Cleft Lip/Palate: Hidden Aspects beyond the Gap

4.2.1 Counseling

Parents are often sad and devastated by the birth of a CLP child [24]. Formation of a supportive parent-child relationship is a must for the child’s development [25]. Counseling plays a pivotal role in the adaptation of the family to the child born with CLP or detected to be afflicted with the malady in the antenatal period. It is also essential to facilitate long-term collaboration between the parents and the various healthcare professionals involved in the multidisciplinary treatment team. Information regarding the etiology of the cleft and the treatable nature of the disease helps in alleviating the guilt and anxiety of the parents. The counseling session should address questions regarding feeding, growth, other health issues, and their management [15]. Complete, accurate, and unbiased information must be shared, and parents must be made aware of the availability and accessibility of healthcare facilities. A comprehensive treatment plan must be outlined. Parents should be helped in contacting organizations that provide one-to-one support and family-centered team care. The need for long-term follow-up extending into adulthood must be explained to the family.

4.2.2 Genetic counseling

CLPs tend to recur in families. A positive family history is found in approximately every sixth child with CL ± P [26]. The risk of a child getting affected is 3.2% if one parent is affected with CL ± P and 6.8% if the parent is affected with CP. If a sibling is affected by CL ± P, the risk of the next child getting affected is 4.4%. If a sibling is affected by CP, the risk reduces to 2.5%. This risk increases to 9% after two affected children are born. The risk is even higher, to the extent of 15%, if one parent and one sibling are affected [27, 28, 29].

4.2.3 Feeding and nutrition

The lip and palate play a significant role in the feeding mechanism. The palate superiorly, lip anteriorly, tongue inferiorly, bilaterally the cheeks, and posterior pharyngeal wall posteriorly form a closed chamber to create negative pressure for sucking milk from the breast or bottle [11]. Monthly monitoring is necessary to observe weight gain, hemoglobin level, and skeletal growth till the cleft palate is closed. After that, the achievement of milestones and weight gain is monitored.

Specially designed feeding bottles are available for these children. Haberman feeder, Mead Johnson feeder, Lamb’s nipple, cross-cut nipple, and long nipple are some of these [11]. Some of these devices deliver the fluid directly over the posterior part of the tongue so that the baby need not depend upon the sucking phenomenon [11]. In our country, feeding with a spoon or paladai (modified spoon with a nozzle) is prevalent. Special bottles in which flow is either gravity-dependent or squeeze-dependent are also available. These devices deliver milk at a controlled rate. However, these devices are expensive. Feeding techniques, such as holding the child in a semi-reclining position and patting the back to cause burping, are taught to the family. Ideally, the feed should not last more than 30 minutes, as longer feeds exhaust the baby.

4.2.4 Presurgical infant orthodontics and lip repair

The primary goal of all infant Orthodontics is to align the cleft segments into a correct anatomical position [17]. The nasal cartilage deformity is improved with the use of a nasal stent to reshape the nasal cartilage, lengthen the columella, and achieve projection of the flattened nasal tip [17]. Nasoalveolar molding is the most common form of passive infant Orthodontics used today.

The standard methods of unilateral lip repair practiced worldwide are the Randall-Tennison geometrical method (Figure 3), Millard’s rotation-advancement method (Figure 4), and the Pfeiffer-Afroze method. The standard methods of bilateral cleft lip repair practiced worldwide are those described by Veau, Mulliken, Black, and Millard.

4.2.4.2 Millard repair

Landmark points are marked (Figure 4). The lateral philtral point is marked as the highest point of Cupid’s bow on the non-cleft side. The deepest point on Cupid’s bow is marked. The corresponding lateral philtral point is marked on the cleft side at the point. The mid-columella point is marked at the midpoint of the columella base. The alar base and columella base are marked bilaterally. On the lateral lip element, the lateral philtral point is marked at the point where the white line begins to fade. Castroviejo caliper is used to measure the heights of the lip on the cleft and non-cleft sides (The second part of Figure 4 demonstrates a case with philtral height of 10 mm and 4 mm on the non-cleft and cleft sides, respectively). This difference gives a rough idea as to the amount of back cut required [16].

After adrenaline infiltration, the cleft edges are pared, and the frenulum is cut. The rotation incision is started from the tattooed point of Cupid’s bow. The incision ascends up to the base of the columella with a gentle convexity facing the cleft [16]. It hugs the columella base for about two-thirds of its distance and ends with a back cut made perpendicular to the incisions. This back cut should stop short of the non-cleft side philtral column. As shown in the second part of Figure 4, the incision at the base of the columella is approximately 3 mm, and the back cut is 3 mm. These incisions bring about adequate rotation. As in Randall-Tennison repair, all false attachments of the orbicularis oris muscle are released. Septum correction, repair of anterior palate, and closure in three layers are done, as described in Randall-Tennison repair.

Conventionally, the anterior palate is closed along with the lip [18]. Previously, surgeons used an inferiorly based nasal septal and vomerine flap from the medial aspect and the mucoperiosteum from the lateral shelf. This led to growth problems, and these flaps were abandoned. Pichler and Sommerlad popularized the use of a superiorly based vomer flap and sutured it to the mucoperiosteum of the cleft-side palate to close the anterior palate in a single layer.

Correction of cleft lip nose deformity is accepted worldwide. McComb, Coghlan, and Salyer et al. popularized the concept of addressing the nose deformity along with cleft lip. Gradually, it was accepted worldwide, and now, it has become an integral part of cleft lip repair in many centers (Figure 5) [18, 19]. There are many variations in practices among surgeons involved in cleft care [20]. A total of 43% of surgeons do primary rhinoplasty at the age of 3–4 months, along with cleft lip repair, whereas 33% wait for 6 months. A total of 31.2% of surgeons believe in minimum dissection, whereas for 62.7%, dissection varies according to the deformity; 89.7% of surgeons feel primary correction reduces deformities; and 54.1% of surgeons feel one-fourth of patients may require revision before 7 years of age [20]. If the deformity is too much, a preschool rhinoplasty (at the age of 4–5 years) or intermediate rhinoplasty (at the age of 9–12 years) is advisable. According to Chang et al., the patients who underwent a combination of nasoalveolar molding, primary rhinoplasty with over-correction of the ala, and the use of nasal conformer for 6 months postoperatively, had the best outcome [21].

4.2.5 Hearing assessment and management

Regular otological examination and audiological surveillance using impedance tympanometry for hearing assessment are done. If required, ventilating tube (grommet) are inserted. Grommets are inserted into the anteroinferior quadrant of the tympanic membrane. These aerate the middle ear and prevent fluid collection, thus, preventing chronic otitis media [22]. These tubes are spontaneously extruded in 6–24 months. Prophylactic insertion of a grommet is no longer recommended. Indication of grommet is recurrent otitis media (more than three episodes in 6 months) or audiological evidence of hearing loss greater than 55 dB [23]. Aggressive otological and audiological surveillance and timely intervention improve hearing and speech outcomes in these children. Hearing aids are an excellent noninvasive option for improving hearing with minor morbidity. They are used to manage mild effusion, which is expected to resolve spontaneously in a few months [31, 32].

4.3.1 Palatoplasty

Hard palate repair techniques: Various techniques are utilized depending on the extent of the cleft. If the cleft extends to the entire length of the palate and the cleft is not too wide, von Langenbeck’s bipedicle flap technique is employed (Figure 6). Bardach’s two-flap technique gives the best results if the entire palate length is cleft and the gap is wide. If the cleft is very wide and the palatal shelves very narrow, it may be advantageous to do alveolar extension palatoplasty. For incomplete cleft of the posterior palate, the Veau–Wardill–Kilner VY technique (Figure 7) or the Dorrance pushback technique offer good repair.

Soft palate repair techniques: In cases where the cleft extends only to the soft palate and the hard palate is not involved, the focus is on repairing the fibers of LVP, along with the closure of the nasal and oral mucosa. Furlow’s double opposing Z-plasty (Figure 8), modified Furlow’s technique, primary closure of mucosa with intravelar veloplasty, or Sommerlad’s radical muscle dissection techniques are practiced.

In many countries, including ours, many patients do not return for palatoplasty after the correction of a cleft lip due to various reasons. To tackle this social problem, Agrawal et al. started practicing a protocol of repair of CP at 6 to 9 months of age or whenever the patient first presents to the clinician and repair of cleft lip 3 to 6 months after palatoplasty. Compliance with the second surgery (lip repair) improved significantly with this schedule. Also, when the functionally important palatoplasty is done first, and the second surgery of cheiloplasty is delayed for any reason, it is not a significant disadvantage to the child [33]. In children older than 3 years of age, a “whole-in-one” procedure has been popularized by Charles Pinto, wherein lip and palate repair are done in the same sitting [34].

4.4.1 Velopharyngeal dysfunction

Velopharyngeal sphincter closure is achieved by synchronous movement of the posterior pharyngeal wall anteriorly, the lateral wall medially, and the soft palate posteriorly [35]. A firm diagnosis of velopharyngeal dysfunction may not be possible before the age of 3 years because appropriate testing is complex in such young children. For most children, reliable testing can be performed when the child is between 3 and 5 years of age. Once the diagnosis of velopharyngeal dysfunction has been made, treatment may consist of nonsurgical speech therapy, corrective palate surgery, reconstructive pharynx surgery, obturation with a speech bulb, or placement of a palatal lift prosthesis [35]. Once the diagnosis is confirmed and it is ascertained that the treatment will be surgical, the timing of intervention should be early to prevent long-term speech difficulties and abnormal articulatory compensations that are difficult to correct later in life [11].

Depending on the site and cause of pathology, the following surgical options are available for managing velopharyngeal dysfunction. For repair/reinforcement of LVP, following procedures may be done: Redo-intravelar veloplasty, Furlow’s double opposing Z-plasty and radical muscle dissection palatoplasty. Palate lengthening is achieved using a buccal myomucosal flap [36]. In rare cases, genu enhancement using fat can suffice. If the velum is functioning well and the closure of the velopharyngeal sphincter is inadequate in the anteroposterior axis, superiorly based pharyngeal flap or augmentation pharyngoplasty can take care of the deficit in closure. Hynes or sphincter pharyngoplasty are procedures to correct the closure of velopharyngeal sphincter in transverse axis. Sometimes, there may be a need to combine multiple procedures [37]. Tables 2 and 3 summarize the choice of procedure with the findings on nasoendoscopy and video fluoroscopy, respectively.

4.5.1 Secondary alveolar bone grafting (SABG)

The alveolar cleft in complete CLP is addressed at the time of primary cleft lip repair or secondarily. Nowadays, the practice of primary bone grafting (also called “boneless bone grafting” or gingivoperiosteoplasty (GPP)) has been abandoned, as it was shown to be associated with more scars, the need for SABG in a significant proportion of patients and more pronounced maxillary hypoplasia [38, 39]. For doing GPP, the alveolar gap is narrowed using presurgical orthopedic devices like the Latham’s device or nasoalveolar molding. Gingioperiosteal flaps then bridge the alveolar gap. The gingivoperisteosteal flaps induce the formation of bone. Hence, this procedure was dubbed “boneless bone grafting”. This practice was pioneered by Skoog [40].

SABG is practiced at all centers these days. SABG is preceded by orthodontic treatment, during which the arches are aligned, and the teeth are derotated and erupted uniformly. Just before the permanent canine appears to erupt, SABG is performed. This allows the canine to erupt through the bone graft. This increases the likelihood of a parallel root positioning, with more excellent stability of the canine [30, 41, 42]. This also lessens the possibility of graft resorption. Post SABG, orthodontic treatment aids in “retention” of the result, as other than the alveolus, the rest of the cleft area is covered only with soft tissue. The disrupted maxilla is more prone to relapse in the initial days, and thus, orthodontic treatment is crucial after SABG.

In those children who present later after eruption of canine in the cleft or whose canine is affected with caries, tooth extraction is advised. After that, SABG is done with tooth implant placement. The long-term success of the tooth implant is good, and the implant acts as a functional stimulus to maintain the bone. However, a significant number of the implants require tertiary grafting [43].

4.6.1 Maxillary advancement

Completion of maxillary development happens around 17 years in females and at about 18 years in males. This can be determined by identifying the carpal sesamoid (pisiform) bone in hand and wrist radiographs or by studying the lower border, height, and shape of the cervical vertebral body [16, 43, 44]. Midface hypoplasia has been reported by several authors to occur between 25 and 70% of all cleft patients, with an estimated 25 to 48% needing surgical treatment for skeletal correction [45, 46].

Some patients with mild midface hypoplasia, mild malocclusion, and good cosmesis can be offered orthodontic camouflage. Maxillary advancement procedures with preoperative and postoperative orthodontic treatment are necessary for the rest of the patients. The mainstay of surgical options in cleft midface hypoplasia is the advancement of the maxilla by Le Fort I osteotomy and rigid fixation using plates and screws [45]. Low Le Fort I level osteotomy about 4 to 5 millimeters above the floor of the pyriform aperture results in a harmonious facial profile in most cases [45]. A maximum advancement of 7 to 8 mm is possible in one go. Beyond this, the bone-to-bone contact is not enough for bony union. The use of bone grafts can allow advancement of about 2 millimeters more [46]. Too little contact between the bony segments leads to healing by the fibrous union (instead of the bony union), which has poor stability and may lead to mobile tooth-bearing segments.

The advancement of maxillary bone stretches the soft tissues. The soft tissues around the maxilla are scarred to varying degrees, having undergone many surgical procedures in the lifetime of the cleft patient. The scarred and stretched soft tissues cause a relapse of malocclusion in these patients. Therefore, maxillary advancement of more than 8–10 millimeters is always accompanied by a distractor device application, which works on the principle of “distraction histogenesis.” Distraction histogenesis is a slow process, taking weeks to months. The patient has to retain the bulky device for that long.

Another strategy is to combine maxillary advancement with mandibular setback. This distributes the required correction between the upper and lower jaws. Thus, each bone’s mobilization is reduced while better stability of the osteotomized fragments is ensured. Several authors have reported that bimaxillary surgery gives overall better esthetic and functional results and stability than maxillary advancement or mandibular setback alone [47]. However, the planning and execution of the osteotomies and fixation have to be more meticulous.

4.6.2 Secondary deformities

Despite advancements in cleft care and the development of excellent comprehensive care centers, most patients, especially in developing countries with clefts, still need to receive ideal treatment as per the current international protocols [48]. Surgeons need to understand the longevity and profound impact of poorly performed surgeries. Secondary deformities of the cleft lips are a rule rather than an exception [48]. Even after the most perfect initial repair, deformities may occur due to scar maturation and growth. Many children do not undergo any presurgical orthopedics/orthodontics or subsequent dental and maxillofacial treatment, thereby aggravating the secondary deformities [48].

The treatment should ideally start with presurgical orthopedics followed by cheiloplasty, palatoplasty, continued orthodontic treatment, preschool minor revisions, distraction of midface when necessary, rhinoplasty, and orthognathic surgery as required in adulthood [48]. Throughout this treatment, the patient’s result is dynamically influenced by growth. The final result of any cleft surgery is hence evident only after the child’s growth is complete. Therefore, it is difficult to envisage a scenario without secondary cleft lip deformities.

Secondary deformities of the lip are classified as scar-related, lip-related, white roll-related, vermillion-related, philtrum-related, muscle-related, or asymmetry of the structures. Scar-related issues may be a wide scar, hypertrophic scar, contracted scar, scar with cross hatches/suture marks, and hyperpigmented/hypopigmented scar. Lip-related problems may be the presence of a long lip, short lips, or tight lips. The white roll may be misaligned, scarred, or thinned. Vermillion may be misaligned, notched, thinned, or deformed (“whistle lip deformity”). Besides being unsightly, lack of muscle repair or poor muscle alignment leads to shallow buccal sulcus or absence of a sulcus, causing an adherent lip. Philtrum, being the central element of the lip, is very apparent when it is wide, narrow, or absent. The secondary deformities must be corrected surgically whenever the patient presents, as they cause ridicule of the child in school, and most of these deformities worsen with the child’s growth.

4.6.3 Secondary rhinoplasty

After a balanced and symmetric skeletal base is achieved, secondary rhinoplasty is performed. No single technique, to date, has provided a definite solution for correcting all the problems accompanying these deformities. Secondary rhinoplasty can be performed using an open or closed technique. The open approach is preferred for better exposure and visualization of the nasal elements. An open technique refers to lifting the skin off the nose to operate, while a closed technique is done through tiny incisions. Transcolumellar and infracartilaginous incisions are most frequently employed to visualize the lower lateral cartilages and the nasal septum.

The usual steps required in definitive rhinoplasty are septum straightening, tip elevation, columella lengthening, alar rim augmentation and dorsum augmentation with cartilage grafts, alar base augmentation and repositioning, correction of vestibular webs, elevation of nostril sill and nasal bones osteotomy. The extent of surgery needed is tailor-made to each patient’s deformity.

The usual steps are as follows. The open dorsal approach with tip-takedown is used to expose the septum. The septum is partially resected, leaving an L-shaped strut for dorsal caudal support. The remaining septum is repositioned in the midline and sutured to the periosteum of the anterior nasal spine. Submucous resection of the hypertrophied inferior turbinate may be required to improve the nasal airway. The inferiorly displaced cleft-side alar cartilage is advanced medially and sutured to its counterpart on the non-cleft side to obtain a balanced nasal tip. Placement of columellar strut graft, along with suturing of the medial and middle crura of both the alar cartilages to the tip of the strut, achieves tip projection, symmetry, and support [11].

The placement of cartilage grafts for support and reinforcement is a significant element of the cleft rhinoplasty surgery. Autologous cartilage grafts, from septum, costa, and auricle, are used for augmenting deficient areas and restoring structural support in the nose. Splinting the alar rim with cartilage grafts is required, as the cleft cartilage is too weak to withstand the deforming forces of the depressed dorsal skin mold, gravity, and growth. Patients with internal nasal valve dysfunction may benefit from a spreader graft. Patients may need lateral osteotomies to narrow the dorsum, or straighten a deviated nasal pyramid. If lateral osteotomies do not adequately narrow the dorsum, a central segment is removed to create an open roof via paramedian osteotomies [11]. The nasal bones are, then, in-fractured to achieve adequate narrowing. Nasal bone osteotomies are performed either percutaneously with a 2-mm osteotome or transnasally.