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A molera (also known as a fontanel) is a "soft spot" on the top of a Chihuahua's skull; it is the equivalent to the bregmatic or anterior fontanelle in human babies, but unlike most mammals Chihuahua's fontanelle persist into maturity. Historically it has been very common amongst Chihuahuas and was regarded as a mark of purity for this miniature dog breed. It is still mentioned in many Chihuahua breed standards, however, it is considered a fault in European countries because of concern that this might reflect underlying malformations such as hydrocephalus and ventriculomegaly, Chiari-like malformation and syringomyelia. Fontanelles are fibrous, membrane-covered gaps that lie between the skull bones and at the intersection of the cranial sutures. The cranial sutures are the junctions between cranial (or skull) bones. The fontanelles serve as the major sites of bone expansion during post-natal skull growth which accommodates the enlarging brain. The Chihuahua likely has a molera because of neuroparenchymal disproportion i.e. a proportionally big brain for the skull. This is likely because there is premature closure of the skull base cranial sutures (brachycephaly due to craniosynostosis). To accommodate the developing brain there is increased growth of the skull bone in a parallel plane giving the dog a characterised domed or "apple-headed" appearance.
A Finnish study from (University of Helsinki with University of Surrey) found that increased number and size of persistent fontanelles in Chihuahuas were associated with small body size, syringomyelia (fluid filled cavities in the spinal cord), ventriculomegaly (i.e. enlargement of the lateral ventricles), and craniocervical junction abnormalities. The challenges the belief that a molera is clinically irrelevant finding not associated with other structural abnormalities. [1]
Explanation of often misquoted veterinary sources (Greene and Braund / Rivers and Walker) Many internet sources state incorrectly that there was a study on molera performed by [2] which (paraphrasing) "did not find an connection between moleras and hydrocephalus in toy breeds such as the Chihuahua". There was no such study. This was an unreferenced statement, although it reflected clinical experience, and was made over 30 years ago in the 3rd edition of Ettinger's Veterinary Internal Medicine. This statement was not kept in subsequent editions and this tome is now being revised for the 9th edition. It is also incorrectly stated on multiple websites that there was "a separate study conducted by Dr. Walker and Dr. Rivers at the University of Minnesota that found no correlation between the presence or size of a molera and hydrocephalus". This is also incorrect. This study was entitled "Hydrocephalus in the Dog: Utility of Ultrasonography as an Alternate Diagnostic Imaging Technique" [3] published in Journal of the American Animal Hospital Association in 1992. The question that this study actually addressed was whether ventricles and associated cerebrospinal fluid pathways could be investigated by ultrasound through the persistent bregmatic fontanelle (molera). There were 26 dogs in the study of which 6 had clinical hydrocephalous (i.e. were neurologically abnormal). Of the 6 dogs with clinical hydrocephalus (2 x chihuahua, 1 x poodle x, 1 x Pomeranian, 1 x Boston terrier and 1 x Yorkshire terrier), ultrasound through the molera was helpful in confirming the diagnosis of hydrocephalus noninvasively and provided information about dilation of the lateral, 3rd and cranial cerebral aqueduct. Ultrasound through the molera was also useful in revealing ventriculomegaly in 5 of 20 (supposedly) clinically normal dogs and these included Chihuahua (9 months old, mentation depressed), Lhasa Apso (5 weeks old), 2x Shih Tzu (3 and 5 weeks old) and Affenpinscher (9 weeks old – and returned to breeder because dull).The remaining 15 dogs (14 shih tzu and 1 Lhasa Apso) had normal ventricle size according to this ultrasound study. 14/15 of the dogs in the study were 6 weeks old or less i.e. the study was predominantly in puppies not adult dogs. The older dog was a 5 year old Shih Tzu. In other words all the Chihuahua in this study were affected with hydrocephalus and molera; this study did not investigate presence or size of a molera and hydrocephalus.
Syringomyelia is a generic term referring to a disorder in which a cyst or cavity forms within the spinal cord. Often, syringomyelia is used as a generic term before an etiology is determined. This cyst, called a syrinx, can expand and elongate over time, destroying the spinal cord. The damage may result in loss of feeling, paralysis, weakness, and stiffness in the back, shoulders, and extremities. Syringomyelia may also cause a loss of the ability to feel extremes of hot or cold, especially in the hands. It may also lead to a cape-like bilateral loss of pain and temperature sensation along the upper chest and arms. The combination of symptoms varies from one patient to another depending on the location of the syrinx within the spinal cord, as well as its extent.
Say–Meyer syndrome is a rare X-linked genetic disorder that is mostly characterized as developmental delay. It is one of the rare causes of short stature. It is closely related with trigonocephaly. People with Say–Meyer syndrome have impaired growth, deficits in motor skills development and mental state.
The skull is a bone protective cavity for the brain. The skull is composed of four types of bone i.e., cranial bones, facial bones, ear ossicles and hyoid bone. However two parts are more prominent: the cranium and the mandible. In humans, these two parts are the neurocranium and the viscerocranium that includes the mandible as its largest bone. The skull forms the anterior-most portion of the skeleton and is a product of cephalisation—housing the brain, and several sensory structures such as the eyes, ears, nose, and mouth. In humans these sensory structures are part of the facial skeleton.
A fontanelle is an anatomical feature of the infant human skull comprising soft membranous gaps (sutures) between the cranial bones that make up the calvaria of a fetus or an infant. Fontanelles allow for stretching and deformation of the neurocranium both during birth and later as the brain expands faster than the surrounding bone can grow. Premature complete ossification of the sutures is called craniosynostosis.
Hydrocephalus is a condition in which an accumulation of cerebrospinal fluid (CSF) occurs within the brain. This typically causes increased pressure inside the skull. Older people may have headaches, double vision, poor balance, urinary incontinence, personality changes, or mental impairment. In babies, it may be seen as a rapid increase in head size. Other symptoms may include vomiting, sleepiness, seizures, and downward pointing of the eyes.
Chiari malformation (CM) is a structural defect in the cerebellum, characterized by a downward displacement of one or both cerebellar tonsils through the foramen magnum. CMs can cause headaches, difficulty swallowing, vomiting, dizziness, neck pain, unsteady gait, poor hand coordination, numbness and tingling of the hands and feet, and speech problems. Less often, people may experience ringing or buzzing in the ears, weakness, slow heart rhythm, or fast heart rhythm, curvature of the spine (scoliosis) related to spinal cord impairment, abnormal breathing, such as central sleep apnea, characterized by periods of breathing cessation during sleep, and, in severe cases, paralysis.
Colpocephaly is a cephalic disorder involving the disproportionate enlargement of the occipital horns of the lateral ventricles and is usually diagnosed early after birth due to seizures. It is a nonspecific finding and is associated with multiple neurological syndromes, including agenesis of the corpus callosum, Chiari malformation, lissencephaly, and microcephaly. Although the exact cause of colpocephaly is not known yet, it is commonly believed to occur as a result of neuronal migration disorders during early brain development, intrauterine disturbances, perinatal injuries, and other central nervous system disorders. Individuals with colpocephaly have various degrees of motor disabilities, visual defects, spasticity, and moderate to severe intellectual disability. No specific treatment for colpocephaly exists, but patients may undergo certain treatments to improve their motor function or intellectual disability.
Hydranencephaly is a condition in which the brain's cerebral hemispheres are absent to a great degree and the remaining cranial cavity is filled with cerebrospinal fluid. "Cephalic" is the scientific term for "head" or "head end of body".
The Cavalier King Charles Spaniel is a British breed of toy dog of spaniel type. Four colours are recognised: Blenheim, tricolour (black/white/tan), black and tan, and ruby; the coat is smooth and silky. The lifespan is usually between eight and twelve years.
Intracranial pressure (ICP) is the pressure exerted by fluids such as cerebrospinal fluid (CSF) inside the skull and on the brain tissue. ICP is measured in millimeters of mercury (mmHg) and at rest, is normally 7–15 mmHg for a supine adult. The body has various mechanisms by which it keeps the ICP stable, with CSF pressures varying by about 1 mmHg in normal adults through shifts in production and absorption of CSF.
A lap dog or lapdog is a dog that is both small enough to be held in the arms or lie comfortably on a person's lap and temperamentally predisposed to doing so. Lapdog is not a specific breed, but a generic term for a type of dog that is small in size and friendly towards humans.
Choroid plexus cysts (CPCs) are cysts that occur within choroid plexus of the brain. They are the most common type of intraventricular cyst, occurring in 1% of all pregnancies.
The Tibetan Terrier is a medium-sized breed of dog that originated in Tibet. Despite its name, it is not a member of the terrier group. The breed was given its English name by European travelers due to its resemblance to known terrier breeds. The Tibetan name for the breed, Tsang Apso, roughly translates to "shaggy or bearded ("apso") dog, from the province of Tsang". Some old travelers' accounts refer to the dog as Dokhi Apso or "outdoor" Apso, indicating a shaggy or bearded working dog which lives outdoors.
The frontal suture is a fibrous joint that divides the two halves of the frontal bone of the skull in infants and children. Typically, it completely fuses between three and nine months of age, with the two halves of the frontal bone being fused together. It is also called the metopic suture, although this term may also refer specifically to a persistent frontal suture.
Dandy–Walker malformation (DWM), also known as Dandy–Walker syndrome (DWS), is a rare congenital brain malformation in which the part joining the two hemispheres of the cerebellum does not fully form, and the fourth ventricle and space behind the cerebellum are enlarged with cerebrospinal fluid. Most of those affected develop hydrocephalus within the first year of life, which can present as increasing head size, vomiting, excessive sleepiness, irritability, downward deviation of the eyes and seizures. Other, less common symptoms are generally associated with comorbid genetic conditions and can include congenital heart defects, eye abnormalities, intellectual disability, congenital tumours, other brain defects such as agenesis of the corpus callosum, skeletal abnormalities, an occipital encephalocele or underdeveloped genitalia or kidneys. It is sometimes discovered in adolescents or adults due to mental health problems.
Cleidocranial dysostosis (CCD), also called cleidocranial dysplasia, is a birth defect that mostly affects the bones and teeth. The collarbones are typically either poorly developed or absent, which allows the shoulders to be brought close together. The front of the skull often does not close until later, and those affected are often shorter than average. Other symptoms may include a prominent forehead, wide set eyes, abnormal teeth, and a flat nose. Symptoms vary among people; however, intelligence is typically unaffected.
Ventriculomegaly is a brain condition that mainly occurs in the fetus when the lateral ventricles become dilated. The most common definition uses a width of the atrium of the lateral ventricle of greater than 10 mm. This occurs in around 1% of pregnancies. When this measurement is between 10 and 15 mm, the ventriculomegaly may be described as mild to moderate. When the measurement is greater than 15mm, the ventriculomegaly may be classified as more severe.
Choroid plexus papilloma, also known as papilloma of the choroid plexus, is a rare benign neuroepithelial intraventricular WHO grade I lesion found in the choroid plexus. It leads to increased cerebrospinal fluid production, thus causing increased intracranial pressure and hydrocephalus.
Chiari-like malformation (CM) the most common cause of foramen magnum obstruction and syringomyelia in dogs. Syringomyelia (SM) is a disease of the spinal cord typified by fluid filled cavities, or syrinxes, within the spinal cord substance but it can cause pain by disrupting the cerebrospinal fluid (CSF), in the brain CM is a condition characterized by the mismatch of size between the brain and the skull. CM is very widespread in many Toy breed dogs and has been studied in the Cavalier King Charles Spaniel and the Griffon Bruxellois and Chihuahua. As many as 95% of Cavalier King Charles Spaniels may have CM. It is worldwide in scope and not limited to any country, breeding line, or kennel, and experts report that it is believed to be inherited CM is so widespread in the Cavalier that it may be an inherent part of the CKCS's breed standard. This disease not only affects thousands of dogs, but a similar condition affects over three hundred thousand children yearly. Therefore, canines are an appropriate model for the treatment of the human condition.
Cranial ultrasound is a technique for scanning the brain using high-frequency sound waves. It is used almost exclusively in babies because their fontanelle provides an "acoustic window". A different form of ultrasound-based brain scanning, transcranial Doppler, can be used in any age group. This uses Doppler ultrasound to assess blood flow through the major arteries in the brain, and can scan through bone. It is not usual for this technique to be referred to simply as "cranial ultrasound". Additionally, cranial ultrasound can be used for intra-operative imaging in adults undergoing neurosurgery once the skull has been opened, for example to help identify the margins of a tumour.
Kiviranta, A‐M, Rusbridge, C, Lappalainen, AK, Junnila, JJT, Jokinen, TS. Persistent fontanelles in Chihuahuas. Part II: Association with craniocervical junction abnormalities, syringomyelia, and ventricular volume. J Vet Intern Med. 2021; 1– 9. https://doi.org/10.1111/jvim.16123
Greene CE. Braund KG. Diseases of the brain. In: Ettinger Sl, ed. Textbook of veterinary internal medicine. 3rd cd. Philadelphia: WB Saunders, 1989:pp584
Rivers WJ, Walter PA Hydrocephalus in the Dog: Utility of Ultrasonography as an Alternate Diagnostic Imaging Technique, Journal of the American Animal Hospital Association, 1992, 28, 578-623