Cleft palate and cleft lip and palate are multi-factorial conditions very commonly encountered. This activity illustrates cleft palate repair and highlights the healthcare team's role in evaluating and managing this condition to positively impact patients who undergo such a repair. The cleft palate repair objectives are to reconstruct the palatal muscles, provide the foundation for adequate palatal movement and good speech, isolate the oral cavity from the nasal cavity, and repair the palatal defect. A thorough clinical exam, a piece of solid anatomical knowledge, and a pristine surgical technique will reduce commonly encountered complications.
Describe the embryological considerations that affect the relevant anatomy resulting in cleft palate.
Outline the typical presentation of an overt cleft palate and a submucous cleft palate.
Summarize the surgical techniques available to address cleft palate.
Review some interprofessional strategies that can improve patient outcomes in cases undergoing surgical repairs for cleft palate.
Cleft palate requires a basic level of understanding of embryology and anatomy. It is critical to know that every patient is different, and every cleft palate may show diverse variants. The healthcare team should perform a thorough clinical assessment of the oral cavity and the external and middle ear and individualize treatment so that potential complications are minimized and possible associated conditions prevented.
Anatomy and Physiology
Embryologically, cleft palate happens while most craniofacial structures form between the 4 to 8 weeks of gestation. A primary cleft palate occurs due to a failure of fusion of the lateral palatine processes and the median palatine process. A secondary cleft palate is due to a loss of fusion of the lateral palatine processes and the nasal septum.
Anatomically, the palate is divided into a primary palate and a secondary palate by the foramen incisivum or incisive foramen (also called anterior palatine foramen or nasopalatine foramen). The incisive foramen's location is in the midline, osseous part of the oral hard palate, immediately posterior to the central incisor teeth. It corresponds to the medial palatine and incisive sutures' junction and divides the primary palate (located anterior to the incisive foramen) from the secondary palate (structures situated posterior to the incisive foramen). Thus, in patients with cleft palate, the defect may involve the soft palate and part of the hard palate or the hard palate's entirety. In other words, cleft may demonstrate the involvement of the primary or the primary and the secondary palates, and can be unilateral or bilateral (Figure 1 -3).
The pterygoid hamulus represents another essential anatomical structure. It is part of the sphenoid bone and the superior origin of the pterygomandibular raphe. In individuals without cleft palate, the tensor veli palatini muscle fibers glide around it like a hammock.
Patients with cleft palate demonstrate an abnormal musculature, as depicted originally by Fergusson and Veau. The tensor veli palatini muscle is partially attached to the palatal shelf's posterior border (hard palate) laterally. The levator palatini muscle demonstrates an abnormal insertion at the cleft margins in the anterior half to two-thirds of the velum. As described below, careful dissection, release, and reconstruction of the palatal muscles will determine to a great extent the adequate movement of the soft palate postoperatively.
The blood supply to the soft and hard palates provides from the descending palatine artery, as it emerges from the greater palatine foramen. Thus, it is vital to perform dissection carefully to preserve this blood vessel. Inadvertent injury to this vessel may demonstrate hemipalatal necrosis, particularly when the palatal flaps are monopedicled.
Some patients may present with a submucous cleft palate characterized by nasal/abnormal speech and velopharyngeal insufficiency. The physical exam may demonstrate a bifid uvula, a zona pellucida, and a palpable notch on the hard palate. Depending on the patient's age and severity of the cleft, management may include speech therapy. However, most patients with a submucous cleft palate require a velopharyngoplasty, followed by speech therapy.
Functionally, the palate soft moves cephalically and posteriorly to close the velopharyngeal port when pronouncing syllables that contain "p," "k," "s." The levator palatini muscle is in charge of this movement, thus the importance of carefully reorienting its muscle fibers during the cleft palate repair.
Histologically, when the palatal epithelium forms in utero, the nasal cavity epithelium will differentiate into the columnar ciliated epithelium between the sixth and the twelfth weeks. On the other hand, the epithelium covering the palate's oral cavity side will differentiate into the stratified squamous epithelium.
While comorbidities may postpone cleft palate repair, an infant born with a cleft palate should receive a cleft palate repair.
There are no absolute contraindications for cleft palate repair. Relative contraindications include any medical conditions that preclude the use of general anesthesia (cardiac conditions, severe illness, etc.). There are patients whose airway conditions or syndromes may need to be addressed before the palatoplasty, such as patients with retrognathia, Pierre Robin sequence, etc.
Cleft palate repair kits should include the following:
A suction cannula (e.g., Frazier 7 Fr)
Mouth retractor (e.g., Dingman, Fisher, etc.) with blades of different sizes.
Long forceps (e.g., Gerald toothed, non-toothed forceps)
Long scissors (e.g., Metzembaum long scissors)
Long needle holder (e.g., Rider)
Electrosurgical knife (electrocautery)
Diathermy with long Colorado tip
Bipolar forceps with a long tip
Surgical loupes are recommended for cleft palate repair.
However, the utilization of an operative microscope (suggested for performing specific palatoplasty techniques is not compulsory).
For cleft palate repair:
A reconstructive surgeon with expertise in cleft palate repair
A surgical assistant
An anesthesiologist, preferably a pediatric anesthesiologist
A nurse circulator
A technician or nurse to aid in passing the instruments
Preoperative Information and Parent Preparation
During prenatal consultations, parents' common concern is to know the recurrence risk for cleft lip and cleft lip and palate. For parents who have no history of cleft lip and palate and who have had one child with a cleft lip and palate, the chances of having a second child with a cleft are approximately 4%. When parents have had two children with cleft lip and palate, the percentage increases to about 9%. Whereas, if one parent has had cleft lip and palate and one sibling has a cleft, the risk rises to approximately 17%. Recurrence risk does increment in cases of severe clefts.
Genetically, while most cleft palate patients may present with a nonsyndromic condition, multiple syndromes are associated with cleft palate. Some syndromes are linked to a single gene alteration (monogenic syndromes), and others can be due to numerous gene abnormalities. Among the monogenic syndromes, velocardiofacial syndrome (Shprintzen syndrome) is an autosomal dominant condition linked to deletion of chromosome 22q11.2. It is imperative to perform imaging studies to verify the internal carotid artery's location in patients with suspected velocardiofacial syndrome. The reason is that the internal carotid artery may be displaced and localized more medially. Should a posterior pharyngeal flap be performed, injury to the internal carotid artery could happen with potentially fatal consequences.
The patient's parents or guardians need to be explained thoroughly about the matters involved with a cleft palate repair.
It is essential to discuss the need to perform the surgical intervention under general anesthesia. Parents or guardians need to be informed about the potential risks and complications, including bleeding (with the potential requirement of blood product administration), infection, dehiscence, need for reoperation, need to keep the baby intubated after surgery (with postoperative monitoring at the pediatric ICU) and death.
The patient needs to be healthy for the procedure, without any active infections, and have a hemoglobin level of ≥ 10 mg/ dL. Confirmation of a nil per os status before the intervention is mandatory.
Cleft palate patients commonly develop otitis media with effusion in the first two years of life (with incidence rates of ≥ 90 %). The development of otitis media has significant derangements on speech, language, and potential emotional and intellectual disability. Therefore, the infant with cleft palate should undergo a newborn hearing screen after birth and tympanometry and otoscopy before the cleft palate repair. The tympanometry will help determine if the patient has any abnormalities suggestive of middle ear disease. If the study demonstrates any finding suggestive of middle ear disease, the otorhinolaryngologist can place pressure-equalizing tubes [PET] during the same anesthetic event for the cleft palate repair intervention. By doing both procedures (placement of pressure equalizing tubes and cleft palate repair) under one anesthetic event, the patient has less exposure to anesthesia and its associated risks.
Cleft palate repair is performed with the infant under general anesthesia in the supine position. Suppose the infant has no contraindication to neck movement. In that case, placement of a shoulder roll can assist in hyperextending the neck, which will help attain a good head position to open the mouth to visualize the oral cavity properly. A mouth retractor (e.g., Dingman mouth retractor) will be employed to maintain the oral cavity open throughout the procedure (Figure 3). It is essential to ascertain that the lips and tongue are adequately positioned and lubricated during the surgery to prevent unintentional injuries or substantial tongue swelling.
Operative Techniques for Palatoplasty
There are multiple surgical techniques described for cleft palate repair. However, this article will focus on the more frequently utilized interventions.
Incisions are made on the soft palate, posterior to the maxillary tuberosity (termed "relaxing incisions"), followed by careful blunt dissection in a plane between the superior constrictor muscle and the velar musculature.
An incision is performed along the cleft soft palate's medial margin, from the cleft's mid-portion and proceeding posteriorly towards the uvula.
Dissection of the nasal mucosa from the muscle is carried, followed by approximation to the contralateral nasal mucosa with simple interrupted sutures.
Approximation of both hemiuvulae using interrupted horizontal mattress sutures is carried.
An incision is made along the medial margin of the cleft hard palate, followed by an incision over the hard palate's lateral margins. The bipedicle mucoperiosteal flaps laterally are dissected (the anterior blood supply is provided by the periosteal attachments anteriorly; the posterior blood supply is given by the greater palatine arteries, posteriorly). Approximation of the mucoperiosteal flaps with suture follows.
Intravelar veloplasty is performed (as described in the following section). Although not described in the original technique, most cleft surgeons perform this procedure while doing the Von Langenbeck palatoplasty.
Oral mucosa approximation is made with the horizontal mattress or simple interrupted sutures.
Also termed V-Y elongation or "pushback" palatoplasty
This technique represents a modification of the Von Langenbeck palatoplasty. This technique's principles are to create two mucoperiosteal flaps, one from the left and one from the right hard palate.
A V-shaped incision is made, followed by subperiosteal dissection. Then, the mucoperiosteal flaps are mobilized, from the hard palate to the soft palate, posteriorly. This maneuver is termed "pushback." The flaps are monopedicled. The greater palatine artery gives blood supply.
Closure proceeds in a V-Y fashion to try to provide elongation. However, the anterior palate repair consists of nasal mucosa closure exclusively. The nasal mucosa closure relies on unilateral or bilateral, superiorly based mucosal vomer flaps solely (one-layer closure).
Due to the high incidence of postoperative fistula formation and midfacial growth disturbance, most surgeons abandoned this surgical technique.
An incision is made along the medial margin of the cleft soft palate, at the junction between the nasal and oral mucosas. The incision extends to the tip of the uvula, dividing the uvula. An incision is performed along both sides of the hard palate to create mucoperiosteal flaps.
Dissection of the mucoperiosteal flaps proceeds on the oral side, at the subperiosteal plane. The greater palatine foramen is identified, preserving the neurovascular bundle. Lifting the neurovascular bundle may help to attain increased mobility of the flap. The nasal mucoperiosteum is dissected similarly.
Dissection of the palatal muscles off the hard palate's posterior edge and from the periosteum on the nasal side is performed. Then, the muscles are repositioned medially and distally—a pivotal step to recreate the palatal muscle sling and lengthen the soft palate.
Muscle repair is performed, starting distally from the uvula, and proceeding anteriorly towards the hard palate's posterior edge. While the original technique advocates using vertical mattress sutures (including the oral mucosa and muscle), placing a horizontal mattress or simple interrupted sutures taking the muscle only is an alternative.
Approximation of the palatal flaps along the hard palate follows.
The areas with exposed bare bone lateral to the mucoperiosteal flaps are assessed. Bardach advocated placing loose sutures to reduce the area of exposure. Certain cases may benefit from placing hemostatic agents or fibrin on the site of exposed bare bone.
Two z-plasties are marked over the hard and soft palates, denoting two flaps that are posteriorly based. The cleft margins represent the central limb of z-plasties designed in the opposite direction.
On the one side, the anteriorly based flap, with careful dissection of the palatal muscle from the nasal mucosa layer (leaving it attached to the oral mucosa layer). On the other side, a posteriorly based flap with careful dissection of the oral mucosa layer from the muscle (leaving it attached to the nasal mucosa layer). Closure proceeds by transposing the flaps with a horizontal mattress or simple interrupted sutures (Figures 1, 3).
Dr. Sommerlad advocates the use of the operating microscope to perform this technique. The microscope can undoubtedly provide better magnification and better quality images. However, using a microscope in patients with limited mouth opening (e.g., Pierre Robin sequence patients) or the lack of a microscope has made surgeons comfortable performing this technique under loupe magnification (3.5x or greater).
An incision is performed along the cleft soft palate's medial margin, usually making the incision slightly more on the oral side of the junction between the nasal and oral mucosas. The incision is extended to the posterior hard palate.
Dissection of posterior mucoperiosteal flaps follows with the use of a dental periosteal elevator, passing it carefully behind the greater palatine vessels and distal to the posterior edge of the hard palate.
The dissection of the oral mucosa investing the velum of the musculature proceeds.
Dissection of the palatal shelves' nasal mucosa and careful approximation in the midline follows. Once suture, dissection proceeds laterally, identifying the plane between the nasal mucosa and musculature.
Dissection of the muscle of the posterior edge of the palatal shelf and from the nasal mucosa is carried, followed by the division of the tensor veli palatini muscle medial to the hamulus.
After confirming the muscle's adequate mobilization, the muscle's union in both sides of the palate proceeds by using loop mattress sutures or interrupted sutures. A surgical approximation with suture of the oral layer follows.
Following a straight line incision over the soft palate, careful dissection of the muscle from the nasal mucosa and oral mucosa layers proceeds. Afterward, detachment of the muscle from the palatal shelf and realignment and suture of the levator palatine muscles transversely to form a muscular sling.
It aims to obtain an additional amount of mucoperiosteal tissue from the vomer to reconstruct the nasal mucosa layer of patients with unilateral cleft palate who demonstrate broad defects.
The procedure consists of a paramedian incision of the mucosa overlying the vomer followed by careful dissection of the tissues in the subperiosteal plane. Once free, the flap is mobilized and sutured to the nasal mucosa layer on the cleft side. While the vomer flap's initial descriptions were based on caudal or inferior blood supply, most of the currently used vomer flaps rely on the cephalic or superior blood supply.
A wide flap (approximately 3 cm in width) can be dissected from the posterior pharyngeal wall at the cricoid cartilage level. Once dissection is complete, ensuring hemostasis, the palatal defect reconstruction finalizes by mobilizing the flap cranially.
Complications of Palatoplasty
Postoperative airway obstruction due to tongue edema. When using an improperly sized mouth retractor or when the mouth gag exerts significant pressure, causing a reduction in the blood supply, the tongue may swell substantially and obstruct the oral airway. Since infant patients are lying supine, the tongue will fall posteriorly due to gravity and hit the posterior pharyngeal wall, obstructing the airway.
Prolonged intubation. Patients with multiple comorbidities, syndromic patients, or any patient whose transoperative behavior has a substantial decline may require to be kept intubated and transferred to the Pediatric ICU for postoperative care and treatment.
Like many other procedures involving the airway, a persistent spasm of the larynx may occur.
In some situations, while the patient progresses from general anesthesia or after being asleep or displays continuous crying, a substantial increase in blood pressure may cause the blood clots formed in the raw hard palatal or along the palatal edges' flaps to dislodge. The patient manifests sudden onset of progressive bleeding in moderate amounts. In most of these scenarios, gentle pressure for five minutes with a gloved finger holding a gauze will stop the bleeding. The bleeding may impact the oral cavity and airway, keeping the babies NPO until complete bleeding control is essential.
A partial or total separation of the previously approximated tissues can happen if there is a substantial amount of tension or the patient has trauma to the recently repaired palate.
Mediate (approximately ≥ two weeks after the cleft palate repair)
A fistula (communication between two epithelial surfaces) may happen due to increased tension in the repair, infection, or accidental trauma to a localized area. Fistulas may occur between the palate and the nasal cavity (palatal fistula), between the dental alveoli and the nasal cavity (nasoalveolar), or between the oral cavity and the nasal cavity (oronasal fistula). Oronasal fistulas usually present with the passage of fluids and solid foods from the oral cavity to the nasal cavity. The diagnosis of a fistula, regardless of its location, is clinical. The condition is confirmed by visualizing the communication when the patient opens his mouth or introducing a cotton tip applicator gently through the nostril and observing its passage into the oral cavity. The treatment of fistulas encompasses another surgery to isolate the oral and nasal cavities. There are several classifications of fistulas after cleft palate repair. Most of these document increased numbers at the hard palate and the hard-soft palate junction and immediately behind the alveolus/dental arch.
Partial dehiscence and bifid uvula. This complication may appear after a few weeks following the surgical repair and is often related to the uvula's improper technical closure. Since the uvula has a muscular and a mucosa layer, both layers' raw surfaces need to be carefully approximated to prevent this complication. Some fistula classification schemes consider this entity.
Inadequate palatal movement. As mentioned previously, the palate moves cephalically and posteriorly to close the velopharyngeal port. Improper movement may be due to improper management of tissues, excess scarring, or idiopathic abnormal mobility.
Infection is a complication that usually manifests as a fistula formed at a localized area of the palate repair. However, it may present as an overt infection, with redness, warmth, purulent exudate, and pain.
A palatal flap's necrosis is a devastating complication due to accidental injury to the greater palatine artery and may occur in a delayed fashion.
Surgical Aspects Encompassed in Cleft Palate Repair
The objectives of cleft palate repair are:
Reconstruction of the palatal muscles to provide the foundation for adequate palatal movement and good speech.
Isolate the oral cavity from the nasal cavity.
Repair the palatal defect.
One of the surgical principles in reconstructing the cleft palate is to “borrow from Peter to pay Paul.” In other words, to dissect and mobilize the tissues from the hard and soft palates' lateral aspect and reposition them and suture them precisely in the midline. The donor areas on the hard and soft palates' lateral aspect will re-mucosalize in 24 to 48 hours.
Another important surgical principle is to dissect, reposition and suture the relevant palatal muscles according to the normal palatal muscle configuration. This is because some of the palatal muscles demonstrate an abnormal attachment to the palatal shelves' posterior aspect. Thus, the repair should encompass detachment of the abnormal insertions and proper repositioning and repair.
Lastly, the most important technical aspect to keep in mind is to avoid tension in the repair. Tension is the main contributor to partial or total dehiscence and fistula formation.
Independent of the surgical technique selected for cleft palate repair, it is imperative to thoroughly irrigate the nose, mouth, and pharynx with betadine or other antiseptic solutions before the intervention. Afterward, infiltration with an anesthetic (xylocaine or lignocaine) with adrenaline and allowing approximately 7 minutes for its action to be complete should follow.
Enhancing Healthcare Team Outcomes
Palatoplasty is a significant procedure and may be challenging and complex in certain clefts due to increased width or bilateral defects. To derive good outcomes, cleft palate repair objectives must be defined before taking the patient to surgery. The preoperative workup must be efficient and include a thorough oral cavity exam by the reconstructive surgeon and a middle ear assessment by the ear nose and throat specialist.
Evidence-based guidelines regarding the best approach for cleft palate repair are not available because of the heterogeneity of cleft palate types, the heterogeneity in technique, types of sutures used, utilization of surgical microscope/surgical loupes, lack of long-term follow-up, and patient characteristics (non-syndromic vs. syndromic, etc.).
In the postoperative period, nurses' role in the recovery unit is critical. The nurses will assist the team by monitoring the patient for airway obstruction, bleeding, and pain. Some patients may display a postoperative course with a deficient oral intake, thus, further emphasizing the need for an interprofessional approach (reconstructive surgeon/pediatrician/nursing personnel) to manage fluids to prevent dehydration and guide parents through the process. In patients with multiple comorbidities or syndromic cases, the need for meticulous planning and discussion with other professionals involved in managing the patient is highly recommended to lower the morbidity and improve outcomes.
Nursing, Allied Health, and Interprofessional Team Interventions
Preventative Measures for Potential Postoperative Airway Obstruction
Airway obstruction may happen in patients who demonstrate significant tongue edema (due to decreased perfusion secondary to the pressure exerted by the mouth retractor) or those who display a small airway followed by the soft palate's remarkable swelling. Two interventions for this feared complication are tongue stitch and placement of a nasopharyngeal airway ("nasal trumpet"). The former consists of placing a suture on the midline (to prevent unintentional injury to the lingual veins) of the junction of the tongue's anterior and middle thirds. In case the patient demonstrates postoperative airway obstruction, pulling the suture can increase the space in the oropharynx. The latter procedure consists of the insertion of a nasopharyngeal airway, which acts as a bridge or passage for air through the nasal cavity and avoiding swelling in the oral cavity. There is a discussion about whether these two interventions work.
As the patient becomes more alert, following general anesthesia, he/she will start opening the eyes and becoming more active. In case the patient wakes up suddenly, a dramatic increase in the blood pressure may cause bleeding from the periosteum due to dislodgement of blood clots adhered to the dissected bone (raw bone). If this situation happens, it is imperative to provide gentle, direct pressure with a gloved finger holding a gauze for a minimum of 5 minutes. Direct pressure will resolve this complication in most cases. Certain patients who display persisting bleeding may require assessment in the operating suite under general anesthesia.
A discussion with the patient's parents should entail the repair and the importance of allowing approximately two weeks with the palate undisturbed for tissues to heal correctly.
Diet should consist of milk or a diet that includes food that has been passed through the blender or pureed. Besides, avoiding carbonaceous beverages is essential. Hygiene is crucial and, in some patients, may require rinsing with a syringe with normal saline after meals.
Infants who are older than 18 months or who tend to introduce their fingers or objects into the oral cavity should have arm splints at all times (except while bathing). These devices do not hurt the infant and help prevent the patient from bending the elbow and introducing their fingers or objects in the mouth.
Nursing, Allied Health, and Interprofessional Team Monitoring
Observation of the patient in the recovery unit by the medical and nursing personnel until fully awake.
Formal monitoring of the patient's vitals, airway/breathing, and oxygen saturation levels is essential.
Controlling pain will provide a smooth recovery and help the patient have an adequate oral intake.
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Operative view of a secondary cleft palate involving hard and soft palate
Copyright and contributed by Ryan Winters, MD
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Patient with a bilateral, complete cleft lip and palate. Involves both primary and secondary palate, as well as lip. Note the protuberance of the premaxillary segment.
Copyright and contributed by Ryan Winters, MD
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Operative view of Furlow repair of patient in Figure 1: incomplete cleft palate involving hard and soft palate. Sometimes termed a "6-flap" palatoplasty; 4 flaps of the standard Furlow repair, plus the hard palate flaps performed concurrently.
Copyright and contributed by Ryan Winters, MD
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Adolescent patient with an isolated cleft palate involving the hard and soft palate. Preoperative.
Copyright and contributed by Jordi Espel, MD
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Adolescent patient with an isolated cleft palate involving the hard and soft palate. Postoperative.
Copyright and contributed by Jordi Espel, MD
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