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Accessory bone

An accessory bone, also known as an accessory ossicle or supernumerary bone, is a secondary that fails to fuse with the adjacent primary during skeletal development, resulting in an extra, well-corticated bony structure that is a normal anatomical variant. These bones differ from sesamoid bones, which are embedded within tendons to reduce friction and improve , whereas accessory ossicles are typically separate from soft tissues and serve no known functional role. They are usually small, round, or ovoid in shape and appear as distinct entities on imaging, often identified incidentally during radiographic evaluations. Accessory ossicles occur throughout the body but are most prevalent in the foot and ankle, where approximately 40 distinct types have been described, representing the primary site for these variants in human anatomy. Common examples in the foot include the accessory navicular (prevalence 2%-21%), os trigonum (7%-25%), and os peroneum (up to 26%), while less frequent occurrences are noted in the , , , , and . In the upper extremity, such as the , they are rarer, with reported incidences below 1% for specific types like the os supratrochleare dorsale. Prevalence varies by population and imaging modality, with anatomical dissections often revealing higher rates than radiographic studies due to detection limitations. Although accessory bones are typically and discovered incidentally, they can become clinically significant when subjected to , repetitive stress, or degenerative processes, leading to conditions such as fractures, , or impingement syndromes. For instance, an irritated accessory navicular may cause medial foot due to posterior tibialis tendon dysfunction, while an os trigonum can contribute to posterior ankle impingement during activities like or soccer. relies on —radiographs to confirm well-defined margins distinguishing them from fractures, with MRI or for assessing associated or complications. Management is usually conservative, involving rest, , or measures, though surgical excision is considered for persistent symptoms refractory to non-operative treatment.

Fundamentals

Definition and Characteristics

Accessory bones, also known as accessory ossicles, represent secondary ossification centers that fail to fuse with the primary skeletal elements during , resulting in separate, well-formed bony structures. These ossicles are considered normal anatomical variants and are not associated with fractures, dislocations, or pathological processes. Morphologically, accessory bones are typically small, round, or ovoid in shape, with smooth, well-corticated margins and diameters commonly ranging from 2 to 10 mm, though sizes can vary slightly depending on location. They often appear as independent entities adjacent to standard bones, exhibiting mature on . Accessory bones must be distinguished from sesamoid bones, which are small, round structures that form within tendons to minimize friction during movement, such as the patella in the quadriceps tendon. In contrast, accessory ossicles derive from unfused ossification centers rather than tendon-embedded development.

Embryological Development

Accessory bones arise from ectopic or accessory ossification centers that develop separately from the primary ossification sites during the process of endochondral ossification in fetal development. These secondary centers form as variations in the normal ossification pathways, where cartilage models of future bones begin to mineralize and replace with bone tissue. Unlike primary centers, which integrate into the main bone structure, accessory centers fail to fuse completely, resulting in distinct ossicles that persist postnatally. The formation of these ossification centers typically occurs during the early fetal period, with initial bone beginning between the sixth and seventh weeks of . Genetic factors play a key role in this process, as variations in developmental genes can lead to the emergence of supernumerary ossification sites; for instance, studies on specific accessory bones like the navicular demonstrate heritable influences on their presence. Environmental influences, such as mechanical stress from fetal movements, may also modulate by promoting or altering the of these centers during growth. of often becomes evident by , when skeletal maturation completes and separate remain as stable structures. Histologically, accessory bones exhibit features akin to those of primary skeletal elements but on a smaller scale, consisting of a dense outer layer of cortical surrounding an inner core of cancellous bone with trabecular architecture. This composition provides mechanical integrity similar to regular s, with well-defined cortical margins that distinguish them radiographically. The cancellous interior facilitates nutrient exchange through vascular channels, supporting the ossicle's viability without integration into larger bones.

Prevalence and Clinical Implications

Accessory bones, also known as accessory ossicles, exhibit varying prevalence across different anatomical regions and populations, with studies reporting an overall incidence of 2% to 30% in the general population depending on the location examined. In the foot and ankle, where they are most commonly studied, the prevalence ranges from 18% to 48%, with higher rates observed in specific ethnic groups; for instance, the occurs in up to 38% of East Asian individuals compared to 8% in North Americans. These variations are attributed to genetic and developmental factors, though exact mechanisms remain under investigation. Detection of accessory bones primarily occurs through imaging modalities such as plain radiographs, , and magnetic resonance imaging (MRI), often as incidental findings during evaluations for unrelated musculoskeletal complaints. Radiographs identify them in approximately 20-30% of routine foot scans, while advanced like provides superior delineation of their morphology and relationships to adjacent structures, and MRI assesses associated involvement. These ossicles can mimic acute fractures or avulsion injuries on initial , necessitating careful radiographic to avoid misdiagnosis. Clinically, the vast majority of accessory bones remain asymptomatic throughout life, but in symptomatic cases—estimated at less than 10%—they may contribute to localized , impingement, or mechanical dysfunction due to , , or degenerative changes. For example, the accessory navicular has been associated with pes planus (flatfoot deformity) in some patients, potentially exacerbating arch collapse and hindfoot . They may also rarely correlate with conditions like , where incomplete fusion involving ossicles can lead to rigid flatfoot or recurrent sprains, though direct causation is debated. Surgical intervention, typically involving excision of the symptomatic ossicle, is reserved for conservative failures and yields relief in over 80% of cases, with success rates reaching 90-93% for procedures like the Kidner for accessory navicular syndrome. Postoperative outcomes emphasize the importance of addressing concurrent or issues to optimize recovery.

Cranial Accessory Bones

Wormian Bones

Wormian bones, also known as sutural or intra-sutural bones, are small, irregular that form within the sutures and fontanelles of the , most commonly along the . These accessory bones arise from independent centers and are typically asymmetrical and shapeless, often measuring less than 6 mm by 4 mm in size. They are predominantly located in the posterior aspect of the , with the being the most frequent site (up to 27.89% of cases), followed by the lambda region (19.04%), while occurrences in the sagittal or coronal sutures are less common but possible. In the general adult population, the prevalence of Wormian bones varies widely by geographic and ethnic groups, ranging from approximately 10% in populations to 40% in populations and up to 80% in some East Asian groups, with studies reporting overall incidences around 35% in North skulls. They are more frequently observed in males than females in certain cohorts, though no consistent exists across all studies. In pathological conditions, their prevalence increases significantly; for instance, they occur in up to 96% of individuals with severe forms of (types III and IV), compared to only 35% in milder type I. Clinically, Wormian bones are generally benign and considered normal variants that may enhance skull compliance and fracture resistance by distributing mechanical stress during bending loads. However, the presence of excessive numbers—defined as more than 10 to 15 bones, often in a mosaic pattern—can signal underlying syndromes, including , , , or , warranting further diagnostic evaluation such as imaging to differentiate from fractures. In forensic and anthropological contexts, they aid in sex estimation, population identification, and distinguishing sutural variants from traumatic injuries.

Inca Bone

The Inca bone, also known as the interparietal bone (os interparietale or os inca), is a triangular accessory ossicle situated in the interparietal region of the occipital squama, typically bounded by the lambdoid sutures superiorly and the mendosal suture inferiorly, with separation from the main occurring via persistent transverse sutures. This structure arises from the failure of fusion between the primary (cartilaginous) and secondary (membranous) centers—or among multiple intramembranous centers including two lateral plates and a median piece—of the occipital bone, which normally integrate during the second and third months of fetal development. The bone's morphology can vary, occasionally presenting as fragmented, multiple, or paired pieces, and it is distinct as a singular, larger entity compared to smaller sutural ossicles. It may represent an evolutionary remnant from postparietal bones integrated into the mammalian occipital squama. Prevalence of the Inca bone exhibits significant geographic and ethnic variation, ranging from approximately 1% in many modern populations (e.g., 1.3% in ) to up to 30% in certain indigenous groups, such as pre-Hispanic South Americans (27.7%), , and East Asian-derived populations like and Sikkimese. In contrast, incidences are lower (under 5%) in and Central Asian populations. This distribution underscores its value as an anthropological marker for tracing migrations and affinities. Clinically, the Inca bone is typically asymptomatic and discovered incidentally during postmortem examinations, computed tomography (CT) scans, or radiographic imaging of the cranium. It holds relevance in , where its sutural lines may mimic fractures, potentially leading to misdiagnosis, and in , as it could complicate procedures like burr-hole trephination near the occipital region. Forensically, its presence aids in individual identification, particularly when antemortem imaging is available, and contributes to ethnic profiling in medico-legal contexts. In rare cases, it has been associated with cranial deformities such as those seen in syndromes.

Upper Extremity Accessory Bones

Os Acromiale

Os acromiale is an accessory bone formed by the incomplete fusion of one or more centers of the process on the , resulting in a mobile distal segment connected by . The typically develops from four primary centers—pre-acromion (anterior tip), meso-acromion (middle segment), meta-acromion (posterior segment), and basi-acromion (proximal base attached to the scapular )—which fuse progressively between ages 15 and 25. Non-fusion creates instability at the , potentially altering deltoid mechanics and subacromial space dynamics. The condition is classified based on the unfused segment: pre-acromiale (involving the pre-acromion, least common at about 4%), meso-acromiale (involving the meso-acromion, most prevalent at 68–94% of cases), and meta-acromiale (involving the meta-acromion, rare at around 1–2%). Meso-acromiale predominates due to the larger size and later of the meso-acromion , with the unfused ossicle averaging 23 mm in length, 24 mm in width, and 10 mm in thickness. Prevalence of os acromiale ranges from 1% to 15% globally, with an estimated average of 8% based on anatomical studies of over 1,000 scapulae, though rates vary by —higher in and African American populations (9.2–18.2%) compared to Caucasians (1–15%, often around 1.9–7.7%) and Asians (0.7%). It occurs more frequently in males and can be unilateral (about 76% of cases) or bilateral (24%), without strong links to age or body size. Clinically, os acromiale is often asymptomatic but can contribute to subacromial impingement syndrome, rotator cuff pathology (associated in up to 50% of cases), and chronic , particularly in active individuals or athletes, due to abnormal motion of the unfused segment. Symptoms typically include superior exacerbated by overhead activities, tenderness over the , reduced , and weakness, with confirmed by radiographs (e.g., axillary view showing a "double-density" ) or advanced imaging like MRI for soft-tissue assessment. Initial management is conservative, involving 6–12 months of activity modification, to strengthen the and deltoid, NSAIDs for control, and subacromial injections, which succeed in many cases. For persistent symptomatic instability, surgical intervention focuses on fusion via open or arthroscopic (e.g., cannulated screws or tension-band wiring with ), preserving deltoid function; excision is reserved for small, irreparable pre-acromiale fragments, though it risks deltoid weakness.

Wrist and Hand Ossicles

Accessory ossicles in the wrist and hand are supernumerary bones arising from unfused centers or ectopic , commonly identified in the carpal and metacarpal regions. These structures are typically but can mimic fractures on or contribute to clinical symptoms when involved in degenerative changes or . A prominent example is the os centrale, a small, trapezoid-shaped accessory bone located dorsally between the scaphoid, capitate, and bones near the radioscaphoid . It originates as a separate in the around 6 weeks, which normally fuses with the scaphoid by 8 weeks but persists in some individuals. The prevalence of os centrale is approximately 0.6-1.6% in radiographic studies. While often incidental, it may lead to intermittent wrist pain, clicking, or due to its mobility, and post-traumatic osteonecrosis has been reported, potentially exacerbating in the adjacent carpal joints. Detection is straightforward on plain radiographs, particularly lateral views, where it appears as a well-corticated ossicle distinct from fragments by its smooth margins. The os ulnostyloideum represents a variant at the ulnar styloid, manifesting as a separate ossicle near the ulnar styloid process on the distal ulna's ulnar margin, adjacent to the carpometacarpal joints. This accessory bone results from failure of fusion of the styloid ossification center and has a prevalence of about 1.5% based on wrist radiographs. Clinically, it is usually benign but can be symptomatic if irritated, contributing to ulnar-sided wrist pain or instability, and is frequently mistaken for an acute ulnar styloid fracture in trauma settings due to its location—differentiation relies on imaging showing rounded, sclerotic borders rather than irregular fracture lines. It is readily visible on standard posteroanterior and lateral wrist X-rays. On the radial side, the os radiale externum is an accessory ossicle positioned adjacent to the space between the scaphoid tubercle and , at the distal lateral margin of the scaphoid. It arises from an independent site and occurs in roughly 0.8-2.6% of individuals per radiographic surveys. Though typically asymptomatic, symptomatic cases may involve localized pain or degenerative at the first , particularly following repetitive stress or injury. Like other ossicles, it is best detected on lateral or oblique X-rays, where its cortical outline helps distinguish it from avulsion fractures. In addition to these carpal variants, sesamoid bones in the hand, such as those embedded in the flexor pollicis brevis at the of , represent normal anatomical features rather than true accessory ossicles and are present in nearly all individuals without clinical consequence.

Elbow and Forearm Ossicles

Accessory ossicles in the and represent rare supernumerary bones arising from independent centers that fail to fuse with adjacent skeletal elements. These small, typically round or ovoid structures are found near articulations, epicondyles, or insertions in the proximal and region, with an overall prevalence of less than 1% in radiographic studies. They are distinct from sesamoid bones but share similar developmental origins and can occasionally be bilateral. Prominent examples include the os supratrochleare, located superior to the humeral trochlea within the or coronoid fossae, where it resides in the and may articulate loosely with the or coronoid process. Small near the medial (os subepicondylare mediale) or radial head also occur, often embedded in flexor or adjacent to the capitellum. The cubiti, a sesamoid-like ossicle in the , exemplifies posterior variants proximal to the . Clinically, these ossicles are usually asymptomatic and discovered incidentally on imaging, but they can mimic avulsion fractures, , or loose bodies, leading to unnecessary interventions. In symptomatic cases, they may cause pain, restricted extension, or contribute to by irritating periarticular soft tissues, occasionally requiring excision for relief. Their low prevalence underscores the need for careful radiographic evaluation to differentiate them from . Accessory ossicles around the and were first described in 19th-century texts, with systematic classifications emerging in later works by anatomists such as Poirier. These findings contribute to the broader spectrum of upper extremity variants, briefly relating to distal ossicles in the continuum of development.

Lower Extremity Accessory Bones

Knee Ossicles

Knee ossicles are small accessory bones located around the joint, primarily consisting of the and cyamella, which develop as sesamoid-like structures within tendons. These ossicles are considered normal anatomical variants but can occasionally contribute to clinical issues in the posterolateral knee region. Unlike primary knee bones, they arise from secondary centers and are more prevalent in certain populations, with overall accessory ossicle rates in the lower extremity varying widely based on and methods. The is a small, typically embedded in the lateral head of the tendon, positioned posterior to the lateral femoral condyle. It articulates with the posterior aspect of the lateral condyle and is enveloped by the gastrocnemius tendon, aiding in force transmission during flexion. Prevalence ranges from 10% to 30% in the general population, with higher rates (up to 87%) observed in Asian cohorts and lower incidences (around 10-20%) in groups, where it may be absent entirely in some individuals. The fabella is often bilateral (in about 80% of cases) and its presence increases with age due to progressive . Clinically, it is usually asymptomatic but can lead to posterior , known as fabella syndrome, through mechanisms such as impingement, chondromalacia, or association with ; in rare instances, it may contribute to formation or require excision for persistent symptoms. On imaging, the fabella is best visualized as a rounded ossicle on lateral knee radiographs, with confirmation via or MRI to differentiate it from fractures or loose bodies. The cyamella, a rarer counterpart, is a sesamoid ossicle located within the popliteus muscle tendon at the posterolateral aspect of the proximal tibiofibular region or distal femur. It develops near the myotendinous junction of the popliteus, which stabilizes the knee during rotation, and is intra-tendinous like the fabella, potentially enhancing tendon efficiency. Its prevalence is low, estimated at 0.57% to 1.8% in ossified form, though it may be underreported due to misidentification as a fabella on imaging; limited data suggest slightly higher occurrence in Asian populations, mirroring trends in other knee sesamoids. Symptomatic cyamellae are exceptional but can cause localized posterior knee pain, tendon irritation, or cyst-like formations, often managed conservatively unless refractory. Radiologically, it appears as a small, oval density on lateral knee views, with MRI providing superior soft-tissue detail to assess tendon involvement and rule out pathology.

Ankle Ossicles

Ankle ossicles are accessory bones located around the talocrural joint, arising from unfused secondary centers or anomalous development, and they may contribute to localized pain or impingement syndromes when symptomatic. These structures are typically identified incidentally on , but their clinical relevance increases in cases of or repetitive stress, particularly among athletes. The os subtibiale is a rare accessory ossicle situated at the posterior aspect of the medial , inferior to the tip of the , measuring approximately 4-15 mm in size. It develops from a secondary that fails to fuse with the medial and has an estimated prevalence of 0.7-1.2% in the general population. Clinically, it can cause medial ankle pain, swelling, and redness, often following an inversion injury, and may mimic an on radiographs. Initial management is conservative with or to restore function, though persistent symptoms after 6 months may necessitate surgical excision. The os trigonum, a common variant of the lateral posterior process of the talus, forms from a separate that remains unfused, appearing as a pyramidal or triangular bone posterior to the talus. Its prevalence varies widely, ranging from 1% to 25% across imaging modalities and populations, with a pooled estimate of 9.0% in meta-analyses of over 36,000 feet. It is strongly associated with posterior ankle impingement syndrome (), where repetitive plantarflexion compresses the ossicle against the posterior or , leading to deep ankle pain, particularly in athletes such as dancers or soccer players; individuals with PAIS are approximately 16 times more likely to have an os trigonum. Symptomatic cases often involve subchondral , flexor hallucis longus , or , and arthroscopic excision is a standard treatment for refractory pain. The os calcaneus accessorius is an uncommon accessory bone located adjacent to the trochlear eminence or anterior of the , typically measuring about 5 mm and resulting from an unfused apophysis. It is generally asymptomatic but can be differentiated from avulsion fractures or os subfibulare on computed tomography, where it appears as a distinct ossicle without acute margins. While prevalence data are limited, related variants like the os calcaneus secundarius occur in 0.6-7% of cases and may contribute to subtle ankle instability if involved in trauma.

Foot Ossicles

Foot ossicles represent a diverse group of bones primarily located in the midfoot and forefoot regions, arising from unfused secondary centers during skeletal development. These structures are often incidental findings on but can contribute to clinical symptoms such as , tendon irritation, or biomechanical alterations when symptomatic. Among the most prevalent are the accessory navicular and os peroneum, with rarer variants including os vesalianum, os supratalare, os calcaneus secundarius, and intermetatarsal ossicles. The is situated on the medial aspect of the navicular tuberosity, often embedded within or adjacent to the tibialis posterior insertion. It is classified into three types based on and attachment: Type I consists of a small, round ossicle (2-3 mm) embedded in the substance; Type II features a larger, triangular connected to the navicular by a ; and Type III represents complete fusion, forming a prominent cornuate navicular tuberosity. Prevalence varies by population but generally ranges from 10% to 20%, with higher rates (up to 38%) in East Asian cohorts and lower (around 8%) in North Americans. Symptomatic cases, particularly Type II, can lead to accessory navicular syndrome, characterized by medial foot , swelling, and pes planus (flatfoot) due to altered tibialis posterior function and hindfoot valgus. The os peroneum is a sesamoid ossicle embedded within the , typically near its passage through the groove. It occurs in 5% to 10% of individuals, though estimates range up to 30% in some radiographic series. This bone enhances tendon leverage but can fracture under tensile stress from inversion injuries or repetitive loading, often associating with peroneus longus or degeneration, resulting in lateral foot and painful os peroneum syndrome. Less common foot ossicles include the os vesalianum, located at the base of the fifth metatarsal adjacent to the peroneus brevis insertion, with a rarity that precludes precise prevalence but is noted as infrequent. It typically remains but may cause lateral foot pain mimicking a proximal fifth metatarsal , potentially linking to peroneal tendonitis through irritation of the peroneus brevis. The os supratalare, positioned superior to the talar head or neck between the ankle and navicular joints, has a low prevalence of approximately 0.5%. It is generally and rarely implicated in . The os calcaneus secundarius appears anterior to the in the tarsi region, with prevalence estimates from 0.6% to 7%. This triangular ossicle can disrupt mechanics if enlarged or malpositioned, leading to chronic ankle or impingement-like symptoms from adjacent hindfoot structures. Intermetatarsal ossicles, such as the os intermetatarseum between the first and second metatarsal bases, are exceptionally rare (less than 0.03%) and may provoke dorsal forefoot through mechanical irritation or .

Axial and Other Accessory Bones

Vertebral Column Ossicles

Vertebral column ossicles, also known as accessory bones of the spine, represent unfused secondary ossification centers or anomalous bony formations along the axial skeleton, distinct from typical vertebral anatomy. These ossicles can occur in the cervical, thoracic, lumbar, and sacral regions, often identified incidentally on imaging studies such as CT or MRI. While most are asymptomatic, some may contribute to clinical issues through mechanical irritation, vascular compression, or altered biomechanics. Their formation is attributed to developmental variations in ossification, with prevalence varying by location and population demographics. The ponticulus posticus, a bony bridge forming the on the posterior arch of the C1 atlas vertebra, is one of the most commonly recognized ossicles. It arises as a variant of the transverse process and posterior arch fusion, potentially enclosing the within the . Prevalence estimates range from 10% to 21% in general populations, with higher rates observed in certain ethnic groups and a slight bilateral in about 40-50% of cases. This structure can lead to compression during head rotation, potentially causing vertebrobasilar insufficiency symptoms such as or syncope. Additionally, it has been associated with cervicogenic headaches, where the prevalence of headaches is significantly higher (up to 44%) among individuals with ponticulus posticus compared to those without, possibly due to irritation of surrounding neural structures or altered . Although direct links to are less established, some case reports suggest potential contributions to craniovertebral junction anomalies in symptomatic patients. In the region, intertransverse , often exemplified by Oppenheimer ossicles, appear as small, rounded bony excrescences near the s or transverse processes, resulting from unfused centers. These are typically located on the superior articular processes of L3-L5 vertebrae and involve incomplete fusion of transverse process elements. Prevalence is approximately 4% (ranging 1-7%), with a higher incidence in males and potential bilaterality. Such ossicles may mimic fractures on imaging but are developmental variants; clinically, they can contribute to through ligamentous tension or irritation, occasionally leading to if associated with flavum ligament buckling. Thoracic accessory ribs, referred to as lumbar ribs when originating from L1, represent elongated transverse processes forming rib-like structures at the thoracolumbar junction. These occur in about 1% of individuals, often bilaterally (65%), and may alter paraspinal muscle attachments or compress neurovascular elements, though symptomatic cases are rare. Sacral variants, such as os sacrum accessorium or accessory sacroiliac ossicles, involve additional bony elements near the sacroiliac joint, often manifesting as accessory articulations between the ilium and sacrum. These arise from separate ossification centers that fail to incorporate into the main sacral body, with prevalence around 13-26% for accessory sacroiliac joints, more common at the S2 level and bilateral in over 50% of affected cases. Anatomically, they extend the joint surface, potentially influencing load distribution across the pelvis. Clinically, these ossicles may correlate with sacroiliac joint dysfunction or lower back pain, particularly in populations with higher mechanical stress, though most remain asymptomatic and are detected via pelvic CT. Overall, vertebral ossicles underscore the spectrum of spinal developmental diversity, with imaging playing a key role in differentiation from pathology.

Episternal Ossicle

The episternal ossicle, also known as the suprasternal ossicle, is a small accessory bone located at the superior margin of the manubrium sterni, typically positioned posterior or superior to this structure near the manubriosternal joint. It is often pyramidal or ovoid in shape, measures 2–15 mm in diameter, and may occur unilaterally or bilaterally, appearing as a well-corticated entity on imaging such as multidetector computed tomography (MDCT). This ossicle represents a normal anatomical variant within the , arising from an independent distinct from the primary sternal . The prevalence of episternal ossicles is low, estimated at approximately 2.1% (95% CI: 1.1–3.0%) across global populations based on a of 16 studies involving 7,997 subjects, with regional variations including a higher rate of 3.8% in Asian . Specific studies report frequencies ranging from 1.5% in a of 800 adults (1.4% in men and 1.7% in women) to 4.1–6.9% in other populations, showing a slight female predominance. Embryologically, it develops from a supernumerary in the sternal anlage that fails to fuse with the manubrium, unlike the typical sternal where multiple centers merge before birth. Clinically, episternal ossicles are asymptomatic and discovered incidentally on chest radiographs or scans, but they can be misinterpreted as pathological entities such as fragments, sequestra, calcified lymph nodes, vascular calcifications, or foreign bodies, necessitating careful radiological evaluation for differentiation. No associated morbidity or functional impairment has been reported, emphasizing their benign nature as a developmental variant. Historically, episternal ossicles were first systematically described by Cobb in 1937, though earlier anatomical observations date back to the , including references by Béclard in 1820 and Breschet in 1838. Subsequent studies in the late , such as those by Stark et al. in 1987, highlighted their radiographic appearance and prevalence, contributing to greater clinical recognition with advanced imaging modalities.

Rare Systemic Accessory Bones

Rare systemic accessory bones are uncommon skeletal variants that arise from aberrant ossification centers and manifest across multiple body regions or in atypical locations, often as components of congenital syndromes rather than isolated anomalies. These ossicles differ from common regional variants by their association with genetic disorders, potentially leading to multisystem involvement and clinical complications such as instability or developmental delays. Their is generally low, estimated at less than 0.5% in the general , though higher in syndromic contexts. One prominent example is os odontoideum, a variant of the dens characterized by a separate, corticated ossicle detached from the vertebral body, resulting from incomplete during embryogenesis. This condition predisposes individuals to atlantoaxial and cervical cord compression, particularly in pediatric cases, and is frequently linked to syndromes such as ( type IVA) and , where ligamentous laxity exacerbates risks. Diagnosis typically involves imaging to confirm the ossicle's morphology and assess stability, with whole-body recommended in syndromic patients to evaluate for concurrent anomalies. Surgical may be indicated for symptomatic . In cleidocranial dysplasia (), a rare autosomal dominant disorder caused by gene mutations, multiple accessory ossicles occur due to defective , affecting the cranium, pelvis, and extremities. Skull sutures often contain numerous —small, irregular accessory ossicles embedded within the lambdoid or sagittal sutures—contributing to delayed fontanelle closure and cranial deformities. Additional systemic manifestations include accessory bones in the hands and feet, alongside clavicular , highlighting the disorder's broad skeletal impact. has a prevalence of approximately 1 in 1,000,000, with diagnosis confirmed via and radiographic surveys, including whole-body imaging to map ossific defects. Management focuses on orthopedic interventions for associated instability. Accessory metacarpals in syndromes represent another systemic rarity, where supernumerary carpal or metacarpal accompany extra digits, often in genetic conditions like Ellis-van Creveld or Bardet-Biedl . These arise from duplicated centers and can lead to hand deformities, reduced function, and syndromic features such as retinal or renal anomalies. Prevalence varies by but remains under 0.5% overall, with central or postaxial types showing proximal accessory metacarpals in up to 20% of cases. Clinical evaluation includes hand radiographs and genetic analysis, with whole-body useful for multisystem assessment; surgical reconstruction addresses functional impairments.

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