What is Cleft Palate?

Cleft lip and palate are among the most frequent birth disorders affecting the face. This condition occurs when the lip and roof of the mouth, also known as the palate, don’t merge properly during the baby’s development, leading to a visible defect. Apart from being noticeable physical irregularities, cleft lip and palate can also cause significant functional problems if not managed properly.

For a newborn with this condition, feeding can become challenging due to increased nose reflux, the inability to latch onto a feeding bottle properly, and tiring easily because feeding requires more effort. Even more, cleft lip and palate are often symptoms of congenital syndromes that need to be identified early for effective care and management.

Therefore, managing these conditions effectively requires the dedicated efforts of a holistic healthcare team, including medical professionals and social support services.

What Causes Cleft Palate?

Cleft palate happens when there is a problem with development in the womb. Starting around the fourth week of pregnancy, the baby’s mouth begins to form. The face grows in different sections: one section in the center and two sections on each side. In the following week, the two sections on either side begin to grow towards the center section, eventually forming the areas around the nose and lips. If these sections fail to connect at the end of the fifth week, the result is a split, or cleft, lip.

Meanwhile, the palate, or roof of the mouth, begins development and typically completes formation around the twelfth week of pregnancy. If there is a failure to properly join down the center of this area, this results in a cleft palate.

Clefts are noted in over 200 different conditions present at birth. The most commonly associated are CHARGE syndrome and DiGeorge syndrome. Cleft palate alone often appears alongside other abnormalities, more so than when a cleft palate and cleft lip appear together. Quite frequently, cleft palates are seen as part of the Pierre Robin sequence, which includes other features like a small lower jaw and cleft palate, usually seen in conditions like Stickler syndrome and fetal alcohol syndrome.

There are a number of biological processes that can contribute to a cleft palate or a cleft lip and palate together. Different genes aid in the development of the lip and palate, and when disrupted, can lead to clefts of varying degree and at different locations.

Moreover, environmental factors can also affect the development of clefts. Risk factors include smoking, diabetes during pregnancy, and exposure to certain substances during pregnancy like certain anti-seizure drugs, Vitamin A derivatives, and some toxins. The chance of clefts appearing in a child increases if a parent or brother or sister also has a cleft. If a parent has a cleft, their child has a 3-4% chance for cleft lip or palate and around 6% chance for cleft palate alone. This risk increases if more than one sibling is affected.

Risk Factors and Frequency for Cleft Palate

CL/P, a craniofacial defect, is the most common kind amongst newborns and is the fourth most common congenital (meaning, present from birth) anomaly. Studies show that these defects may occur in different ranges, from every 1 in 1,000 to every 1 in 650 live births. This condition is seen to be twice as common in Asians compared to Whites, with males being affected twice as much as females. The National Institute for Dental and Craniofacial Research conducted a worldwide study that included over 7.5 million births and found that CL/P affects about 6.64 per 10,000 births (including stillbirths and terminations). The prevalence of cleft palate alone was 3.28 per 10,000 in these observations. It’s observed that 77% of these were isolated cases, 16% had other malformations, and 7% were identified with a recognized syndrome.

Signs and Symptoms of Cleft Palate

Prenatal diagnosis of cleft lip or cleft palate is typically made around the 18th week of pregnancy using an ultrasound. However, the accuracy of this test largely depends on the skill and experience of the ultrasound technician. Ordinary 2D ultrasounds are better at detecting cleft lip than cleft palate. But the advent of 3D ultrasound technology has greatly improved the detection rates for cleft palate, yielding a 100% success rate in fetuses already diagnosed with a cleft lip. Further details of the condition can be obtained using prenatal MRI scanning, primarily when a cleft lip and/or palate have been spotted. This procedure also checks for other defects, especially those related to the brain. Early identification of these conditions helps in planning for the right treatments and interventions as soon as possible.

After birth, a cleft palate can be easily identified by examining the newborn. Signs to look out for include breathing difficulties and spit-up during feeding, and longer feeding times due to fatigue. Most babies with this condition also struggle to latch on properly during breastfeeding. During a physical exam, the doctor will look for a visible defect in the roof of the mouth. In cases where the cleft is covered by the mucous membrane (submucosal cleft), the doctor will feel for an indentation along the middle of the hard palate.

Testing for Cleft Palate

Once a baby with cleft lip and/or palate (CL/P) is born, they’ll start working with a health care team that includes many professionals. These professionals may include a pediatrician or primary doctor, speech therapist, genetics counselor, ear, nose and throat doctor (otolaryngologist), audiologist, social worker, orthodontist, and craniofacial surgeon.

The first focus for these professionals is ensuring the baby can breathe and eat properly, which is important for the baby’s growth and development prior to surgery. They’ll check if the baby is having any trouble breathing during sleep (sleep apnea) and provide guidance to parents on how to manage this. A speech therapist, in particular, will teach parents special feeding techniques and provide tools that make feeding easier, help the baby eat enough, and prevent exhaustion during feedings. Special feeding bottles and nipples are often used to prevent food from going into the nasal cavity and causing breathing difficulties. Some babies may also need a dental evaluation to see if oral prosthetics could aid in feeding before the surgery. It’s extremely important for the baby to gain weight to ensure healthy development and prevent any delay in surgery.

As the baby grows, they’ll require ongoing check-ups, including:

  • Hearing tests and treatment for any hearing problems
  • Management of a recurring issue known as middle ear effusion by an otolaryngologist, they’ll also assess the growing child for sleep apnea and determine if surgeries are needed to help with breathing
  • Speech therapy sessions to track feeding and language development
  • Genetic counseling to help family plan for the future
  • Dental check-ups and treatments as soon as the child’s teeth start to appear
  • Mental health counseling for both the parents and child

Treatment Options for Cleft Palate

The main treatment for cleft palate, a condition where a baby’s upper lip or roof of the mouth doesn’t form properly during pregnancy, is surgery. The process generally involves several stages, usually starting with the upper lip, then moving to the roof of the mouth. This first step often takes place when the baby is around 10 weeks old, weighs at least 10 pounds, and has a hemoglobin (a protein in red blood cells that carries oxygen) level of 10.

There are a variety of methods for repairing a cleft lip or palate. Two common techniques for the lip are called the Millard rotation-advancement and the Mulliken, depending on whether the cleft is on one or both sides of the lip. For the palate, surgery usually happens later, when the child is between 9 to 15 months old. Several procedures can be used, including the straight line method, the Furlow double Z-plasty, and Veau-Wardill-Kilner V-Y pushback.

Before surgery, the lip is usually taped starting about a week after birth to reduce the size of the cleft and improve symmetry. An alternative method that can result in even better results after surgery is nasoalveolar molding (NAM), where a custom prosthesis is fitted to the baby’s jaw. Worn 24/7 and adjusted regularly, NAM can significantly improve nasal symmetry and alignment of the jaw. However, it demands a considerable commitment of time and effort from the family.

In cases where the cleft is very wide, an alternative surgical procedure called lip adhesion could be used to make the cleft narrower before the main repair. This treatment, usually done when the baby is 4 to 6 weeks old, involves lifting flaps of tissue from the lip, which are then stitched together. However, it’s not commonly used since it involves an additional surgical procedure.

Regarding the palate surgery techniques, the most important step is the reconstruction of a crucial muscle that helps to lift the palate during swallowing. Failure to properly reattach this muscle can result in issues like food or liquid coming back up through the nose during swallowing and a nasal-sounding speech.

One of the techniques, two-flap palatoplasty or straight-line repair, involves lifting tissue from either side of the cleft and stitching it in the middle to recreate the muscle. The second technique, the Furlow double Z-plasty, uses a Z-shaped arrangement of flaps to remake the muscle and the uvula (the dangly bit at the back of the mouth). This technique can also extend the palate if it’s too short. The third method, the Veau-Wardill-Kilner V-Y pushback involves lifting flaps of tissue in a way that allows the palate to be lengthened.

Determining if a baby has a cleft palate is usually easy. However, it’s essential to check whether the cleft palate is part of a bigger syndrome. Some of these syndromes could include:

  • CHARGE syndrome
  • Stickler syndrome
  • 22q11 deletion syndromes
  • Pierre Robin sequence
  • Various chromosomal deletions or duplications

In these cases, the cleft palate is just one of many symptoms or characteristics that a child may have.

What to expect with Cleft Palate

From the perspective of a cleft palate, the outlook is very positive when it’s surgically repaired. Any difficulties with feeding are usually resolved when the cleft is closed. Any long-term effects due to scarring from the surgery are dealt with through additional surgeries to revise the scars and revise the palate repair. These additional procedures also typically have good results.

Possible Complications When Diagnosed with Cleft Palate

Treatment for a cleft lip, like taping and nasoalveolar molding (NAM), sometimes lead to skin irritation or other skin issues because of the adhesives used. If a lip adhesion is used, there can be excessive scarring which might not look good cosmetically and could make the final repair more difficult. These procedures are typically performed quite early, when the baby is between 4-6 weeks old, and problems can occur such as the wound reopening or formation of a fistula – a unwanted hole in the lip or nose.

Palatoplasty, a surgery to correct a cleft palate, can also have complications. The wound can reopen and could cause a fistula. If the surgeon does not correctly realize the palate is too short, or doesn’t adequately fix the levator veli palatini muscle (this helps lift the palate), it can cause velopharyngeal insufficiency (VPI). With VPI, the patient isn’t able to lift the palate enough to keep food or drink from going into the nose, and it can also cause speech to sound very nasal.

Common Complications:

  • Skin irritation or breakdown due to adhesives
  • Excessive scarring from lip adhesion
  • Wound reopening post-surgery (wound dehiscence)
  • Nasolabial fistula development
  • Short palate recognition inadequacy
  • Failure to properly adjust the levator veli palatini muscle
  • Velopharyngeal insufficiency (VPI)

Preventing Cleft Palate

It is crucial for families who already have a child with a cleft palate to understand the greater likelihood of the condition occurring in future children. Parents should seek advice from genetic services for further examination and guidance.

Preventing such conditions largely relies on suitable pregnancy and childbirth care provided by an obstetrician. This helps reduce or eliminate exposure to harmful substances and environmental factors during pregnancy, which can increase the risk of developmental problems in the unborn child.

Frequently asked questions

Cleft palate is a condition where the roof of the mouth does not merge properly during a baby's development, resulting in a visible defect.

Cleft palate affects about 3.28 per 10,000 births.

Signs and symptoms of Cleft Palate include: - Breathing difficulties and spit-up during feeding: Babies with a cleft palate may have trouble swallowing and may experience difficulty breathing while feeding. This can lead to spit-up or regurgitation of milk or formula. - Longer feeding times due to fatigue: Babies with a cleft palate may tire easily while feeding due to the extra effort required to suck and swallow. This can result in longer feeding times and frequent breaks during feeding. - Difficulty latching on during breastfeeding: The structural abnormality of the cleft palate can make it challenging for babies to create a proper seal and latch on to the breast during breastfeeding. This can lead to inadequate milk intake and poor weight gain. - Visible defect in the roof of the mouth: A cleft palate can be visually identified by examining the newborn. The doctor will look for a gap or opening in the roof of the mouth, which may extend from the front of the mouth to the back. - Indentation along the middle of the hard palate (submucosal cleft): In cases where the cleft is covered by the mucous membrane, the doctor will feel for an indentation along the middle of the hard palate. This can help diagnose a submucosal cleft, where the cleft is not visible but can still cause feeding and speech difficulties. It is important to note that these signs and symptoms may vary in severity depending on the size and location of the cleft palate. Early identification and intervention are crucial for managing the condition and ensuring proper feeding and speech development.

Cleft palate occurs due to a failure in the proper joining of the sections of the palate during development in the womb. This can be caused by genetic factors or environmental factors such as smoking, diabetes during pregnancy, and exposure to certain substances.

The doctor needs to rule out the following conditions when diagnosing Cleft Palate: - CHARGE syndrome - Stickler syndrome - 22q11 deletion syndromes - Pierre Robin sequence - Various chromosomal deletions or duplications

The types of tests that may be needed for Cleft Palate include: - Hearing tests to assess for any hearing problems - Evaluation and management of middle ear effusion by an otolaryngologist - Speech therapy sessions to track feeding and language development - Dental check-ups and treatments as soon as the child's teeth start to appear - Genetic counseling to help with family planning - Mental health counseling for both the parents and child.

The main treatment for cleft palate is surgery. The process typically involves multiple stages, starting with the upper lip and then moving to the roof of the mouth. The first step is usually performed when the baby is around 10 weeks old, weighs at least 10 pounds, and has a hemoglobin level of 10. There are various surgical techniques for repairing a cleft lip or palate, including the Millard rotation-advancement, Mulliken, straight line method, Furlow double Z-plasty, and Veau-Wardill-Kilner V-Y pushback. Before surgery, the lip may be taped or nasoalveolar molding (NAM) may be used to reduce the size of the cleft and improve symmetry. In some cases, lip adhesion may be done to make the cleft narrower before the main repair. The reconstruction of a crucial muscle during palate surgery is important to prevent issues like nasal-sounding speech and food or liquid coming back up through the nose during swallowing. Techniques such as two-flap palatoplasty, Furlow double Z-plasty, and Veau-Wardill-Kilner V-Y pushback are used to recreate the muscle and extend the palate if necessary.

The side effects when treating Cleft Palate can include: - Skin irritation or breakdown due to adhesives used in taping and nasoalveolar molding (NAM) - Excessive scarring from lip adhesion - Wound reopening post-surgery (wound dehiscence) - Development of a nasolabial fistula - Inadequate recognition of a short palate - Failure to properly adjust the levator veli palatini muscle - Velopharyngeal insufficiency (VPI), which can cause nasal-sounding speech and difficulty with swallowing.

The prognosis for individuals with a cleft palate who undergo surgical repair is generally positive. Feeding problems associated with the cleft palate are typically resolved after the cleft is closed surgically. Long-term effects related to scarring can be managed with additional surgery to revise the scars and the palate, which tend to have positive outcomes.

An otolaryngologist.

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