Cleft lip and palate management require the right stages and timing because it requires holistic and comprehensive management. Cleft lip management consists of various surgical and non-surgical procedures by multidisciplinary health workers, start before the patient is born until the adult. 

The timing protocol for cleft lip management is as follows:

  • Prenatal: diagnosis, start counseling
  • Born: nutrition management, installation of orthopedic facilities, observation of hearing function
  • 3 months: labioplasty surgery
  • 12 months: palatoplasty surgery
  • 0-12 months: myringotomy ear tube installation (if there is chronic otitis media)
  • 1-4 years: speech evaluation
  • 4-6 years: pharyngoplasty surgery, revision of the lips and nose if needed
  • 6-12 years: orthodontics, ABG (alveolar bone grafting) surgery
  • Adolescent-bone maturation (12-21 years): definitive rhinoplasty, orthognathic surgery.

The techniques and timing above may differ depending on the cleft management protocol used. Surgery may also be performed late because of difficult access to health facilities, socioeconomic factors, patient preferences or the patient's family, and the presence of comorbidities.

Stages and Timings in Cleft Lip Management

The whole series of treatments are carried out by a multidisciplinary craniofacial team consisting of nurses, social workers, psychologists, dentists, pediatricians, speech therapists, and surgeons. This is following ACPA (American Cleft Palate-Craniofacial Association) guidelines.

a. Prenatal: Diagnosis and Counseling 

Comprehensive therapy includes the prevention of clefts by minimizing risk factors such as;
  • cigarette exposure,
  • pregestational and gestational diabetes, 
  • alcohol consumption, 
  • use of anticonvulsants, 
  • and deficiency of folic acid, vitamin B6, and B12.
Cleft diagnosis can be made prenatally by ultrasonography. 2-dimensional ultrasound has a detection ability ranging from 0-70%, depending on the ultrasonographer's experience, gestational age, and routine imaging of the fetus's face. 3D ultrasound has higher accuracy than 2D.

Counseling is carried out throughout the cleft lip management stages to provide support for the parents and family. Health workers, social workers, and cleft lip associations can support patients and their families.

b. Born: Nutrition Management

Patients with cleft lip often have breastfeeding problems due to difficulty forming a seal on the cleft lip and the inability to create negative pressure on the cleft palate. This problem can lead to malnutrition and dehydration in patients, even depression in the mother. Management is done by observing the growth of children, educating breastfeeding techniques, and using special bottles. Breastfeeding babies with cleft lip should also be done according to existing breastfeeding guidelines.

Pre-surgical Labial Adhesion

Pre-surgical Labial Adhesion is the process of dentofacial bone formation using orthodontic techniques. This stage is useful to form the alveolar arch to normal, reduce the cleft's width, improve nasal symmetry, increase the length of the column on bilateral cleft, and support family psychology. Molding can be done passively (by lip taping) or actively (using special nasoalveolar molding). An alternative method is surgical labial / nasolabial adhesion before definitive labioplasty. Labial adhesion is usually done at the age of 1 month.

Lip taping is performed from the age of 1 week. The two separate lip segments are brought together, and then the tape is placed across the cleft gap. The patient's family is educated to install the tape themselves at home. If complications occur in skin irritation, a dressing can be installed to protect the skin before taping.

Molding effective method requires planning, infrastructure, and specific follow-up by an orthodontist. The patient's maxillary model is formed in the first weeks of birth, then prosthetic nasoalveolar molding (NAM) is made, which is paired 24 hours a day and then adjusted periodically. NAM prosthetics will need many adjustments, so more control is required (every 1-2 weeks) and commitment from the patient's family. Complications of this method include skin irritation, poor compliance, and prosthetics that fail to remain attached.

c. 3 Months yo: Labioplasty

Labioplasty is a surgical procedure to close a cleft lip, usually done at the age of 3-6 months. ACPA guidelines recommend labioplasty done before the age of 12 months.

Labioplasty aims to restore oral competence, orbicularis oris muscle approximation, and achieve symmetrical and aesthetic results. Labioplasty can be accompanied by primary rhinoplasty with lower lateral cartilage repositioning to improve the rice and nasal tip's shape and position. The prerequisites for conducting this first stage of operation are the rule of Ten introduced by Wilhelmsen and Musgrave, namely 10 pounds (5 kg), ten weeks of age, and 10 mg / dL of hemoglobin.

The family must be educated about postoperative feeding techniques, the possibility of being treated, postoperative wound care, and time to control. Possible complications include injury, scarring, a residual deformity that may require revision surgery, and nasolabial fistula. Revision surgery is usually done at the age of 4-6 years before the child starts school.

d. 12 Months: Palatoplasty

Palatoplasty is an operation to close the cleft palate. Palatoplasty aims to approve the levator palatini muscle and extend the palate. Palatoplasty is usually done between the ages of 1-2 years.

As per ACPA guidelines, palatoplasty is recommended before 18 months of age. Timing is important because procedures performed too early risk causing problems in the airway and maxillary bone growth. Meanwhile, late palatoplasty will interfere with the development of speech function.

Family education includes the possibility of postoperative hospitalization and how to feed. Possible complications include bleeding, infection, tongue edema, difficulty breathing, fistula (can cause nasal regurgitation and hypernasal sounds), velopharyngeal dysfunction, impaired maxillary growth, and obstructive sleep apnea (OSA).

Some experts debate the timing of early palatoplasty because it causes disruption to the growth of the maxilla. Schweckendiek developed the 2-stage palatoplasty method (soft palate closure at six months and 14-year-old hard palate), which was intended to aid speech development and prevent jaw hypoplasia. Nevertheless, it was found that this method was not far superior in terms of the trauma caused. The speech function results of patients who must use a hard palate obturator are also considered unsatisfactory.

Another alternative method is labia and palatoplasty surgery simultaneously. The patient's age at the time of the surgery also varied. However, this group of patient data is not sufficient for long-term evaluation.

e. 0-12 Months: Ear Evaluation and Myringotomy

Pediatric patients with cleft palate have a higher risk of developing middle ear infections due to Eustachian tube dysfunction, which causes chronic middle ear effusion. Middle ear secretions in cleft patients also have a higher viscosity making drainage difficult. A stagnant middle ear condition without aeration becomes an inflammatory nidus, causing otitis media.

There is also an increased incidence of sensorineural, conductive, or mixed hearing loss in children with cleft palate. Ear disorders that occur can lead to speech, language, cognitive, and emotional disabilities.
Examinations that can be done to detect hearing loss or middle ear disease include tympanometry, audiometry, otoscopy, newborn hearing screening, measurement of sphenopalatine angles, mastoid air cell systems analysis (with Roentgen), wideband reflectance, and ABR (auditory brainstem response).

Interventions to improve hearing function include myringotomy and installing a ventilation tube, palatoplasty, and/or hearing aids. [3,6] Infants who breastfeed for a longer duration are associated with lower otitis media episodes. Post adenoidectomy patients have a lower incidence of otitis, but the procedure is not routinely performed because it can cause velopharyngeal incompetence. Follow up is done until adulthood to detect hearing loss and cholesteatoma.

f. 1-4 yo: Evaluation of speech function and pharyngoplasty

VPD (velopharyngeal dysfunction, velopharyngeal dysfunction) is the inadequacy of of velopharyngeal closure when talking so that air comes out through the nose when voicing consonants. This causes hypernasal sounds. VPD is classified into insufficiency (due to structural/anatomical deficits), incompetence (functional deficits), and mislearning (errors of articulation and placement/closure of the palate).

Detection of VPD should be started by a pathologist speaking for about 18 months and continued routinely into adulthood. Evaluation of adequate speech function can only be done at 4-5 years of age. Diagnosis is assisted by nasometric examination, air pressure flow test, nasal endoscopy, and videofluoroscopy.

Speech therapy can be the main therapy, especially in VPD due to the velopharyngeal mislearning, or acts as a pre / postoperative adjunct therapy.

Pharyngoplasty surgery is performed based on the patient's velopharyngeal closure pattern found on investigations. Actions can be augmented by the posterior pharyngeal wall, lengthening the palate, and or velopharyngeal changes. A small gap in the middle can be corrected with injection pharyngoplasty. In the levator muscle dehiscence, Furlow palatoplasty is used to reorient muscles and extend the palate. Velopharyngeal changes that can be done by sphincter pharyngoplasty and posterior pharyngeal flaps. Complications of action include persistent VPD,  licensing, and OSA.

g. 6-12 yo: Orthodontics and Alveolar Bone Grafting (ABG)

Dental anomaly is one of the common problems in cleft patients. These anomalies can be number abnormalities, eruption errors, and ectopic, transposition, or irregular teeth. Orthodontic therapy is done by installing orthodontic wires to help restore the teeth' optimal alignment, optimize the position of the maxillary segment before surgery, and maxillary expansion. The management of orthodontics requires careful planning by a multidisciplinary team.

Alveoloplasty and bone grafting are done to close gaps in the gums. This action is important to close the oronasal fistula and support the anterior maxilla and nasal base. A successful ABG will produce an adequate dental arch for tooth growth or dental implant placement. ABG also helps control the orthodontics of alveolar segments and improves teeth position in the maxillary arch.

Alveoloplasty is classified into primary (together with soft tissue correction), secondary (age 8-9 years before the permanent canine eruption), and tertiary (in adult patients). The bone graft can be taken from the iliac bone to fill the alveolar opening. Secondary ABG on mixed dentition duration does not cause facial growth restriction compared with primary ABG. At this stage, a minor revision of the lips can also be made if needed.

f. 12-21 yo: Orthognathic Surgery and Rhinoplasty

Cleft patients have a maxillary deficiency, both due to intrinsic factors of the cleft embryology itself and iatrogenic due to previous surgery. Orthognathic surgery is an osteotomy and resettlement of the maxillary and/or mandible to achieve normal occlusion. Procedures that can be performed include Le Fort 1 osteotomy, BSSO (bilateral sagittal split osteotomy), and distraction osteogenesis. Orthognathic surgery can be accompanied by bone grafting from the iliac to fill in the remaining palate or nasal defects. Orthognathic surgery is performed before rhinoplasty.

Rhinoplasty is performed to improve the shape of the nose that is deformed due to a cleft. The nasal deformity can occur due to cartilage, bone, or skin abnormalities (primary) and scar tissue due to surgery (secondary). Because of poor cartilage structure, breathing difficulties can also occur in cleft patients.

Primary rhinoplasty is performed simultaneously with labioplasty. The rhinoplasty procedure's main objectives are nasal bed closure, lower lateral cartilage repositioning, and ala bed repositioning.

Definitive rhinoplasty is usually done after nasal growth is complete, and bone is mature, i.e., in adolescence. The optimal age of rhinoplasty for male patients is 16-18 years, while women are at 14-16 years. However, the earlier intervention can be done if there is airway disturbance or nasal tip deformity. Actions that can be taken at this stage include resectioning the deviated septum, repositioning the septum, stitch fixation of the cartilage, and cartilage graft (of the resected septum, concha, or rib), or reduction of the nasal base.