A cleft nose is a nasal deformity that occurs alongside a cleft lip, where the structure of the nose is altered because the tissues of the upper lip and nasal floor didn’t fully fuse during early fetal development. It affects the shape, symmetry, and sometimes the function of the nose. Cleft lip and palate together represent the most common congenital craniofacial abnormality, occurring in roughly 1 in 500 to 1,000 live births, and all complete cleft lips (those extending into the nasal floor) involve the nose to some degree.
How a Cleft Nose Looks and Feels Different
The specific appearance depends on whether the cleft is on one side (unilateral) or both sides (bilateral), and how severe it is. In a unilateral cleft nose, the nostril on the cleft side is typically wider and flatter, the columella (the strip of tissue between the nostrils) is shorter on that side, and the nasal tip may lean away from the cleft. The base of the nose often looks asymmetrical because the cartilage that shapes the nostril rim has been displaced.
In a bilateral cleft nose, both sides are affected. The columella can be extremely short, sometimes so short that the lip appears to connect almost directly to the nasal tip. The nostrils tend to flare outward, the nasal tip is flat and wide, and the floor of the nose is absent on both sides. The cartilages that normally give the nose its shape are splayed apart rather than sitting in their usual position.
Beyond appearance, a cleft nose commonly causes breathing problems. The nasal septum (the wall dividing the two nasal passages) is frequently deviated toward the non-cleft side, narrowing one airway. This obstruction can occur at multiple levels inside the nose, increasing the risk of habitual mouth breathing and sleep-disordered breathing.
Why It Happens During Development
The face forms between the fourth and tenth weeks of pregnancy through the migration of specialized cells called cranial neural crest cells. During the fifth week, structures called the medial and lateral nasal processes take shape around the developing nasal pits. The medial nasal processes are responsible for forming the central upper lip, the nasal tip, the columella, and the primary palate. The lateral nasal processes form the sides of the nose.
When these processes fail to grow together and fuse properly, the result is a cleft. Because the lip and nose develop from the same embryonic structures, a cleft in the lip almost always disrupts the nose as well. The exact point during development when fusion is interrupted determines how severe the cleft will be. A minor interruption might produce only a small notch in the lip with subtle nasal asymmetry, while a major failure of fusion creates a complete cleft extending from the lip through the nasal floor.
Both genetic and environmental factors contribute. Animal studies have identified several genes involved in the signaling pathways that guide facial fusion. Disruptions in growth factors that control cell adhesion and tissue remodeling in the midline of the face can prevent the palatal shelves from meeting and fusing. Certain genetic syndromes also include clefting as a feature.
Rare Midline Nasal Clefts
Most cleft noses are associated with standard cleft lip, but there is a rarer category: a true midline cleft of the nose. This occurs when the nose itself is split down the center, sometimes with a visible groove or gap along the nasal bridge. In the Tessier classification system (a numbering system surgeons use to categorize facial clefts from 0 to 14), clefts involving the nose are designated by specific numbers depending on their path. A Tessier 0 cleft runs straight down the midline. A Tessier 1 cleft passes through the nostril rim at its middle third and extends upward along the nasal bridge.
Midline nasal clefts are a hallmark of frontonasal dysplasia, also called median cleft face syndrome. This condition can include widely spaced eyes, a broad nasal bridge, and in some cases a gap in the skull’s midline. Related but distinct conditions include craniofrontonasal dysplasia, which involves premature fusion of skull bones, and frontofacionasal dysplasia, which adds eye defects and underdevelopment of the midface.
Treatment Timeline From Infancy to Adolescence
Correcting a cleft nose is not a single event. It unfolds over years in stages timed to a child’s growth.
The earliest intervention can begin before any surgery. A technique called nasoalveolar molding (NAM) uses a custom oral appliance with nasal prongs to gently reshape the nose and align the gum segments in the weeks after birth. Studies comparing babies who received NAM to those who didn’t show measurably better nasal symmetry: the deviation of the columella was significantly smaller in the NAM group, nostril height differences between the cleft and non-cleft sides were reduced by roughly 80%, and the sideways displacement of the nasal dome was brought much closer to the non-cleft side. NAM also significantly decreased the likelihood of needing nasal revision surgery later.
The first surgical repair of the lip typically happens around three to six months of age, and surgeons often reshape the nose at the same time, repositioning the displaced cartilages and rebuilding the nasal floor. This primary repair addresses the most obvious asymmetry but cannot fully correct the nose because the structures are still growing.
Minor refinements to the nasal tip can be performed between ages 5 and 15, but the definitive correction, a full septorhinoplasty, is delayed until skeletal maturity, generally between ages 15 and 18. Operating before the facial skeleton has finished growing risks disrupting further development.
What Definitive Rhinoplasty Involves
The final nasal surgery for cleft patients is more complex than a standard cosmetic rhinoplasty. The cartilage framework of a cleft nose is often weakened, scarred from previous surgeries, or structurally insufficient. Surgeons frequently need to add cartilage grafts to rebuild the internal support of the nose, improve the nasal tip projection, and straighten the septum to open the airway.
The cartilage for these grafts can come from the patient’s own nasal septum if enough usable tissue remains, but in many cleft cases, rib cartilage is the preferred source. It provides a large supply of strong, supportive material with no risk of rejection since it comes from the patient’s own body. The sixth or seventh rib is most commonly used, though the eighth, ninth, or tenth ribs may be chosen when more material is needed. Ear cartilage is another option for smaller grafts.
Specific graft types serve different purposes. Spreader grafts are placed along the nasal bridge to widen a collapsed middle section and improve airflow. Septal extension grafts are used to push the nasal tip forward and give it better definition. In a large cohort study following cleft patients over 30 years, 57% ultimately underwent a definitive rhinoplasty, and the revision rate after that final procedure was about 10%.
Breathing and Long-Term Function
While the appearance of a cleft nose draws the most attention, the functional effects matter just as much. Airway obstruction in cleft nose patients is well documented and stems from multiple overlapping problems: the deviated septum narrows one passage, the weakened cartilage of the outer nose may not hold its shape during inhalation, and scar tissue from earlier surgeries can further restrict airflow.
In unilateral cleft noses, the septum reliably deviates toward the non-cleft side, with the nasal bridge also shifting in that direction. This pattern means the non-cleft side is often the more obstructed passage, which can surprise patients who assume the cleft side would be the problem. Correcting this during the definitive rhinoplasty involves straightening the septum, reinforcing the outer nasal wall, and ensuring both passages remain open.
Children and adults with uncorrected cleft nose deformities are more likely to breathe through their mouths, which over time can affect dental development, sleep quality, and exercise tolerance. Addressing both the structural and functional components of the nose in a single well-timed surgery gives the best chance of a lasting result.