A bifid nose is a rare birth defect in which the nose is divided into two separate parts. The extent to which the nose is affected can be very different from one person to the next. In its mildest form, there is a small, minimally noticeable groove at the tip of the nose. In the most severe form, the bones and cartilage of the nose are completely divided, resulting in two half noses. Bifid nose is seen fairly often with widely spaced eyes and cleft lip/palate (an opening in the lip and or roof of the mouth). Sometimes a bifid nose is the only difference in development or birth defect in a person. Other times a bifid nose can be part of a condition that includes multiple differences in growth and function such as the fusion of both halves of the brain.
Bifid nose can appear for the first time in a person or be passed from one generation to the next based on the inheritance of one or more gene changes (mutations).
Patients with a bifid nose are encouraged to seek medical services through a specialized craniofacial clinic. Craniofacial clinics specialize in the diagnosis, treatment and follow-up care of patients with a face and skull differences like a bifid nose. The following organizations provide a list of craniofacial clinics throughout the world on their websites:
FACES: The National Craniofacial Association
Children’s Craniofacial Association
World Craniofacial Foundation
Bifid nose may also be called:
median nasal cleft
median fissure of the nose
double nose
cleft nose
nasal cleft
Frequently Asked Questions About Bifid nose
Where can I find other people with autosomal recessive bifid nose?
There are several organizations that serve the needs of patients and families living with a craniofacial difference. A bifid nose is a type of craniofacial difference. A craniofacial difference is a malformation of the head or face. One or more of the organizations listed below may be able to connect you with another patient (or patients) with a bifid nose:
What is bifid nose?
A bifid nose is a rare birth defect in which the nose is divided into two separate parts. The extent to which the nose is affected can be very different from one person to the next. In its mildest form, there is a small, minimally noticeable groove at the tip of the nose. In the most severe form, the bones and cartilage of the nose are completely divided, resulting in two half noses. Bifid nose is seen fairly often with widely spaced eyes and cleft lip/palate (an opening in the lip and or roof of the mouth). Sometimes a bifid nose is the only difference in development or birth defect in a person. Other times a bifid nose can be part of a condition that includes multiple differences in growth and function such as the fusion of both halves of the brain.
Bifid nose can appear for the first time in a person or be passed from one generation to the next based on the inheritance of one or more gene changes (mutations).
Patients with a bifid nose are encouraged to seek medical services through a specialized craniofacial clinic. Craniofacial clinics specialize in the diagnosis, treatment and follow-up care of patients with a face and skull differences like a bifid nose. The following organizations provide a list of craniofacial clinics throughout the world on their websites:
What are the signs and symptoms of autosomal recessive bifid nose?
A bifid nose is a rare birth defect of the nose. This malformation causes the nose to be divided into two separate parts. The extent to which the nose is affected can be very different from one patient to the next. In its mildest form, there is a small, minimally noticeable groove at the tip of the nose. In the most severe form, the bones and cartilage of the nose are completely divided, resulting in two half noses. About 8% of all people who have a bifid nose also have wide-spaced eyes, called hypertelorism. Additional individuals can have a opening in the lip or roof of the mouth (cleft lip and palate) with the bifid nose. Still other people with a bifid nose have a genetic condition called BNAR syndrome or "bifid nose, renal agenesis, and anorectal malformations syndrome" that is caused a change in the FREM1 gene. In addition to a bifid nose, people with BNAR syndrome also usually have kidney, anus, and rectum defects. The anus is the opening between the buttocks where the large intestine ends through which stool leaves the body. The rectum is the last part of the large intestine just above the anus.
The best way to determine if a bifid nose is part of a genetic syndrome or running in a family is to talk to a medical geneticist and genetic counselor. A medical geneticist can be found by asking your doctor for a referral or looking on the American College of Medical Geneticists website . Genetic counselors in the United States can be found on the National Society of Genetic Counselors website. Genetic counselors in Canada can be found at the Canadian Association of Genetic Counselors website.
Is there treatment for autosomal recessive bifid nose?
Treatment for a bifid nose typically consists of surgical reconstruction to repair the malformation of the nose. Depending on the severity of the bifid nose, the surgery may be quite challenging. Because the specific malformations seen in bifid nose can differ greatly from one patient to the next, an individualized plan for surgery is necessary. Patients are encouraged to seek surgical services through a specialized craniofacial team. These teams specialize in the diagnosis, treatment and follow-up care of patients with a craniofacial difference. A craniofacial difference is a malformation of the head or face. A bifid nose is a type of craniofacial difference. The following organizations provide a list of craniofacial clinics throughout the world on their websites:
Is there clinical research going on for bifid nose?
As of July 2019, there were no clinical research studies being conducted on bifid nose specifically.
For updated information on clinical trials for bifid nose or craniofacial malformations, you can visit www.clinicaltrials.gov.
How is autosomal recessive bifid nose inherited?
Inheritance refers to how a condition is passed from one generation to another in a family. A bifid nose found with no other health problems can be inherited in an autosomal recessive or an autosomal dominant pattern.
In autosomal recessive bifid nose, the nose malformation is caused when a person inherits a change or mutation in the same gene from both their parents. In autosomal dominant bifid nose, the nose malformation can happen when a change or mutation is passed down from just one parent.
Genes are structures found within a person’s cells that contain the body’s genetic information. People have about 20,000-25,000 genes. Genes are inherited from our parents and passed on to our children. Most genes come in pairs, meaning there are two copies of each gene. A person inherits one copy of each gene from their mother and the other copy of each gene from their father. Genes are like our body’s instruction manual – they control the growth, development and normal function of the body. When mutations happen in our genes, they cannot work properly. It is like a section of the body’s instruction manual is not correct, and it causes problems with the normal development and functioning of the body.
Scientists have not yet pinpointed all specific gene or genes that are associated with bifid nose without other symptoms that is running in the family.
In bifid nose seen with other birth defects or health problems, there are several genetic conditions caused by different genes that run in families. One inherited form of bifid nose that appears with kidney, anus, and rectum defects is called BNAR syndrome or "bifid nose, renal agenesis, and anorectal malformations syndrome". BNAR syndrome is caused a change in the FREM1 gene.
A medical geneticist (a doctor specially trained to diagnose and treat genetic conditions) can help figure out if someone has autosomal recessive, autosomal dominant bifid nose, or another disorder involving a bifid nose. A medical geneticist can be found by asking your doctor for a referral or looking on the American College of Medical Geneticists website .
How is autosomal recessive bifid nose caused?
A bifid nose occurs when the left and right sides of the nose, also called the medial and lateral nasal processes, fail to connect properly when the nose is being formed. This typically occurs during the first two months of a pregnancy.
There are several reasons why a bifid nose may happen. In some cases the malformation is the result of a problem or interference in forming the nose before birth and is not related to a genetic change. Other times, a bifid nose is related to one or more unexpected changes, called mutations, in the genes. Still other times the gene change is passed down from one or both parents. One inherited form of bifid nose that appears with kidney, anus, and rectum defects is called BNAR syndrome or "bifid nose, renal agenesis, and anorectal malformations syndrome". BNAR syndrome is caused a change in the FREM1 gene.
Genes are structures found within a person’s cells that contain the body’s genetic information. People have about 20,000-25,000 genes. Genes are inherited from our parents and passed on to our children. Most genes come in pairs, meaning that there are two copies of each gene. A person inherits one copy of each gene from their mother and the other copy of each gene from their father. Genes are like our body’s instruction manual – they control the growth, development and normal function of the body. When mutations happen in our genes, they cannot work properly. It is like a section of the body’s instruction manual is not correct, and it causes problems with the normal development and functioning of the body. Scientists have not yet pinpointed the specific gene or genes that are associated with all forms of bifid nose.
The best way to learn more about the possibility of a bifid nose happening in other family members or to determine if someone’s bifid nose is caused by a single gene change is to consult a genetic counselor or medical geneticist. A medical geneticist can be found by asking your doctor for a referral or looking on the American College of Medical Geneticists website . Genetic counselors in the United States can be found on the National Society of Genetic Counselors website. Genetic counselors in Canada can be found at the Canadian Association of Genetic Counselors website.
How do I know if I have autosomal recessive bifid nose?
A bifid nose is usually found after a doctor examines a person’s nose carefully. The nose change may be very mild and not picked up until adulthood or severe and obvious at birth. If more than one family member has a bifid nose or the bifid nose appears with other health problems and/or birth defects, genetic testing may be done to identify what gene change is causing the bifid nose. Although the genetic cause for all forms of bifid nose is not known, there are some genetic tests available for the known genetic causes of bifid nose. Speaking with a genetics professional can help to determine which tests might be relevant in your family.
A medical geneticist can be found by asking your doctor for a referral or looking on the American College of Medical Geneticists website . Genetic counselors in the United States can be found on the National Society of Genetic Counselors website. Genetic counselors in Canada can be found at the Canadian Association of Genetic Counselors website.
How can I find a specialist for autosomal recessive bifid nose?
Patients with a bifid nose are encouraged to seek medical services through a specialized craniofacial clinic. A bifid nose is a type of craniofacial difference. A craniofacial difference is a malformation of the head or face. Craniofacial clinics specialize in the diagnosis, treatment and follow-up care of patients with a craniofacial difference. The following organizations provide a list of craniofacial clinics throughout the world on their websites:
Do I need to see special doctors for autosomal recessive bifid nose?
Patients with a bifid nose are encouraged to seek medical services through a specialized craniofacial clinic. A bifid nose is a type of craniofacial difference. A craniofacial difference is a malformation of the head or face. Craniofacial clinics specialize in the diagnosis, treatment and follow-up care of patients with a craniofacial difference. The following organizations provide a list of craniofacial clinics throughout the world on their websites:
Are there other names for bifid nose?
Bifid nose may also be called:
Are there other conditions that look like autosomal recessive bifid nose?
A bifid nose is a rare birth defect of the nose. This malformation causes the nose to be divided into two separate parts. In some people, a bifid nose occurs without any other major symptoms. However, a bifid nose can also be seen with other health problems as a part of other genetic disorders or clusters of birth defects. For example, some people with a bifid nose have a genetic condition called BNAR syndrome or "bifid nose, renal agenesis, and anorectal malformations syndrome" that is caused a change in the FREM1 gene. In addition to a bifid nose, people with BNAR syndrome also usually have kidney, anus, and rectum defects. The anus is the opening between the buttocks where the large intestine ends through which stool leaves the body. above the anus). Some other examples of genetic conditions that a bifid nose can be seen in include:
Are there any support groups for individuals with autosomal recessive bifid nose?
There are several support groups that patients with bifid nose and their families may find helpful. A bifid nose is a type of craniofacial difference. A craniofacial difference is a malformation of the head or face. There are a number of organizations that serve the needs of patients and families living with a craniofacial difference. Several are listed below:
Are there any genes involved in autosomal recessive bifid nose?
Scientists have not yet pinpointed the specific gene or genes that are associated with all forms of bifid nose. One inherited form of bifid nose that appears with kidney, anus, and rectum defects is called BNAR syndrome or "bifid nose, renal agenesis, and anorectal malformations syndrome". BNAR syndrome is caused by a change in the FREM1 gene.
Genes are structures found within a person’s cells that contain the body’s genetic information. People have about 20,000-25,000 genes. Genes are inherited from our parents and passed on to our children. Most genes come in pairs, meaning that there are two copies of each gene. A person inherits one copy of each gene from their mother and the other copy of each gene from their father. Genes are like our body’s instruction manual – they control the growth, development and normal function of the body. When unexpected changes, called mutations, happen in our genes, they cannot work properly. It is like a section of the body’s instruction manual is not correct, and it causes problems with the normal development and functioning of the body.