Case Report

Multiple Wormian Bones in the Lambdoid Suture: A Report of

Rare Occurrence
José Aderval Aragão 1, *, Roberto Ferreira de Oliveira 2, Smilly Oliveira de Sousa Silva 3, Lucas Inacio Riffel 4, Iapunira
Catarina Sant’Anna Aragão 5, Felipe Matheus Sant’Anna Aragão 6, Giulianna da Rocha Borges 7, Francisco Prado
Reis 8, Deise Maria Furtado de Mendonça 9

1 Titular Professor of Clinical Anatomy, Department of Morphology, Federal University of Sergipe (UFS),
Aracaju, SE, Brazil.
2 Professor of Anatomy, Higher Education Unit of Feira de Santana (UNEF), Feira de Santana, BA, Brazil.
3 Physiotherapy student, Higher Education Unit of Feira de Santana (UNEF), Feira de Santana, BA, Brazil.
4 Physiotherapy Student, Centro Universitário Nobre (UNIFAN), Feira de Santana, BA, Brazil.
5 Medical Clinic Resident of Municipal Hospital Munir Rafful (HMMR), Volta Redonda, RJ, Brazil.
6 Medical Clinic Resident, Faculty of Medicine of São José do Rio Preto (FAMERP), São José do Rio Preto, SP,
Brazil.
7 Adjunct Professor of Human Anatomy, Department of Education and Health, Federal University of Sergipe
(UFS), Sergipe, SE, Brazil.
Citation: Aragão JA, Oliveira RF,
Silva SOS, Riffel LI, Aragão ICS, Ara-
8 Titular Professor, Medical School of Tiradentes University (UNIT), Aracaju, SE, Brazil.
gão FMS, Borges GR, Reis FP, Men-
9 Associate Professor of Human Anatomy, Department of Morphology, Federal University of Sergipe (UFS),
donça DMF. Multiple Wormian São Cristóvão – SE, Brazil.
Bones in the Lambdoid Suture: A Re-
port of Rare Occurrence. Brazilian * Correspondence: [email protected].
Journal of Case Reports. 2024 Oct-
Dec;04(4):141-149.
Abstract: Wormian bones are irregular bony structures developed from independent ossification
centers located at the junction of the sutures between the cranial bones. Although they are com-
Received: 5 May 2024 monly found in healthy individuals, their presence can be associated with pathological conditions.
Accepted: 6 June 2024 Their number above 10, large size, or mosaic pattern are clinically considered as indicators of several
Published: 6 June 2024 pathological conditions, such as abnormalities in the central nervous system, musculoskeletal sys-
tem, or metabolic changes throughout the body. Our study aims to report a rare occurrence of 40
Wormian bones found in the lambdoid suture of an adult human skull and provide a literature
review. Understanding the presence of Wormian bones is crucial for professionals such as neuro-
anatomists, neurosurgeons, radiologists, anthropologists, and morphologists, as they may be mis-
Copyright: This work is licensed un- taken for fractures in cases of traumatic brain injury.
der a Creative Commons Attribution
4.0 International License (CC BY 4.0). Keywords: Anatomical variation; Sutural bone; Skull; Radiology; Basilar invagination.

1. Introduction
Wormian bones, also known as sutural bones, are small accessory bones found in the
complex structure of the human skull. They vary in shape, number, position, and size and
are often symmetrical on both sides, but can be irregular and independent. These bones
were first described by Olaus Worm, a Danish doctor in the 17th century [1-3]. They are
predominantly located in the cranial sutures, although they can occasionally be found in
the fontanelles [4-6]. It is estimated that 8% to 15% of the global population has at least
one of these bones [6-8], with a higher prevalence of location in the lambdoid suture [4, 9-
11]. A special aspect is the identification of a large sutural bone in the lambda region,
known as the "Inca bone". This name originated from the frequent observation of such

Brazilian Journal of Case Reports 2024, 4, 4, 141-149. https://doi.org/10.52600/2763-583X.bjcr.2024.4.4.141-149 www.bjcasereports.com.br

Brazilian Journal of Case Reports 2024, 4, 4, 141-149 142 of 149

structures in Peruvian mummies, suggesting an anthropological and historical relevance

[7, 12, 13].
The presence of Wormian bones may be influenced by both genetic and environmen-
tal factors [7, 14]. There is evidence suggesting that these bones could be anatomical vari-
ations or related to pathological processes, as they are commonly observed in patients
with various diseases. An important point to note is that when identified as a simple an-
atomical variation, Wormian bones are typically smaller in size and fewer in number. In
pathological cases, it is common to identify more than 10 bones [15, 16]. For instance, an
association between the presence of these bones and genetic diseases like Menkes disease,
Primrose syndrome, Hajdu-Cheney syndrome, Oto-palato-digital syndrome type II,
among others, has already been demonstrated [17-20]. The most well-established associa-
tion is with osteogenesis imperfecta, in which the presence of Wormian bones is consid-
ered by some authors as a pathognomonic sign [21, 22]. Regarding environmental factors,
a non-fusion of the cranial sutures has been suggested, as well as mechanical stresses,
mainly in the occipital region [6, 22, 23].
Wormian bones are identified through imaging tests such as X-rays, computed to-
mography, and magnetic resonance imaging. Their presence can be noted even during the
intrauterine phase through ultrasound during routine prenatal care [24]. Three-dimen-
sional computed tomography (3D-CT) has been shown to be important for understanding
the behavior of these bones for the biomechanics of the skull [6,25]. Studies with multide-
tector computed tomography-computed tomography angiography (MDCT-CTA) have
also been reported [26].
Understanding the various causes of Wormian bones is crucial for healthcare profes-
sionals, such as clinicians, neurologists, orthopedists, and radiologists. By conducting
thorough clinical and imaging assessments, it is possible to identify genetic disorders,
congenital anomalies, structural abnormalities, or trauma lesions that may be associated
with these bones. This knowledge is particularly important for neurosurgeons as it helps
them plan surgical procedures involving the skull. Interest in Wormian bones also extends
to the fields of anthropology and forensic medicine, in view of their relevance in deter-
mining identities and understanding ancient cultural practices [26-28]. Given the im-
portance of sutural bones in both clinical and anthropological contexts, the present study
aims to report the multiplicity and distribution of sutural bones in the lambdoid suture in
a dry skull of an adult male and provide a literature review.

2. Case Report
During the cataloging and maintenance of specimens at the osteology museum of the
Feira de Santana Higher Education Unit (UNEF), a 19-year-old male skull was identified.
The skull stood out for its uniqueness due to the presentation of a notable amount of
Wormian bones. The Wormian bones were located predominantly along the lambdoid
suture but also in other regions of the skull. Meticulous observation was carried out to
accurately determine the presence and distribution of these sutural bones. Thus, 47
Wormian bones were found, of which 40 were in the lambdoid suture (Figure 1).
Other sutures, such as the parietomastoid (Figure 2a), occipitomastoid (Figure 2b),
sphenosquamous (Figure 2c), and parietosquamous (Figure 2d) contained sutural bones
(Figure 2). Detailed analysis of the distribution of these bones showed notable variability,
particularly, the location of these bones regarding the side of the skull. In the lambdoid
suture, 19 bones were found on the right side and 21 on the left. As for the other sutures,
one sutural bone was found on the right side of the following sutures: occipitomastoid,
parietomastoid, and sphenosquamous; and two in the parietosquamous suture. Regard-
ing morphology, the Wormian bones were mostly irregular in shape (40 bones), followed
by triangular (4 bones) and, to a lesser extent, oval (1 bone) formats.
Brazilian Journal of Case Reports 2024, 4, 4, 141-149 143 of 149

Figure 1. Number of Wormian bones found in the lambdoid suture.

Figure 2. Wormian bones found in other cranial sutures.

3. Discussion
Wormian bones are additional and small cranial bones that develop from separate
ossification centers. They are found within sutures and fontanelles and are a very common
anatomical variation. In general, the frequency of Wormian bones in the literature has
ranged from 15.9% to 88.8%, with an average of 48.72%. As for location, these bones have
been most frequently found at the level of the lambdoid suture with a frequency of 25%
to 81.1% and an average of 60.4% (Table 1). In the present case, Wormian bones were
Brazilian Journal of Case Reports 2024, 4, 4, 141-149 144 of 149

present in 88.9% at the lambdoid suture. This finding is in accordance with the literature,
which reinforces the relevance of the lambdoid suture as a point for the occurrence of
these bones.
In relation to the locations of Wormian bones in other studies, the literature has
shown a wide range of percentages: in the occipitomastoid suture from 3.8% to 27.14%
[3,10,27,35]; in the asterion from 2.1% to 66.6% [3-5,10,29,33,35-37]; in lambda from 5.26%
to 19.4% [3,5,37-39]; in the parietomastoid suture from 28.9% to 41.43% [10,35]; in the tem-
poroparietal suture (6.1%) [27]; in bregma 1.05% (33); and in the pterion from 0.68% to
36.36% [5,12,35,37,40]. According to Aragão et al., [38], the presence of Wormian bones in
the pterion region could affect access to the anterior and middle cranial fossa, particularly
in surgeries for repairing middle cerebral artery aneurysms and procedures in Broca's
area. In the present case, the Wormian bones, in addition to the lambdoid suture, were
also located in only one bone in the sutures: parietomastoid (2.2%), occipitomastoid
(2.2%), sphenosquamous (2.2%), and two in the parietosquamous suture. (4.4%). The dis-
covery of 40 Wormian bones in the lambdoid suture in this study appears to be the highest
number reported in the literature for the same suture (Table 2).

Table 1. Frequency of skulls with Wormian bones and their presence in lambdoid sutures.
Number of Skulls with
Number of Number of Skulls with
Authors Wormian bonés in lambdoid
Skulls Wormian bones n (%)
sutures n (%)
Walulkar, Ksheersagar,
225 77 (34.22) 57 (74.2)
Walulkar [29]
Shivaleela et al. [30] 108 47 (43.52) 36 (76.6)
Satpathi, Mangalgiri [31] 150 65 (43.33) 37 (56.9)
Showri, Suma [32] 132 74 (56.06) 60 (81.1)
Kumar, Ratna Prabha [2] 200 113 (56.5) 64 (56.63)
Natsis et al. [10] 166 124 (74.7) 74 (44.6)
Goyal, Garg, Kumar [5] 147 52 (35.3) 41 (78.84)
Nayak, Shetty [4] 27 24 (88.8) 6 (25)
Kiliç Safak, Taskin, Yücel
28 12 (42.86) 7 (58.3)
[3]
Ratnaningrum [12] 69 11 (15.9) 8 (72.7)
Asharani [33] 95 24 (25.2) 13 (54.1)
Ortadeveci, Babacan [11] 29 24 (82.8) 15 (62.5)
Amadi-Ikpa et al. [13] 43 13 (30.23) 8 (61.54)
Ogut, Yildirim [34] 110 58 (52.72) 25 (43.1)
Presente estudo 1 - 1 (88.9)

Table 2. Number of Wormian bones in the lambdoid suture.

Authors Number of Wormian bones found in the lambdoid suture
Vishali, Ebenraj, Rojomon, [39] 34
Nayak, Shetty, [4] 20
Kobayashi et al., [27] 6
Ortadeveci, Babacan, [11] 12
Present study 40
Brazilian Journal of Case Reports 2024, 4, 4, 141-149 145 of 149

Regarding the morphology of the Wormian bones, in the present study, 88.9% were
irregular in shape, 8.9% triangular, and 2.2% oval. This finding differs from Walulkar,
Ksheersagar, and Walulkar [29], who reported 46.24% irregular in shape, 39.88% oval, and
13.87% triangular. Since its discovery, several hypotheses have been raised to explain the
presence of Wormian bones. This has led to a large amount of information in the literature,
much of it controversial. However, it appears that some points can currently be consid-
ered: (a) General development – Wormian bones have an irregular morphological appear-
ance and are formed from independent ossification centers found along the cranial sutures
and fontanelles, but the location with the highest significant frequency so far is the lamb-
doid suture (Table 1); (b) Prevalence – (b.1) These are very common anatomical variations
(Table 1), more prevalent in early childhood; (b.2) Studies demonstrate that the younger
the child age, the greater the frequency of Wormian bones [25]; (b.3) Ethnicity or cultural
aspects seem to be related to the frequency of Wormian bones, as studies demonstrate an
increase in occurrence in specific populations; (c) Association with diseases – The presence
of more than 10 Wormian bones may be indicative of genetic or congenital disorders, and
the clinician should investigate carefully when the bones are identified in the prenatal
period or early childhood [9, 22, 25, 41, 42].
During development, various structures can be observed in the squamous portion of
the occipital bone, which are divided into two parts: a superior and an inferior one. The
first part, the interparietal segment, is a membrane bone, while the second part, the su-
praoccipital segment, is a cartilage bone [25, 43]. In addition to this division, three ossifi-
cation centers are observed in the interparietal segment [25]. They grow similarly to other
cranial bones, starting at the center and radiating outwards. This morphological organi-
zation clearly favors the formation of accessory bones at the lambdoid suture as well as
the origin of additional sutures.
For Al Kaissi et al. [6], the formation of these bones results from the non-fusion of the
cranial sutures, a process influenced by genetic and environmental factors. Furthermore,
changes in the sutural bones, commonly identified in radiographic examinations, are at-
tributed to the progressive softening of the sutures, like an excessively stretched dough.
This process is analogously described as the stretching of pastry dough, with the lamb-
doid suture being particularly susceptible to this phenomenon and responsible for the
development of subclinical basilar impression/invagination. The clinical relevance of
changes in sutures goes beyond mere anatomical observation, with direct implications in
pathological conditions such as basilar invagination.
Basilar invagination is a serious complication resulting from the invasion of the odon-
toid process of the axis. This invasion restricts the space in the posterior fossa of the skull,
causing compression of the brain stem and spinal cord in the foramen magnum, which
leads to neurological changes [6, 44]. Patients with this condition must be closely moni-
tored to allow for timely intervention in case it progresses to neural tissue compression.
A study by Al Muroy et al. [25] evaluated CT scans from 167 patients under 2 years
old with mild head trauma. The authors identified the presence of superior median fis-
sures (21%), mendosal sutures (35%), other interparietal segment accessory sutures (9%),
and interparietal sutures (6%) on the occipital bones. Wormian bones within the lambdoid
suture were present in 54 children (32%). Interestingly, superior median fissures were
identified in children aged 0–5 months, with 0 months being the median age. The same
was observed with mendosal suture, with a median age of 1 month. Marti et al., [45] eval-
uated CT scans from 605 patients under 3 years old. They identified Wormian bones in
320 children (53%) and did not find a positive correlation between the presence of bones
and the clinical indication that led to the imaging exam. It is worth mentioning that in this
study, the maximum number of bones found per child was 8. Thus, it is possible to think
that the presence of Wormian bones and additional sutures is just a part of normal devel-
opment in most cases, without any pathological correlations, especially when considering
the presence of less than 10 bones.
Brazilian Journal of Case Reports 2024, 4, 4, 141-149 146 of 149

In addition to age, a factor that appears to be related to the presence of Wormian

bones is ethnicity or cultural aspects, as some studies point to a high incidence in specific
populations. Natsis et al., [10] evaluated 166 adult dry skulls from a Greek cohort and
observed Wormian bones in 74.7% of the cases. Nayak et al. evaluated 24 adult dry skulls
from a South Indian cohort and observed Wormian bones in 88.8% of the cases. However,
in this last study, it must be considered that the sample was small. There are other studies
that point to a high incidence in Indian populations [2, 31], as well as in Chinese [37],
Nepalese [35], among other populations.
The presence of supernumerary bones has been linked to anomalies in the central
nervous system, musculoskeletal system, and metabolic changes, underscoring the signif-
icance of these structures beyond their anatomical role [30,46]. Wormian bones in lamb-
doid suture seems to be the most significative [39]. Conditions such as Menkes disease,
Primrose syndrome, Hajdu-Cheney syndrome, Oto-palato-digital syndrome type II, and
particularly osteogenesis imperfecta have been identified in association with Wormian
bones.
Menkes disease is a disorder caused by mutations in the ATP7A gene, which leads
to reduced copper transport in the body [47]. This results in impaired function of copper-
containing enzymes, affecting various tissues. Menkes disease is characterized by abnor-
mal collagen formation, impacting blood vessels, bones, skin, hair, and the nervous sys-
tem. Symptoms include sparse, kinky hair, harm to growth, hypotonia, neurological
anomalies, and developmental delays [17, 47].
Primrose syndrome is a disorder caused by mutations in the ZBTB20 gene, a tran-
scription factor that regulates neurogenesis, glucose and lipid homeostasis, and postnatal
growth [48]. Symptoms include macrocephaly and a distinct facial appearance, develop-
mental delay, with intellectual, behavioral, and physical-functional impacts, and altered
glucose metabolism [18].
Oto-palato-digital syndrome type II is a genetic disorder within the Otopalatodigital
spectrum disorders (OPDSD) caused by mutations in the FLNA gene. FLNA is a cytoskel-
etal protein that regulates cell membrane integrity and signal transduction. It interacts
with integrins and second messengers, playing a role in cytoskeleton remodeling, cell
shape, and migration (49). This disorder is characterized by skeletal abnormalities and, in
some cases, malformations of the brain and heart [20].
Hajdu-Cheney syndrome is a disorder caused by mutations in exon 34 of the
NOTCH2 gene. Notch receptors are single-pass transmembrane proteins that determine
cell fate and play a critical role in skeletal development and homeostasis [50]. It is a con-
nective tissue disease characterized by acroosteolysis of the hands and feet, developmen-
tal defects of bones, teeth, and joints, associated with progressive osteoporosis, and occa-
sional renal abnormalities. Signs and symptoms include dolichocephaly, absence of
frontal sinuses, joint laxity, dental anomalies, and short stature [19].
Osteogenesis imperfecta is a connective tissue disorder in which mutations in the
COL1A1/COL1A2 genes are considered one of the contributing factors to the excessive
stretching typical of sutures. Null alleles, missense, or splice mutations give rise to differ-
ent types of phenotypes (types I to IV) [45]. Pathogenic variants in non-collagenous genes
are responsible for a smaller number of cases. These genes play various roles such as in
collagen post-modification, collagen folding, intracellular trafficking, ossification, miner-
alization, and osteoblast development. Examples include LEPRE1, CRTAP, PPIB, FKBP10,
SERPINF1, WNT1, CREB3L1, and SP7 genes [51-55]. Clinically, it is characterized by bone
fragility and deformity. multiple fractures, and short stature. Signals and symptoms can
include brittle teeth, blue sclerae, hearing loss, impaired respiratory function, and cardiac
valvular pathology.
Radiographic features of all these diseases include the presence of Wormian bones.
In pathological cases, some characteristics are evident, such as the presence of ten or more
bones, organization in a general mosaic-like pattern and size greater than 6 mm by 4 mm
[22].
Brazilian Journal of Case Reports 2024, 4, 4, 141-149 147 of 149

The exact process by which these bones form is not fully understood. Possibly mul-
tiple genes can be involved in their development, with their effects combining in a com-
plex manner. The expression of these bones is likely influenced by genetic inheritance and
developmental thresholds, although the specific details of these mechanisms are still be-
ing studied. Whether due to genetic causes or mechanical forces in the developing skull,
pathological or induced, the deformation of the cranial joints appears to stimulate the os-
sification process, resulting in the presence of additional sutures, small and irregular
bones near sutures, and sometimes premature closure of sutures and fontanelles.

4. Conclusion
The multiplicity of bones in the lambdoid suture found in this study illustrates the
complexity and anatomical variability of Wormian bones, emphasizing the importance of
these observations for significant contributions to the fields of anthropology, forensic
medicine, and clinical osteology. Unfortunately, it was not possible to include further clin-
ical data about the sample in this study, as well as any associated medical history or ge-
netic information.
Understanding anatomical variations becomes crucial not only to expand scientific
knowledge but also to rule out mistaken diagnoses of bone trauma during cranial x-rays.
In this way, the present study may become relevant for professionals in areas such as ra-
diology, orthopedics, and neurology.

Funding: This research did not receive any specific grants from public, commercial, or non-profit
sector funding agencies.
Research Ethics Committee Approval: We declare that this work did not pass the ethics committee,
as all cadavers found in the Human Anatomy Laboratory of the Higher Education Unit of Feira de
Santana (UNEF) were obtained in accordance with Law 8,501, of November 30, 1992, which pro-
vides for the use of unclaimed corpses for the purposes of studies or scientific research and provides
other measures. However, the study followed the ethical guidelines established by the Declaration
of Helsinki.
Acknowledgments: The authors wish to sincerely thank those who donated their bodies to science
so that anatomical research could be carried out. The results of this research can potentially improve
patient care and increase the overall knowledge of humanity. Therefore, these donors and their
families deserve our utmost gratitude.
Conflicts of Interest: None.
Supplementary Materials: None.

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