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European Archives of... Jun 2024The term petrous bone cholesteatoma (PBC) refers to lesions extending deep to the bony labyrinth via superior, inferior, and posterior cell tracts. PBC is a rare...
BACKGROUND
The term petrous bone cholesteatoma (PBC) refers to lesions extending deep to the bony labyrinth via superior, inferior, and posterior cell tracts. PBC is a rare incidence accounting for only 4-9% of petrous bone lesions. Lesions of petrous bone represent a real surgical challenge due to its complex relationship with critical neurovascular structures.
OBJECTIVE
To demonstrate our 40-plus years' experience in the management of PBC, depict the clinical features of PBC according to Sanna's Classification, evaluate the postoperative follow-up of surgically treated PBC patients, and determine the recurrence rate.
STUDY DESIGN
Retrospective medical record review.
MATERIAL AND METHODS
Medical records of 298 PBC cases operated from the year 1983 to 2024 were thoroughly evaluated.
RESULTS
A total of 298 PBC cases were surgically treated at our center. The average age at presentation in this series was 47 years. Males are more affected than females with a male-to-female ratio of 2.2:1. The most common presenting symptoms were hearing loss (84%), tinnitus (48%), and facial nerve paralysis (45%). Mixed hearing loss (41%) was the commonest audiometric pattern of hearing loss followed by conductive hearing loss (26%) and profound sensorineural hearing loss (4%) and a total of 86 (29%) had anacusis at presentation. On preoperative facial nerve function examination, 133(45%) of patients had various degrees of paresis and complete paralysis whereas 55% had normal HB-I function. The commonest degree of paresis noted was HB-III (18%) followed by HB-VI (5%). A total of 150 (50%) patients had previous otologic surgery and two-thirds of these cases had two or more prior otologic surgeries. According to Sanna's PBC Classification system, we identified that the supralabyrinthine class (44%) is the commonest of all classes followed by massive (33%), infralabyrinthine-apical (9%), infralabyrinthine(8%), and apical (5%) classes in that order. However, only ten patients had congenital type of PBC. Extension to clivus, sphenoid, nasopharynx, intradural space, and occipital condyle was found in 8, 2, 1, and 2 cases respectively. The most commonly used surgical approaches at our center were TO, MTCA with rerouting of the facial nerve, and TLAB with external auditory canal (EAC) closure. Postoperative complications were minimal and the duration of follow-up ranged from one to 458 months with a mean duration of 65 months. Residual lesions were evident in 11 cases (3.7%), with the surgical cavity, middle and posterior fossa dura, and jugular bulb being the commonest sites.
CONCLUSION
Petrous bone cholesteatoma represents diagnostically and surgically challenging lesions of temporal bone which are usually frustrating to the treating surgeon. A high index of clinical suspicion, thorough clinical evaluation examination, and preoperative radiologic evaluation make the diagnosis easier. Preoperative anatomic classification of the lesion enables the physician to choose the appropriate surgical approach. Sanna's classification is widely used to classify PBC in relation to the labyrinthine block. Radical disease removal should always come before hearing preservation. Cavity obliteration is the solution to the problems related to a large cavity. Finally, advancements in lateral skull base approaches create adequate surgical access for the complete removal of the lesion with excellent control of critical neurovascular structures.
PubMed: 38829556
DOI: 10.1007/s00405-024-08752-4 -
Fetal and Pediatric Pathology May 2024We describe five abnormal crania which may provide more diagnostic data for assessment of abnormal crania in newborns.
INTRODUCTION
We describe five abnormal crania which may provide more diagnostic data for assessment of abnormal crania in newborns.
METHODS
Five malformed perinatal crania from the Saxtorphian Collection are described using published prenatal abnormal cranial development criteria. These malformations were compared to normal cranial development arising from the migration of neural crest cells. Visual and photographic investigations were performed.
RESULTS
The malformed crania were occipital encephalocele, holoprosencephaly, anencephaly, and two without a recognizable diagnosis. The anthropological crania were malformed in the same regions as formerly observed in fetal pathology. These regions were comparable to fields formed during normal cell migration from the neural crest. This has seemingly not previously been demonstrated. One undiagnosed cranium may represent a Treacher Collins syndrome (Case 3). The other undiagnosed cranium (Case 4) could be from a scaphocephalic specimen.
DISCUSSION
Sharp borderlines between malformed and non-malformed regions in cranial syndromes may enable improvement in diagnostics.
PubMed: 38817107
DOI: 10.1080/15513815.2024.2338434 -
Medicina (Kaunas, Lithuania) May 2024Klippel-Feil syndrome (KFS) is characterized by the congenital fusion of the cervical vertebrae and is sometimes accompanied by anomalies in the craniocervical junction....
Klippel-Feil syndrome (KFS) is characterized by the congenital fusion of the cervical vertebrae and is sometimes accompanied by anomalies in the craniocervical junction. In basilar invagination (BI), which is a dislocation of the dens in an upper direction, compression of the brainstem and cervical cord results in neurological defects and surgery is required. A 16-year-old boy diagnosed with KFS and severe BI presented with spastic tetraplegia, opisthotonus and dyspnea. CT scans showed basilar impression, occipitalization of C1 and fusion of C2/C3. MRI showed ventral compression of the medullocervical junction. Posterior occipitocervical reduction and fusion along with decompression were performed. Paralysis gradually improved postoperatively over 3 weeks. However, severe spasticity and opisthotonus persisted and intrathecal baclofen (ITB) therapy was initiated. Following this, opisthotonus disappeared and spasticity of the extremities improved. Rehabilitation therapy continued by controlling the dose of ITB. Five years after the surgery, self-propelled wheelchair driving was achieved and activities of daily life improved. The treatment strategy for patients with BI and congenital anomalies remains controversial. Posterior reduction and internal fixation using instrumentation were effective techniques in this case. Spasticity control achieved through a combination of surgery and ITB treatment enabled the amelioration of therapeutic efficacy of rehabilitation and the improvement of ADL.
Topics: Humans; Baclofen; Male; Klippel-Feil Syndrome; Adolescent; Cervical Vertebrae; Spinal Fusion; Injections, Spinal; Muscle Relaxants, Central; Occipital Bone; Treatment Outcome; Decompression, Surgical
PubMed: 38792938
DOI: 10.3390/medicina60050755 -
Journal of Clinical Medicine May 2024Bone-anchored maxillary protraction (BAMP) aims to correct midfacial deficiencies, with proven positive skeletal changes without potential unwanted side effects....
Three-Dimensional Cone Beam Computed Tomography (CBCT)-Derived Soft Tissue Changes in Patients with Unilateral Cleft Lip, Alveolus, and Palate with Midfacial Deficiency after 1.5 Years of Bone-Anchored Maxillary Protraction.
Bone-anchored maxillary protraction (BAMP) aims to correct midfacial deficiencies, with proven positive skeletal changes without potential unwanted side effects. However, the influence of BAMP treatment on facial soft tissues, particularly in subjects with complete unilateral cleft lip, alveolus, and palate (CUCLAP), remains unclear. This single-center longitudinal cohort study examined the effects of 1.5 years of BAMP treatment on facial soft tissues in growing subjects with complete unilateral cleft lip, alveolus, and palate. The sample consisted of 25 patients, age range 9.7 to 12.6 years. Three-dimensional surface models derived from CBCT scans were superimposed on stable structures of the anterior cranial base and on the occipital area posterior of the foramen magnum to assess three-dimensional changes due to growth and BAMP therapy. The results revealed a moderate positive correlation (Pearson's correlation coefficient from 0.203 to 0.560) between changes in hard tissue and soft tissue; some correlations were found to be weak (<0.300). Linear changes in soft tissue following BAMP were in the same direction as skeletal changes, showing downward, forward, and outward displacement. The only exception was in the vertical dimension. The lower facial third showed a slight but significant reduction, mainly in lip length (-1.2 mm), whereas the middle facial third showed a small increase (1.1 mm). It was concluded that during BAMP, soft tissue changes occur in the same direction as skeletal changes, although with a larger variability and less pronounced effects.
PubMed: 38792430
DOI: 10.3390/jcm13102890 -
Acta Oto-laryngologica Mar 2024Inflammatory conditions such as chronic otitis media (COM) can cause irreversible impairments in the microarchitecture and functions of the incus, which subsequently...
BACKGROUND
Inflammatory conditions such as chronic otitis media (COM) can cause irreversible impairments in the microarchitecture and functions of the incus, which subsequently leads to conductive hearing loss.
OBJECTIVES
To investigate bone mineral density (BMD) of the incus body (IB) and long process (ILP) on preoperative temporal CT in COM patients with and without incudo-stapedial joint discontinuity (ISJD), and also to determine the association between BMD values and the postoperative air-bone gap (ABG) in the ISJD group.
MATERIAL AND METHODS
The mean IB density (IBD)/occipital bone density (OBD) and ILP density (ILPD)/OBD values were compared between the patients with and without ISJD. The correlation between ABG gain and preoperative incus density values was assessed in the ISJD group.
RESULTS
The mean IBD/OBD and ILPD/OBD values were significantly higher in patients with intact ISJ. There was a moderate positive correlation between postoperative ABG gain and ILPD/OBD values in the ISJD group.
CONCLUSION AND SIGNIFICANCE
The decrease in BMD of the incus may involve ILP as well as IB in patients with ISJD caused by ILP lysis in COM. A higher preoperative ILPD/OBD was correlated with a higher postoperative ABG gain in COM patients with ISJD.
Topics: Humans; Otitis Media; Bone Density; Male; Tympanoplasty; Female; Chronic Disease; Adult; Incus; Middle Aged; Hearing Loss, Conductive; Young Adult; Retrospective Studies; Tomography, X-Ray Computed; Adolescent; Aged
PubMed: 38787327
DOI: 10.1080/00016489.2024.2356611 -
Journal of Anatomy May 2024The craniocervical junction (CCJ) forms the bridge between the skull and the spine, a highly mobile group of joints that allows the mobility of the head in every...
The craniocervical junction (CCJ) forms the bridge between the skull and the spine, a highly mobile group of joints that allows the mobility of the head in every direction. The CCJ plays a major role in protecting the inferior brainstem (bulb) and spinal cord, therefore also requiring some stability. Children are subjected to multiple constitutive or acquired diseases involving the CCJ: primary bone diseases such as in FGFR-related craniosynostoses or acquired conditions such as congenital torticollis, cervical spine luxation, and neurological disorders. To design efficient treatment plans, it is crucial to understand the relationship between abnormalities of the craniofacial region and abnormalities of the CCJ. This can be approached by the study of control and abnormal growth patterns. Here we report a model of normal skull base growth by compiling a collection of geometric models in control children. Focused analyses highlighted specific developmental patterns for each CCJ bone, emphasizing rapid growth during infancy, followed by varying rates of growth and maturation during childhood and adolescence until reaching stability by 18 years of age. The focus was on the closure patterns of synchondroses and sutures in the occipital bone, revealing distinct closure trajectories for the anterior intra-occipital synchondroses and the occipitomastoid suture. The findings, although based on a limited dataset, showcased specific age-related changes in width and closure percentages, providing valuable insights into growth dynamics within the first 2 years of life. Integration analyses revealed intricate relationships between skull and neck structures, emphasizing coordinated growth at different stages. Specific bone covariation patterns, as found between the first and second cervical vertebrae (C1 and C2), indicated synchronized morphological changes. Our results provide initial data for designing inclusive CCJ geometric models to predict normal and abnormal growth dynamics.
PubMed: 38783688
DOI: 10.1111/joa.14067 -
Acta Neurologica Belgica May 2024Our study aims to prospectively compare an autologous duraplasty in situ technique (IS group) with the synthetic dural graft duraplasty (SDG group) to clarify the...
OBJECTIVE
Our study aims to prospectively compare an autologous duraplasty in situ technique (IS group) with the synthetic dural graft duraplasty (SDG group) to clarify the effectiveness and superiority of the former in the treatment of patients with Chiari malformation type 1 (CM-I).
METHOD
29 patients with CM-I were randomly assigned to either IS or SDG group. In both groups, a dissection from the occipital bone was performed. All procedures were performed by the same surgeon. The two duraplasty methods were compared in terms of surgical factors and complications. Data analysis was done for the baseline material, the neurological outcome and MRI-documented syrinx size at the 6 month follow-up.
RESULT
29 patients were enrolled in this study, 14 in the IS group and 15 in the SDG group. The results showed no significant difference in operation time (P = 0.916), amount of bleeding (P = 0.120), operation complications, hospitalization time (P = 0.854) and prognosis between the two groups. The hospitalization cost of IS group was 15,125 yuan less than that of SDG group (P < 0.05).
CONCLUSION
The autogenous duraplasty in situ technique is a novel, simple, effective and economical surgical management for patients with CM-I.
PubMed: 38769273
DOI: 10.1007/s13760-024-02579-w -
Anatomy & Cell Biology May 2024In this report, atlantooccipital assimilation (AS), anterior arch defect (AAD), and posterior arch defect (PAD) of the atlas, and several variations around the...
In this report, atlantooccipital assimilation (AS), anterior arch defect (AAD), and posterior arch defect (PAD) of the atlas, and several variations around the craniocervical junction were identified on computed tomography (CT) of a patient of unknown sex and age. Coronal and sagittal CT scans showed AS and bilateral fusion of the atlas and the base of occipital bone. Axial CT scan at the atlas revealed PAD type B on the left side and midline AAD. Morphometric measurements indicated a potential ventral spinal cord compression. In addition, mid-sagittal CT revealed the presence of fossa navicularis magna and incomplete formation of the transverse foramen on the right side. This study reports an extremely rare AS associated with AAD, PAD, and other variations of the clivus and the atlas. To our knowledge, no similar case has been reported in the literature.
PubMed: 38735652
DOI: 10.5115/acb.23.281 -
The Journal of Craniofacial Surgery Jun 2024The occipital emissary foramen (OEF) located on the occipital bone transmits the occipital emissary vein, which connects the occipital vein to the confluence of cranial...
The occipital emissary foramen (OEF) located on the occipital bone transmits the occipital emissary vein, which connects the occipital vein to the confluence of cranial venous sinuses. The OEF varies in incidence, number, size, and location. Knowledge of this foramen is essential for carrying out suboccipital and transcondylar surgeries without clinical implications. Hence, the study was planned. The aim of the present study is to elaborate on incidence, location, and morphometry consisting of the number and size of this foramen in light of clinical bearing in the context of the Indian population. The study was carried out in the Department of Anatomy using 80 skulls of unknown age and sex. The occipital bone of the skull was observed for the incidence, number, size, and location of the occipital emissary vein and associated clinical implications were elaborated. The incidence of occipital foramen was 36.25% and detected in 29 skulls. All these occipital foramina were patent. The mean diameter of this foramen was 0.6 mm. The most common location of these foramina was the left side of the foramen magnum, followed by the left side of the external occipital crest. The information about the incidence, number, size, and location of OEF is important to prevent catastrophic bleeding during surgery in the region of the occipital bone. The awareness of differential morphometry and morphology of occipital foramina is of great importance for neurosurgeons during suboccipital craniotomy and skull base surgeries, including far lateral and transcondylar approaches to access posterior cranial fossa for management of pathologies in the cranial cavity.
Topics: Humans; Occipital Bone; Cadaver; Cerebral Veins; India; Prevalence; Male; Cranial Sinuses; Foramen Magnum; Female; Anatomic Variation
PubMed: 38722329
DOI: 10.1097/SCS.0000000000010264 -
Journal of Morphology May 2024The skull anatomy of amphisbaenians directly influences their capacity to burrow and is crucial for the study of their systematics, which ultimately contributes to our... (Comparative Study)
Comparative Study
The skull anatomy of amphisbaenians directly influences their capacity to burrow and is crucial for the study of their systematics, which ultimately contributes to our comprehension of their evolution and ecology. In this study, we employed three-dimensional X-ray computed tomography to provide a detailed description and comprehensive comparison of the skull anatomy of two amphisbaenian species with similar external morphology, Amphisbaena arda and Amphisbaena vermicularis. Our findings revealed some differences between the species, especially in the sagittal crest of the parietal bone, the ascendant process, and the transverse occipital crest of the occipital complex. We also found intraspecific variation within A. vermicularis, with some specimens displaying morphology that differed from their conspecifics but not from A. arda. The observed intraspecific variation within A. vermicularis cannot be attributed to soil features because all specimens came from the same locality. Specimen size and soil type may play a role in the observed differences between A. arda and A. vermicularis, as the single A. arda specimen is the largest of our sample and soil type and texture differ between the collection sites of the two species.
Topics: Animals; Skull; Lizards; Tomography, X-Ray Computed; Species Specificity; Osteology
PubMed: 38693678
DOI: 10.1002/jmor.21702