The clinical presentation of cleft lip is highly variable. The most frequently observed appearance is a left sided, unilateral cleft lip accompanied by a cleft palate. Other combinations include a unilateral or bilateral cleft lip with or without a cleft palate. Note that a cleft palate is found in nearly 90% of children with a bilateral cleft lip and almost 70% of patients with a unilateral cleft.

With regards to comorbid anomalies, 25% of children with a cleft lip exhibit cardiac and neurologic defects, and clubfoot [6] [7].

Complications

Children with clefts develop trouble with speech and hearing, as well dental deformities. Additionally, they are at risk for developmental and social delays [8].

Clinical signs

In addition to the remarkable physical appearance of the cleft lip, the infant has difficulty with sucking and feeding, especially if a cleft palate is also present.

• Dentist

  To correct the condition, you will likely work with a team involving a plastic surgeon, maxillofacial surgeon, an ENT (ear, nose and throat specialist), general dentist, orthodontist and oral surgeon. [uthscsa.edu]

  Children with a cleft should be seen by a dentist shortly after their first teeth come through. It is important to see a dentist experienced in the care of people with clefts. It is important that your child's teeth are looked after carefully. [rch.org.au]

  A pediatric dentist or orthodontist can assist with this problem. [entnet.org]

  Halofsky, a Pediatric dentist. A facial impression and x-rays are also taken at this time. 2 INFANT MOLD LATHAM DEVICE Dr. Ralph Latham, inventor of this device, custom makes all of the Latham Devices. [pbase.com]

• Pathologist

  Your child's speech pathologist will help your child to create all the sounds needed for speech, which will be easier for them once the cleft palate has been repaired through surgery. [rch.org.au]

  The prosthodontist coordinates treatment with the oral or plastic surgeon and with the speech pathologist. [webmd.com]

  Services are provided by speech-language pathologists who work in conjunction with dental and medical specialists, including audiologists, plastic surgeons and ear-nose-throat specialists. [hollandbloorview.ca]

• Surgical Procedure

  After identifying the underlying pathology, platelet infusions and aminocaproic acid were given to decrease bleeding from a second surgical procedure. [ncbi.nlm.nih.gov]

  Our team performs more than 1,000 surgical repairs each year. Read on below for an introduction to the various procedures that may be included in the surgical repair of cleft lip and cleft palate at Children's Hospital. [chop.edu]

• Disability

  The Equality and Human Rights Commission said that this element of the Abortion Act "is offensive to many people; it reinforces negative stereotypes of disability and is incompatible with valuing disability and non-disability equally". [www1.cbn.com]

  Stories such as Aidan’s and Manning’s ought to encourage parents who have children with disabilities. Their stories prove that disabilities do not define a person or hinder them from achieving success. [lifenews.com]

  Disability and Health (ICF). [who.int]

  Curtis Rogers, Malformations among the X‐linked intellectual disability syndromes, American Journal of Medical Genetics Part A, 161, 11, (2741-2749), (2013). [doi.org]

  As a matter of courtesy we request that the content provider (Centers for Disease Control and Prevention, National Center on Birth Defects and Developmental Disabilities) be credited and notified in any public or private usage of this image. [web.archive.org]

• Pain

  […] categories Bones, Joints & Spine Heart & Vessels Blood & Cancer Surgery Visceral Surgery Plastic and Aesthetic Surgery Gynecology & Reproduction Urology & Prostate Internal organs Head & Throat Lungs & Allergy Children Skin & Dermatology Psyche & Nerves Pain [leading-medicine-guide.com]

  For a day or two, your child will feel mild pain, which can be relieved with a nonaspirin pain medication. A prescription medication may also be given for use at home. [stanfordchildrens.org]

  Prescription pain medicine may be given. Ask your child's healthcare provider how to give this medicine safely. Some prescription pain medicines contain acetaminophen. [drugs.com]

  Your child will have an intravenous line (IV) to provide fluids, pain medicines, and antibiotics. A monitor will be used to observe your child's heart rate and breathing patterns while receiving IV pain medicines. [childrensmn.org]

  After surgery for cleft palate: Your child may experience more discomfort and pain with cleft palate repair than cleft lip repair. Your child may have nasal congestion. This can be relieved with medication. [chop.edu]

• Pharyngitis

  The patient was born with a unilateral cleft lip and palate and underwent primary lip and palate repair and later a pharyngeal flap for severe velopharyngeal insufficiency. [ncbi.nlm.nih.gov]

  Substitute pharyngeal fricatives for fricative consonants. [d.umn.edu]

  The Branchial Region The Branchial or Visceral Arches and Pharyngeal Pouches. [bartleby.com]

  The surgical techniques most commonly used for treatment of velopharyngeal insufficiency are bilateral sphincter pharyngoplasty, Furlow palatoplasty, and superior pharyngeal flap. [web.archive.org]

• Nasal Congestion

  You may notice signs of nasal congestion, a bit of blood or blood-tinged discharge from the nose or mouth, some swelling or bruising and decreased appetite. [hopkinsmedicine.org]

  Many infants show signs of nasal congestion after surgery. These signs may include nasal snorting, mouth breathing, and decreased appetite. Your child's doctor may prescribe medication to relieve the nasal congestion. [stanfordchildrens.org]

  Your child may have nasal congestion. This can be relieved with medication. Your child may stay in the hospital for one to three days and will be given antibiotics to prevent infection. Your child will have stitches on his palate. [chop.edu]

• Failure to Thrive

  These problems can lead to babies becoming malnourished, causing failure to thrive unless special help and advice is given. If a cleft palate is not repaired surgically, speech problems will develop when a child is older. [bapras.org.uk]

  Although not a major cause of mortality in the developed world, it can lead to poor feeding, failure to thrive and impaired survival in places where healthcare resources are poor. [patient.info]

  Frequent early symptoms include feeding difficulties, nasal regurgitation, malnutrition, failure to thrive and hearing loss, while later in life, speech and voice problems and orthodontic problems often necessitate treatment [ 6, 7, 10, 14 ]. [doi.org]

• Diarrhea

  Pharyngitis, rhinitis, cheilitis, dysphagia, or diarrhea may follow reduced mucous glands in the respiratory and gastrointestinal tracts. Recurrent high fever may lead to seizures and neurological sequelae. [doctors.co.il]

• Abnormal Teeth

  As a result of the abnormalities, teeth may not develop normally and orthodontic treatment may be required. [hopkinsmedicine.org]

  As a result of the abnormalities, teeth may not erupt normally and orthodontic treatment is usually required. Feeding my baby with cleft palate The most immediate concern for a baby with cleft palate is good nutrition. [urmc.rochester.edu]

  Dental problems As a result of the abnormalities, teeth may not erupt normally and orthodontic treatment is usually required. [chop.edu]

  Orthodontist --a dentist who evaluates the position and alignment of your child's teeth and coordinates a treatment plan with the surgeon and other specialists. Pediatric dentist --a dentist who evaluates and cares for your child's teeth. [stanfordchildrens.org]

• Dental Caries

  It is recommended that children at high risk for dental caries, including children with CL/P, receive at least twice-yearly fluoride varnish applications beginning with the first tooth eruption. [pediatrics.aappublications.org]

  Measurement of dental caries experience - modification of the DMFT index. Community Dent Oral Epidemiol. 1984 ; 12: 43 – 46. [doi.org]

• Mouth Breathing

  These signs may include nasal snorting, mouth breathing, and decreased appetite. Your child's doctor may prescribe medication to relieve the nasal congestion. Your child will be on antibiotics to prevent infection while in the hospital. [stanfordchildrens.org]

• Eruptions

  This phase of orthodontia includes treatment for atopic eruption of teeth and other potential complications that emerge as a result of bone grafting. [chop.edu]

  Teeth may even erupt into the palate or crowd each other. The mouth/palate area may be too short front to back, with an extremely narrow and asymmetrical upper jaw shape. [craniofacial.net]

  After teeth erupt, an orthodontist can further assess a child's short and long-term dental needs. After the permanent teeth erupt, orthodontic treatment can be applied to align the teeth. Prosthodontic care. [webmd.com]

  By cranial and dorsal insertion of the premaxilla were created satisfactory occlusal relationship for ongoing eruption of permanent teeth, together with functional lip closure, improved pronunciation and a positive change in appearance of the centrofacial [ncbi.nlm.nih.gov]

• Hearing Problem

  These disorders can result in feeding problems, speech problems, hearing problems, and frequent ear infections. Less than half the time the condition is associated with other disorders. [dbpedia.org]

  To prevent this from happening, children with cleft palate usually need special tubes placed in the eardrums to aid fluid drainage, and their hearing needs to be checked once a year. Speech problems. [webmd.com]

  These disorders can result in feeding problems, speech problems, hearing problems, and frequent ear infections. Less than half the time the condition is associated with other disorders. See more at Wikipedia.org... [translation.babylon-software.com]

  They may also have hearing problems, problems with their ears, or problems with their teeth. The major symptom is the presence of the cleft at birth. A cleft lip could be as small as a tiny notch in the upper lip. [familydoctor.org]

• Hearing Impairment

  impairment); to assess and monitor hearing A nurse coordinator to provide ongoing supervision of the child's health A social worker/psychologist to support the family and assess any adjustment problems A geneticist to help parents and adult patients [webmd.com]

  Cleft lip and cleft palate can present a number of challenges for a child, including possible: feeding issues dental and orthodontic problems speech and language difficulties hearing impairment social and self-esteem challenges Because cleft lip and cleft [childrenshospital.org]

• Normal Hearing

  […] to the auditory canal and Eustachian tube to allow for normal hearing Promoting as much as possible the normal growth and development of the upper jaw and teeth Repairing, when appropriate, any defects in the gumline to allow for permanent tooth eruption [plasticsurgery.org]

  These tubes are often placed at the time of cleft lip or palate repair, and may need to be replaced as necessary to preserve normal hearing. [chop.edu]

• Suggestibility

  In the antenatal and neonatal periods, the diagnosis is usually suggested by the presence of typical congenital abnormalities such as intrauterine growth retardation, microcephaly and radial ray defects. [ncbi.nlm.nih.gov]

  These results suggest that MSX1 and TGFB3 mutations make a contribution to clefts in South American populations. [emedicine.com]

• Language Delays

  Speech and language delays —Normal development of the lips and palate are essential for a child to properly form sounds and speak clearly. Cleft surgery repairs these structures; speech therapy helps with language development. [entnet.org]

  Speech and language delay. Due to the opening of the roof of the mouth and the lip, muscle function may be decreased, which can lead to a delay in speech or abnormal speech. [hopkinsmedicine.org]

  Additional Information & Resources: Tests & Screenings During Pregnancy Congenital Abnormalities Language Delays in Toddlers: Information for Parents American Cleft Palate-Craniofacial Association - Provides guidelines and standards for cleft-related [healthychildren.org]

  Infections that come back again and again can then lead to hearing loss Speech and language delay. Muscles involved with speech may not work well. This can lead to a delay in speech or odd speech. [childrensnational.org]

• Speech Disorder

  The main purpose was to test if parent led intervention is comparable to traditional speech therapy intervention in children with cleft palate speech disorders. [cleft.ie]

  Patients may have impaired facial growth, dental anomalies, and speech disorders (if a cleft palate is present), and they may experience late psychosocial difficulties. [emedicine.medscape.com]

  Unilateral Clefts Bilateral Clefts COMMUNICATION DISORDERS SPEECH DISORDERS Good place of articulation and intelligible speech in association with nasal emission and consonant weakness resulting from lack of intraoral breath pressure. [d.umn.edu]

  SLPs specialize in the evaluation, diagnosis, and treatment of communication disorders (speech disorders and language disorders), cognitive-communication disorders, voice disorders, and swallowing disorders. [en.wikipedia.org]

  […] infants with orofacial clefts, Journal of Pediatric Psychology, 2000, vol. 25 (pg. 185 - 190 ) * An investigation of reading and spelling performance in speech disordered children, British Journal of Disorders of Communication, 1982, vol. 17 (pg. 53 [academic.oup.com]

• Irritability

  Palatal prosthesis also prevent the tongue's irritation and inflammatory reactions, and it might be support the wound healing procedures. [doi.org]

  Though breast milk is not irritating to the mucous membranes, nasal regurgitation makes it more difficult to tell how much milk the baby is ingesting, and can make feedings more difficult. [americanpregnancy.org]

  After surgery for cleft lip: Your child may be irritable and feel mild pain. Your child may have to wear padded restraints on his elbows to prevent rubbing at the surgery site. Swelling, bruising and blood around stitch sites are normal. [chop.edu]

  Fecal transplant is used to treat gut infections and is now being studied as a treatment for obesity, urinary tract infections, irritable bowel syndrome and more. [nytimes.com]

• Nasal Speech

  During speech air escapes through the cleft into the nasal cavity, resulting in a characteristic nasal tone. [britannica.com]

  Additional surgery may be required on the roof of the mouth to help improve the mobility of the soft palate during speech and limit the escape of air into the nose creating nasal speech (staphylorrhaphy). [mycenters.com]

  Finally, patients needing prolonged speech therapy, especially those presenting with nasal speech and compensatory articulation, should alert speech pathologists about the possibility of CPO. [doi.org]

  […] resulting in nasal speech.  This occurs because the repaired soft palate is too short or does not move adequately. 108.  This condition is diagnosed primarily by the trained ear of speech pathologist.  However,special diagnostic procedures such as [slideshare.net]

During the evaluation of the patient, the medical team should perform a thorough physical examination of the lips, nose, palate, the throat including the uvula, as well as the head and neck. The team should also conduct a complete exam of the infant's body looking for findings indicative of other organ involvement such as cardiac and/or neurologic anomalies.

The medical team should also assess the infant's capacity to suck and breastfeed and monitor oral intake, weight, and growth.

Further testing will depend on the patient's clinical picture, physical findings, and family history. For example, if a trisomy is suspected, a chromosomal analysis is indicated. Additionally, infants with features suggestive of cardiac abnormalities warrant echocardiography and other appropriate studies [8].

Prenatal ultrasonography

Note that orofacial clefts can be detected by prenatal ultrasonography. If identified during pregnancy, the parents are offered genetic counseling and resources to prepare and coordinate care of infant following delivery.

Preoperative testing

A complete blood count is essential prior to any surgical procedure.

The therapeutic management of patients with a cleft lip is best provided by a thoroughly coordinated multidisciplinary team at specialized centers. Throughout the child's development, the comprehensive care should address speech, hearing, dental, and the psychosocial aspects tailored to the patient's needs.

Surgery

Cleft lip surgery is performed by experienced surgical specialists who employ advanced techniques.The goals of the surgery are to establish functional outcome and to improve the cosmetic appearance. The complicated procedures aim to repair the underlying orbicularis muscle and to construct an intact upper lip/nose and nearby structures that are size-appropriate and symmetric.

Surgical planning is challenged by important considerations such as the laterality and width of the cleft, presence of cleft palate, and whether the deformity is syndromic or not. Hence, the surgical repair is typically complex as the distorted anatomy features varying degrees of severity [9].

While the majority of surgeons perform the surgical procedure at 10 to 12 weeks old [10] [11].,the optimal timing of the surgery is debatable. The timing is decided by a number of factors such as the surgeon's preference, anesthetic risks, the presence of concurrent comorbid defects, and family's input. Additionally, surgeons may adhere to the rule of 10s. which refers to the following criteria 1) weight of infant reaches 10 lbs, 2) the hemoglobin is 10g/dL, and 3) the white blood cell count is below 10,000mm3. Another rule is to wait until the infant is older than 10 weeks of age.

Follow-up

Patients should undergo periodic follow-up for evaluation of speech, hearing, dental, as well as psychosocial assessment.

Other

There are various resources available to assist parents while caring for their child. The support resources provide education, networking, financial help, psychosocial therapy, etc.

Postsurgical prognosis

The patient outcome is determined by the contour and symmetry of the repaired lip as well as the overall facial growth.

Note that surgical revisions of the vermilion or scar and further nasal cosmetic procedures are common.

Special considerations

Very importantly, the psychosocial, speech, hearing and dental issues of the patient should be continually managed throughout childhood and possibly adulthood to maximize the individual's ability to live up to his/her potential.

Cleft lip can appear in isolation or as a component of a syndrome. Non-syndromic orofacial defects represent 70% of cases. With regards to non-syndromic cleft lip, the etiology is multifactorial with genetic, environmental and teratogenic interplay.

Genetic predisposition accounts for 40% to 60% of affected individuals as observed in studies investigating monozygotic twins. Moreover, researchers have implicated the interferon regulatory factor 6 (IRF6) gene in non-syndromic cleft lip and plate and vander Woude syndrome. The latter is the most common syndrome that features a cleft lip. Other genes have also been identified and these include VAX1, FGFR2, and BMP4.

Risk factors

There are numerous risk factors such as maternal smoking, pregestational diabetes, advanced maternal age (greater than 40 years of age), and possibly maternal infection. Additionally, zinc deficiency may be a risk as well.

Protective effects may include folate use during pregnancy.

The list of potential teratogens includes antiepileptic drugs such as valproic acid and phenytoin, and the acne medication retinoic acid.

The incidence of cleft lip is highest in Asians and Native Americans, which is 2 in 1000 live births, whereas it is 1 to 1.5 in 1000 live births in Caucasians. This defect is much lower in African and African-Americans with a value of less than 0.5 per 1000 live births [4].

There is a predilection for males [5].

The probability for parents with no family history to produce an offspring with a cleft is approximately 0.1%. The majority of children with clefts do not have affected family members. However, a positive family history is associated with an increased likelihood of developing the defect. Specifically, the risk for two normal parents without a cleft who have one offspring with a cleft lip/palate exhibit a 5% probability of recurrence.

During embryogenesis, the lip develops between weeks 4 and 7. In the fetus with a cleft lip, there is a disruption of the normal processes that form the lip. Specifically, there is a failure of the mesodermal penetration of the frontonasal and maxillary processes and the lack of fusion between the tissues of the lip. Therefore, the result is the malalignment of the orbicularis oris muscles.

A cleft lip may develop as microform, incomplete, or complete. Microform is the milder type, which is characterized by a vertical groove, vermilion notch, and lip shortening. An incomplete cleft lip displays a range of lip disruption but the nasal still is intact whereas a complete cleft exhibits an interruption of the lip, alveolus as well as the nasal still.

Orofacial defects likely result from a combination of genetic and environmental factors as well maternal exposures. For example, maternal smoking, pregestational diabetes, and the use of medications such as phenytoin, valproic acid, and retinoic acid all increase the risk of developing clefts. Hence, women contemplating pregnancy (or if already pregnant) should address the following issues (if applicable):

• Cease smoking
• Improve and control diabetes
• Discontinue use of retinoic acid
• Discuss with the healthcare provider the possibility of switching antiepileptic drugs to safer ones (if possible)

While not definitively proven, zinc and folate deficiencies may be associated with these defects. Hence, women are advised to take a daily multivitamin.

A cleft lip is a congenital craniofacial defect that affects the lip and nose, and exhibits a deformity of the nostrils and nasal sill [1] [2] [3]. This condition is the result of the lack of proper fusion between the tissues of the lip. While the exact etiology is unknown, this condition is likely multifactorial in origin with genetic, environmental, and maternal exposure interplay. Furthermore, there are risk factors associated with cleft lip such as maternal smoking, pregestational diabetes, and certain teratogenic medications.

There is a wide spectrum of clinical presentations for cleft lip. For example, a cleft lip may occur unilaterally or bilaterally, and may form with a cleft palate. Additionally, a cleft lip can either develop in isolation or manifest as a feature of a syndrome. In the latter, other anomalies may coexist such as those of the heart. Furthermore, there are numerous complications that arise from craniofacial deformities such as speech, hearing, dental, and psychosocial issues.

A cleft lip can be identified during prenatal ultrasonography and confirmed on physical exam of the neonate. The exam should include a full assessment of the lips, nose, throat, head, neck, and of course the patient's full body. The medical team should be careful not to miss any associated findings.

The treatment of the patient warrants a comprehensive multitherapeutic approach which includes coordinated care from skilled professionals and specialists. Early, prompt, and long-term care is essential to achieve good outcomes. A major component of treatment is surgical repair of the cleft lip and nose to restore function and anatomy of these structures. Moreover, these patients require management from all aspects as well.

What is a cleft lip?

A cleft lip is a common birth defect in which the lip does not form correctly during pregnancy. Normally, while the lips of the baby are developing during the 4th and 7th weeks of pregnancy, the tissue on each side of the face connect to each other to form the lips and mouth. In babies with a cleft lip, the tissues in the upper lip do not link to each other. Therefore, there is a gap in upper the lip.

The cleft lip can exist as a condition alone without other issues or it may be part of a syndrome that includes other birth defects.

What causes this condition?

The exact cause of this not known. However, there are risk factors such as genetics, environmental, and maternal exposures. The following are risk factors:

• Maternal smoking
• Diabetes prior to pregnancy
• Use of antiseizure medications such as phenytoin or valproic acid
• Use of acne medications such as retinoic acid

What are signs and symptoms of a cleft lip?

Cleft lip can be part of different presentations. For example, it may appear as:

• Left-sided (most common), right-sided, or on both sides
• With a cleft palate
• Without a cleft palate

If part of a syndrome, the patient may have heart and/or neurologic defects.

Clinical signs include:

• Difficulty with sucking and breastfeeding

Complications include:

• Trouble with speech
• Trouble with hearing
• Dental deformities
• Developmental and social delays
• Low self-esteem

How is it diagnosed?

Cleft lip can be diagnosed on prenatal ultrasound and confirmed after birth. During the physical exam of the newborn, the clinician should perform a complete exam of the lips, palate (roof of the mouth), throat, head, and neck. Also, the clinician should examine the baby's whole body looking for abnormalities of the heart, nervous system, and other organs/systems.

How is it treated?

The medical team that cares for infants with cleft lips should include a multidisciplinary approach with surgeons, otolaryngologists, speech therapists, psychologists, dentists, and social workers.

The main treatment is surgical repair of the lip, nose, and other related structures. The surgical procedures are complicated as the skilled surgeon performs specialized techniques to reconstruct the muscle beneath the lip and to restore its function. Also, the surgeon improves the overall cosmetic appearance. It is common for patients to have additional surgeries later to revise nose symmetry and scars.

What is the prognosis?

In order to ensure that the child lives up to his/her fullest potential, care must be provided early and comprehensively in terms of all aspects: speech, hearing, dental, psychological, etc.

Are there special considerations?

There are plenty of resources available to assist and support parents while caring for their child. Parents should also be reassured that this is not their fault.

Note that while feedings may be challenging and difficult, there are special bottles and nipples for the babies.

Can this be prevented?

Since smoking and pre-pregnancy diabetes are risk factors, women who are planning to get pregnant or already pregnant should quit smoking and control their diabetes. Also, women taking medications associated with cleft lip/palate development should speak to their physician about discontinuing them and switching to safer ones. Additionally, women should take a multivitamin daily including folate.

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2. Henry C, Samson T, Mackay D. Evidence-based medicine: The cleft lip nasal deformity. Plastic Reconstrive Surgery. 2014;133(5):1276-88.
3. James JN, Costello BJ, Ruiz RL. Management of cleft lip and palate and cleft orthognathic considerations. Oral Maxillofacial Surgery Clinics of North America. 2014;26(4):565-72.
4. Kosowski TR, Weathers WM, Wolfswinkel EM, et al. Cleft Palate. Seminars in Plastic Surgery. 2012;26(4):164-169.
5. Michalski AM1, Richardson SD, Browne ML, et al. American Journal of Medical Genetics, Part A. 2015;167A(5):1071-81.
6. Skuladottir H, Sivertsen A, Assmus J, et al. Hearing outcomes in patients with cleft lip/palate. Cleft Palate. Cleft Palate-Craniofacial Journal. 2015;52(2):e23-31.
7. Fitzsimons DA. International confederation for cleft lip and palate and related craniofacial anomalies task force report: speech assessment. Cleft Palate Craniofacial Journal. 2014;51(6):e138-45.
8. Hartzell LD, Kilpatrick LA. Diagnosis and management of patients with clefts: a comprehensive and interdisciplinary approach. Otolaryngologic Clinics of North America. 2014;47(5):821-52.
9. Niranjane PP, Kamble RH, Diagavane SP, et al. Current status of presurgical infant orthopaedic treatment for cleft lip and palate patients: A critical review. Indian Journal of Plastic Surgery. 2014;47(3):293-302.
10. Shaye D. Update on outcomes research for cleft lip and palate. Current Opinion in Otolaryngology & Head and Neck Surgery. 2014;22(4):255-9.
11. Miachon MD, Leme PL. Surgical treatment of cleft lip. Revista do Colégio Brasileiro de Cirurgiões. 2014;41(3):208-14.