cases presented in the literature to better define this rare entity.
METHODS
Retrospective chart analysis of 3 congenital cholesteatomas located posterior to the sigmoid sinus treated surgically in 2 skull-base centers and literature review. Though congenital cholesteatoma can arise outside the middle ear, only a few cases presenting in the retrosigmoid occipital bone have been described earlier.
RESULTS
In all 3 patients, there was a delay in the presentation, as symptoms were nonspecific or lacking, leading in 1 case to severe complications. Computed tomography and magnetic resonance imaging, especially diffusion-weighted imaging scans, allowed accurate diagnosis and surgical planning. Surgery happened to be challenging due to the tight adherence of the cholesteatoma to the thinned dural surface. Complete excision was achieved in all the cases.
CONCLUSION
Congenital cholesteatoma located posterior to the sigmoid sinus is a rare entity and is even more exceptional after a critical review of the literature. Complete excision is quintessential to prevent intradural extension or infection. The most important surgical issue is the management of the posterior fossa dura and the sigmoid sinus. We recommend meticulous dissection with slow peeling of the epithelial lining from the dura. Bipolar coagulation of the dura may help in avoiding recidivism. Moreover, cerebrospinal fluid (CSF) leak during dissection has to be avoided as long as possible, because the loss of tension of the already thinned dura makes its peeling particularly difficult.
Topics: Humans; Retrospective Studies; Cholesteatoma; Ear, Middle; Mastoid; Cerebrospinal Fluid Leak; Cholesteatoma, Middle Ear
PubMed: 37789626
DOI: 10.5152/iao.2023.22798 -
Turkish Journal of Orthodontics Sep 2023This study assessed the relationship of the fusion stage of spheno-occipital synchondrosis (SOS) with midpalatal (MPS) and zygomaticomaxillary (ZMS) sutures on cone-beam...
Relationship of the Fusion Stage of Spheno-Occipital Synchondrosis with Midpalatal and Zygomaticomaxillary Sutures on Cone-Beam Computed Tomography Scans of Patients Aged Between 7 and 21 Years.
OBJECTIVE
This study assessed the relationship of the fusion stage of spheno-occipital synchondrosis (SOS) with midpalatal (MPS) and zygomaticomaxillary (ZMS) sutures on cone-beam computed tomography (CBCT) scans of 7 to 21-year-old patients.
METHODS
This cross-sectional study evaluated the CBCT scans of 176 patients between 7 and 21 years presenting to a maxillofacial radiology clinic. The fusion stage of SOS was determined using a five-stage classification system. The percentage of opening depth of MPS was measured on two middle coronal cuts in the anterior and posterior half of the palate. To assess ZMS, suture fusion was evaluated in four age groups in the axial cut visualizing its maximum length. Data were analyzed using the Kruskal-Wallis, Mann- Whitney U, and Bonferroni tests and regression models.
RESULTS
The percentage of MPS opening significantly decreased in both the anterior and posterior halves with age (p<0.002). With an increase in SOS grade, the percentage of MPS opening in both the anterior and posterior halves significantly decreased (p<0.001). By an increase in the ZMS stage, the SOS grade significantly increased (r=0.565, p<0.001).
CONCLUSION
The MPS opening percentage in the anterior and posterior halves decreased with age, with a greater reduction in the posterior half. A significant inverse correlation exists between the SOS fusion stage and the percentage of MPS opening. In SOS grades I-III, the mean percentage of MPS opening was 100% in all age groups (with the highest frequency of ZMS stage I), indicating a higher chance of success for orthodontic treatments such as rapid maxillary expansion in these individuals.
PubMed: 37782008
DOI: 10.4274/TurkJOrthod.2022.2022.65 -
The Canadian Veterinary Journal = La... Oct 2023This report describes the clinical, computed tomography, and magnetic resonance imaging findings for a Jacob sheep lamb diagnosed with meningoencephalocele and...
This report describes the clinical, computed tomography, and magnetic resonance imaging findings for a Jacob sheep lamb diagnosed with meningoencephalocele and supernumerary ectopic limb. Key clinical message: This case demonstrates the utility of tomographic imaging in diagnosing congenital malformations in sheep and can be used to assess the extent of the lesion. This may help to determine any viable treatment, or, as in the case presented here, determine that the extent of the lesion precludes surgical intervention.
Topics: Animals; Sheep; Encephalocele; Meningocele; Occipital Bone; Extremities; Tomography, X-Ray Computed; Magnetic Resonance Imaging; Sheep Diseases
PubMed: 37780471
DOI: No ID Found -
Medicina (Kaunas, Lithuania) Sep 2023: Before the introduction of griseofluvin, the use of X-ray radiation was the treatment of choice for tinea capitis. More than half a century later various types of...
Reconstruction of Moderately and Severely Atrophic Scalp-A Multicentric Experience in Surgical Treatment of Patients Irradiated for Tinea Capitis in Childhood and Surgical Algorithm.
: Before the introduction of griseofluvin, the use of X-ray radiation was the treatment of choice for tinea capitis. More than half a century later various types of tumors have been found to be associated with childhood irradiation due to tinea capitis, most commonly cancers of the head and neck, as well as brain tumors. The often unusually aggressive and recurrent nature of these tumors necessitates the need for repeated surgeries, while the atrophic skin with an impaired vascular supply due to radiation often poses an additional challenge for defect reconstruction. We present our experience in the surgical treatment of such patients. : This is a retrospective cohort study. In this study, 37 patients treated for acquired defects of the scalp with a history of irradiation therapy due to tinea capitis in childhood were included in this study, 24 male and 13 female patients. The mean age at the first appointment was 60.6 ± 7.8, with the youngest included patient being 46 and the oldest being 75 years old. Patients' characteristics, surgical treatment, and complications were analyzed and a reconstructive algorithm was developed. : Local flaps were used for reconstruction in 34 patients, direct sutures were used in 10 patients and 20 patients received split-thickness skin grafts for coverage of both primary and secondary defects for reconstruction of flap donor sites. One regional flap and one dermal substitute covered by an autologous skin graft were also used for reconstruction. Complications occurred in 43.2% of patients and were significantly associated with the presence of comorbidities ( = 0.001), aseptic bone necrosis ( = 0.001), as well as skin atrophy in frontal, occipital, and parietal region ( = 0.001, = 0.042 and = 0.001, respectively). A significant correlation between major complications and moderate skin atrophy was found only in the parietal region ( = 0.026). : Unfortunately, many protocols developed for scalp reconstruction are not applicable in the setting of severe or diffuse scalp skin atrophy associated with high tumor recurrence rate and radiation-induced vascular impairment, such as in tinea capitis patients in Serbia. An algorithm has been developed based on the authors' experience in managing these patients.
Topics: Humans; Female; Male; Aged; Scalp; Retrospective Studies; Neoplasm Recurrence, Local; Tinea Capitis; Atrophy; Algorithms
PubMed: 37763798
DOI: 10.3390/medicina59091678 -
Journal of Neurosurgery. Case Lessons Aug 2023Tenosynovial giant cell tumor (TGCT) occurs most commonly in the appendicular skeleton and is only rarely found in the vertebral column. Lesions of the craniocervical...
BACKGROUND
Tenosynovial giant cell tumor (TGCT) occurs most commonly in the appendicular skeleton and is only rarely found in the vertebral column. Lesions of the craniocervical junction are particularly rare, with only 4 cases reported in the literature. The authors describe the case of a diffuse-type TGCT at the craniocervical junction.
OBSERVATIONS
A patient presented with a 1-year history of right-sided neck pain and bilateral neurological symptoms in the distribution of the right occipital nerve. A 20-mm homogeneously contrast-enhancing mass in the suboccipital and posterior C1 region was discovered on magnetic resonance imaging of the cervical spine. The tumor was operated on via a posterior approach, and gross-total resection (GTR) was achieved. Immunohistochemical (IHC) examination revealed a diffuse-type TGCT. The patient had an uneventful recovery.
LESSONS
TGCT can arise at the craniocervical junction and is easily misdiagnosed because of its rare occurrence. IHC examination of a tumor specimen should be done to confirm the diagnosis. GTR is the objective when treating these tumors, especially when they are the diffuse type, as they have a high recurrence rate. Radiation and small-molecule therapies are viable postoperative therapies if GTR cannot be achieved or in cases of recurrence.
PubMed: 37728324
DOI: 10.3171/CASE23288 -
World Journal of Clinical Cases Sep 2023The mixed lineage leukemia (MLL)-eleven-nineteen lysine-rich leukemia (ELL) fusion gene is a rare occurrence among the various MLL fusion genes. We present the first...
BACKGROUND
The mixed lineage leukemia (MLL)-eleven-nineteen lysine-rich leukemia (ELL) fusion gene is a rare occurrence among the various MLL fusion genes. We present the first case in which myeloid sarcoma (MS) was the only manifestation of adult MLL-ELL-positive acute myeloid leukemia (AML).
CASE SUMMARY
We report a case of a 33-year-old male patient who was admitted in June 2022 with a right occipital area mass measuring approximately 7 cm × 8 cm. Blood work was normal. The patient underwent right occipital giant subscalp mass excision and incisional flap grafting. Immunohistochemistry was positive for myeloperoxidase, CD43 and CD45 and negative for CD3, CD20, CD34, and CD56. The bone marrow aspirate showed hypercellularity with 20% myeloblasts. Flow cytometry showed that myeloblasts accounted for 27.21% of the nucleated cells, which expressed CD33, CD38, and CD117. The karyotype was 46, XY, (11, 19) (q23; p13.1), -12, + mar/46, XY. Next-generation sequencing showed a fusion of MLL exon 7 to exon 2 of ELL. A diagnosis of MLL-ELL-positive AML (M2 subtype) with subcutaneous MS was made.
CONCLUSION
MLL-ELL-positive AML with MS is a rare clinical entity. Additional research is needed to elucidate the molecular mechanisms of the pathogenesis of MS.
PubMed: 37727473
DOI: 10.12998/wjcc.v11.i25.6000 -
Frontiers in Veterinary Science 2023Schistosomus reflexus (SR) is one of the most common congenital anomalies found in cases of cattle dystocia; this disorder occurs mostly in cattle. Congenital anomalies...
Schistosomus reflexus (SR) is one of the most common congenital anomalies found in cases of cattle dystocia; this disorder occurs mostly in cattle. Congenital anomalies such as SR are caused by various genetic and environmental factors, but no specific cause has been elucidated for SR. This study reports a case of SR in a Holstein dairy cattle fetus with congenital anomalies in Korea. Grossly, a distinct spine curvature was observed between the thoracic and lumbar vertebrae, accompanied by a consequential malformation from the sacrum to the occipital bone. Furthermore, the thoracic and abdominal organs were exposed. In computed tomography (CT) images, mild and severe kyphoscoliosis was observed in T1~11 and L1~6, respectively. Additionally, vertebral dysplasia was observed in S1~5 and Cd 1~5. To pinpoint the causal genes and mutations, we leveraged a custom 50K Hanwoo SNP-Chip and the Online Mendelian Inheritance in Animals (OMIA) database. As a result, we identified a nonsense mutation in apoptotic protease activating factor 1 () within HH1 that was associated with a decrease in conception rate and an increase in abortion in Holstein dairy cattle. The genotype of the SR case was A/A, and most of the 1,142 normal Holstein dairy cattle tested as a control group had the genotype G/G. In addition, the A/A genotype did not exist in the control group. Based on the pathological, genetic, and radiological findings, the congenital abnormalities observed were diagnosed as SR.
PubMed: 37671278
DOI: 10.3389/fvets.2023.1238544 -
Medicine Aug 2023Clinically, vertebral osteomyelitis commonly occurs in immunocompromised individuals, such as people with diabetes, immunosuppression, chronic liver disease, and... (Review)
Review
Group A Streptococcus dysgalactiae subspecies equisimilis vertebral osteomyelitis accompanied by progressive atlantoaxial subluxation: A case report and literature review.
RATIONALE
Clinically, vertebral osteomyelitis commonly occurs in immunocompromised individuals, such as people with diabetes, immunosuppression, chronic liver disease, and malignancy. Microbiologically, vertebral osteomyelitis is commonly caused by Staphylococcus aureus; however, Streptococcus dysgalactiae subspecies equisimilis (SDSE) may also potentially cause vertebral osteomyelitis, albeit rarely. Since no case reports have documented the occurrence of SDSE cervical osteomyelitis accompanied by progressive atlantoaxial subluxation, its clinical characteristics remain uncertain. Herein, we report the first case of progressive atlantoaxial subluxation in addition to cervical osteomyelitis due to septic atlantoaxial arthritis caused by SDSE in an immunocompetent individual, and provide a review of the relevant literature.
PATIENT CONCERNS
A 63-year-old man with hypertension but no history of trauma or musculoskeletal disorders presented with worsening neck pain for 1 month without fever. Physical examination revealed neck pain due to neck retroflexion and tenderness with swelling of the upper cervical spine. No neurological deficit was observed. Magnetic resonance imaging revealed low-intensity areas on a T1-weighted image and high-intensity areas on a short tau inversion recovery image at the C2, C5, and C6 vertebral bodies with atlantoaxial subluxation. Two sets of blood culture tests (aerobic and anaerobic) were performed.
DIAGNOSES
The anaerobic blood culture bottle showed the presence of beta-hemolytic pyrrolidonyl arylamidase-negative SDSE expressing Lancefield group A antiserum. Hence, the patient was diagnosed with SDSE cervical osteomyelitis with atlantoaxial subluxation; intensive intravenous ampicillin (2 g every 6 hours) - which is effective against SDSE - was administered.
INTERVENTIONS
Posterior fusion (occipital bone, C4) was performed on day 33 because a follow-up magnetic resonance imaging on day 31 revealed progression of atlantoaxial subluxation with thickened atlantodental soft tissue.
OUTCOMES
The patient's neck pain was completely relieved after treatment with intravenous ampicillin for 6 weeks, followed by oral amoxicillin (1500 mg) daily for an additional 4 weeks. The patient did not experience recurrence or sequelae during the 2-year follow-up period.
LESSONS
SDSE expressing Lancefield group A antiserum can cause afebrile vertebral osteomyelitis and progressive atlantoaxial subluxation due to the occurrence of septic atlantoaxial arthritis in immunocompetent individuals. Spinal instrumentation for vertebral osteomyelitis may be acceptable after 6 weeks of antimicrobial therapy.
Topics: Male; Humans; Middle Aged; Neck Pain; Neck Injuries; Joint Dislocations; Osteomyelitis; Cervical Vertebrae; Ampicillin; Arthritis, Infectious
PubMed: 37653834
DOI: 10.1097/MD.0000000000034968 -
F1000Research 2021Despite several hypotheses, our understanding of syringomyelia's pathophysiology remains limited. The hypothesis proposed by Oldfield et al. suggests that piston-like...
Despite several hypotheses, our understanding of syringomyelia's pathophysiology remains limited. The hypothesis proposed by Oldfield et al. suggests that piston-like movement of the cerebellar tonsils propels the cerebrospinal fluid (CSF) into the syrinx via the spinal perivascular space. However, a significant question remains unanswered: how does the CSF enter and stay in the syrinx, which has a higher pressure than the subarachnoid space. In the current study, we attempted to verify Oldfield's hypothesis using phase-contrast magnetic resonance imaging (MRI) data from patients with syringomyelia. We analyzed phase-contrast MRI scans of 18 patients with Chiari-I malformation associated with syringomyelia, all of whom underwent foramen magnum decompression, and 21 healthy volunteers. We obtained velocity waveforms for CSF and brain tissue from regions of interest (ROI) set at the various locations. These waveforms were synchronized at the peak timing of downward CSF flow. We compared the preoperative patient data with the control data and also compared the preoperative patient data with the postoperative patient data. The syrinx shrank in 17 (94%) of the patients, and they experienced significant clinical improvement. When comparing pre- and postoperative MRI results, the only significant difference noted was the preoperative elevated velocity of the cerebellar tonsil, which disappeared post-surgery. The CSF velocities in the subarachnoid space were higher in the preoperative patients than in the controls, but they did not significantly differ in the postoperative MRI. The tonsillar velocity in the preoperative MRI was significantly lower than that of the CSF, suggesting that the elevated tonsillar velocity was more of an effect, rather than the cause, of the elevated CSF velocity. Given these findings, a completely new paradigm seems necessary. We, therefore, propose a novel hypothesis: the generative force of syringomyelia may be the direction-selective resistance to CSF flow in the subarachnoid space.
Topics: Syringomyelia; Arnold-Chiari Malformation; Retrospective Studies; Humans; Male; Female; Cerebrospinal Fluid; Foramen Magnum
PubMed: 37637502
DOI: 10.12688/f1000research.72823.2 -
Cureus Jul 2023This case report describes an uncommon presentation of lung adenocarcinoma, which appeared as a skull mass. While not the first reported case in medical literature, it...
This case report describes an uncommon presentation of lung adenocarcinoma, which appeared as a skull mass. While not the first reported case in medical literature, it is still a rare occurrence for lung adenocarcinoma to present in this manner. This report focuses on the clinical presentation and treatment of an elderly male patient who had a progressively enlarging and painful skull mass. The initial imaging revealed an about 5 cm soft tissue mass at the dorsal midline of the parietal-occipital bone. Subsequent imaging identified a lung mass, and a biopsy of the skull bone confirmed that the mass was metastatic adenocarcinoma originating from the lung. For treatment, the patient underwent occipital partial resection of the mass, followed by wire mesh cranioplasty. Chemotherapy and external beam radiotherapy were administered to alleviate symptoms and control the spread of cancer. Lung carcinoma with distant metastasis is generally associated with a poorer prognosis. However, some supporting data suggest that early detection and aggressive management play crucial roles in preventing further metastasis and improving the patient's quality of life and overall survival rate. Skull bone metastasis from lung cancer is indeed a rare phenomenon, and cases like these contribute valuable knowledge to the field. By reporting such cases, healthcare professionals can gain a better understanding of the clinical manifestations, diagnostic challenges, and appropriate management strategies for these uncommon occurrences. This case report underscores the significance of maintaining a high index of suspicion and utilizing a multimodality approach to diagnose rare instances of calvarial metastasis.
PubMed: 37621819
DOI: 10.7759/cureus.42399