Obstetrics

The first report on prenatal detection of a CLP deformity in two fetuses by transabdominal ultrasound was published by Christ and Meininger (1981). Currently, almost all pregnant women receive routine ultrasound screening during their pregnancy. This examination usually involves two‐dimensional (2D) ultrasound and is performed in a primary care setting. The problem is that transabdominal 2D ultrasound screening for orofacial clefts in a low‐risk population has relatively low detection rates, ranging from 9% to 100% for CL ± P, from 0% to 22% for CP only, and from 0% to 73% for all types of clefts (Maarse et al., 2010). The favorable result of this finding is that it is associated with few false‐positive diagnoses. If there is suspicion of a fetal anomaly, the woman should be referred for more advanced three‐dimensional (3D) ultrasound imaging, which has, in high‐risk women, much higher detection rates of 100% for cleft lip, and 86–90% for CLP. However, CP only is hardly detected on 2D ultrasound, and 3D ultrasound is also unable to arrive at a reliable diagnosis for CP only, as detection rates vary widely from 0% to 89% (Maarse et al., 2010).

Today, in our center, more than 60% of the expecting parents have received a prenatal diagnosis of their child and have been counseled accordingly. Detection of the facial cleft in utero by ultrasound has resulted in an established routine between the referring obstetrician and the CLP team for informing the parents of the deformity and the sequential steps necessary for its correction. The way of communicating the diagnosis, “breaking the news,” and the quality of the information received at the time of diagnosis can considerably affect the long‐term well‐being of expecting parents, as well as their attitudes toward the diagnosis, further antenatal testing, and even pregnancy termination (Stock et., 2019a). Due to unsatisfactory information and a perceived lack of empathy from health professionals, respondents reported feeling upset, offended, and/or anxious following the diagnosis (Stock et al., 2019b). Expecting parents need to be aware that a negative ultrasound result does not necessarily mean that their unborn child is without an orofacial cleft (Maarse et al., 2010).

Pediatrics

The pediatrician is an essential part of the CLP team, providing initial diagnosis and referral, close monitoring of the day‐to‐day medical issues including immunizations, management of growth (particularly for infants who have difficulty with feeding and weight gain), treatment of acute ear infections, assessment of development, and family support (Kasten et al., 2008). More importantly, CL ± P may occur in association with other congenital malformations. In a Swedish study, 21% of the infants with clefts had associated malformations that required follow‐up treatment. A systematic review regarding associated developmental defects that included 13 studies with postnatal data revealed that the prevalence of associated anomalies was lowest in babies with a cleft lip (7.6–41.4%; Maarse et al, 2012). A more extensive cleft seems to be associated with a higher risk for associated malformations (for CLP 21.1–61.2%). However, girls with CP only, which has a different embryological origin, showed the highest percentage of associated anomalies (22.2–78.3%; Maarse et al., 2012).

Clinical nursing and/or social work

The clinical nurse or social worker is the vital link between the CLP team and the parents and child. He or she guides them through the treatment process from birth until maturity, or sometimes even earlier if playing a role in the prenatal counseling. Because many children have difficulties nursing from a breast or bottle, the clinical nurse is there to assist the parents directly after birth regarding feeding issues, and to provide psychological support for the parents to deal with acceptance of their child with a deformity. The clinical nurse assumes the role of “case manager,” organizing and coordinating services throughout the clinical pathway followed by the patient, serving as a walking encyclopedia for questions from the family and the patient. In our experience, the availability of advice and counseling by phone or through a protected online environment is very helpful, but sometimes home visits are necessary.

The clinical nurse or social worker will organize, if needed, contacts with social and vocational rehabilitation services, insurance companies, schoolteachers, and governmental support systems (Frederick et al., 2022).

Surgery

Anatomical defects of the lip, oral cavity, nose, pharynx, and upper airway need surgical reconstruction to restore function, facial esthetics, speech, and hearing (Frederick et al., 2022). Children with a complete unilateral or bilateral cleft undergo at least three surgical interventions: cleft lip repair, hard and soft palate repair, and alveolar bone grafting. Additional secondary surgery may be needed for velopharyngeal incompetence, skeletal discrepancies, lip revision, and nasal deformities. Table 7.1 gives an overview of one center’s treatment plan and timing of interventions. It should be realized that large variation exists between centers, as there is no approved “superior” schedule. It depends on national agreements and from which discipline the surgeon who is performing these operations originates. Possibilities are maxillofacial surgery, and/or plastic and reconstructive surgery, and/or otorhinolaryngology.

The experience and skills of the surgeon seem to be a major issue in achieving a good treatment outcome. Results from the EUROCLEFT study suggest that decentralized care and low‐volume surgeons are associated with low‐quality treatment results (Shaw et al., 1992a, 1992b). These findings induced the Clinical Standards Advisory Group (CSAG) study in the UK in 1998 that confirmed the poor outcomes of cleft care in the country (Bearn et al., 2001). Eventually, the UK government took the decision to reduce the number of cleft centers from 57 to 16 that function as managed clinical networks (Sandy et al., 2020). A follow‐up study, performed in 2013 and known as the CCUK (Cleft Care United Kingdom) study, showed that centralization of cleft care was indeed associated with better outcomes for facial and dento‐alveolar variables (Al‐Ghatam et al., 2015). This finding emphasizes the great importance of centralized care for children with clefts.

Orthodontics

The orthodontist has an active role in the interdisciplinary care of children with orofacial clefts, from birth until they become adults. Longitudinal evaluation, including standardized follow‐up documentation, and record taking to provide a general overview of treatment outcomes are in the hands of the orthodontist. Five distinct stages of orthodontic mechanotherapy can be distinguished. A detailed description of these stages is provided later in the chapter.

Genetics

Genetics is becoming increasingly important in the search for the etiology of orofacial clefts, and therefore the role of the clinical geneticist in the CLP team increases as well. All parents expecting a child who has been diagnosed prenatally as having a cleft with associated anomalies should be counseled antenatally by the clinical geneticist. About 3–6 months after birth, all children with clefts are examined by the clinical geneticist, preferably before their first operation (Mink van der Molen et al., 2021). This timing is important, as not all associated congenital malformations may manifest at birth. However, when growth problems or developmental delay with associated abnormalities become manifest, and a syndrome diagnosis or chromosomal abnormality is suspected, earlier genetic examination is recommended (Mink van der Molen et al., 2021). Parents are also counseled about the risk of having another child with a cleft. Over the years we have learned that adults with CLP who were treated by the team return with questions about their own risk of having a child with a cleft.

ENT and audiology

The ENT specialist and audiologist are responsible for the audiological and otological management of children with clefts. The association of the presence of a CP with middle ear effusion is widely recognized (Hubbard et al., 1985; Gani et al., 2012). CP may lead to disruption of the tensor veli palatini muscle, which plays a role in the function of the Eustachian tube. Dysfunction of the Eustachian tube increases the risk of serous otitis media and more than 90% of patients with CP have middle ear effusions. Placement of ventilating tubes (grommets) is frequently utilized. Increasing evidence suggests the benefits of grommets for speech and language development in CLP children with otitis media with effusion (Kuo et al., 2014). Some children require multiple sets of tubes, and there is increased risk of persistent hearing loss. The ENT specialist is responsible for prescribing hearing aids when necessary, and may also address issues of tonsillar and adenoidal hypertrophy, sinusitis, nasal abnormalities, and nasal obstruction (Kasten et al., 2008).

Speech‐language pathology

A speech‐language pathologist, specialized in CP speech, is indispensable in a CLP team. Children with CLP are at a higher risk for speech disorders, and to a lesser degree for language disorders. The speech disorders in children with orofacial clefts concern articulation, phonation, and resonance disorders, primarily hypernasality. The speech‐language pathologist treats communication disorders and contributes the necessary information to the team when surgical interventions are considered to improve speech. Careful planning of orthodontic treatment in relation to speech therapy is also needed, as certain developmental stages of the dentition and some orthodontic appliances interfere with speech therapy. The speech‐language pathologist is heavily involved in the care of children with clefts during the first nine years of life. After that age, most speech problems have already been solved. In some teams the speech therapist also serves as a lactation consultant or feeding expert; in others this is the role of the clinical nurse.

Dental care

Caries is often a problem in children with CL ± P. They have more decayed, missing, or filled teeth/surfaces than noncleft controls, in both the deciduous and the permanent dentition (Abirami et al., 2022). The pediatric dentist of the team plays an important role in implementing caries prevention strategies for the child with an orofacial cleft. Routine dental care should be provided by the family dentist, in close contact with the CLP team. As many children with orofacial clefts have a suboptimal oral hygiene, an oral/dental hygienist could assist the dentist and the orthodontist in maintaining good oral hygiene through the years of orthodontic treatment. The prosthodontist is responsible for replacement of missing teeth, and embellishment of abnormally shaped teeth, as an adjunct to the orthodontic and surgical treatment plan. Therefore, the prosthodontist always works in close collaboration with the orthodontist and, eventually, the surgeon.

Psychology

Last, but not least, the psychologist is mentioned as an important team member. When a child with a cleft is born, at first the parents experience psychological distress and the event will have an impact on the family (Stock et al., 2020; Sagar and Sahu, 2022). For some children psychosocial support is never needed, while others have numerous psychological and social problems. Living with a facial disfigurement is not easy, as the deformity is always visible and may be hampered by speech and hearing difficulties. This may have a severe impact on the well‐being of the child. Moreover, teasing and social rejection at school and by peers can grow into a severe problem that needs to be addressed professionally. Fostering psychosocial adjustment, positive self‐esteem, and healthy social skills are objectives that should be met by the team treatment (Sagar and Sahu, 2022).

From birth to 7 years of age

At the first visit to the team a clinical examination is performed, while concomitant medical conditions of the child are also taken in consideration (Worley et al., 2018). Together with the parents, an initial holistic treatment plan is made. The short‐term goals are supporting the family to overcome psychological or social distress, solving eventual feeding difficulties, and reconstructive surgery of the lip and/or the palate, while infant orthopedics can precede the surgical closure of the lip.

Baseline documentation is then created, consisting of frontal, right, and left lateral facial views, a close‐up of the lips, and a worm’s‐eye view (a view of the patient’s face from below). It is quite difficult to make standardized pictures of young toddlers. It is easier to photograph them lying down, at least until the age of 6 months when they begin to sit up by themselves. A palatal intraoral view could be obtained in the operating room. The Institute of Medical Illustrators of the UK has developed an excellent illustrated guideline for photography of patients with clefts, which can be downloaded from its website (lnstitute of Medical Illustrators, 2021).

3D stereophotogrammetry images have the advantage that they produce a 3D image of the deformity, which allows for sophisticated quantitative analysis of the face in three dimensions and does not require ionizing radiation (Brons et al., 2019b; Kuijpers et al., 2022). However, in our experience such images are difficult to obtain before the age of 3 months, despite the very short capture time, as head balance has not developed before that time and the parents need to hold the child’s head, which may disturb the facial soft tissues. Furthermore, it is impossible to ask a baby to keep a neutral facial expression (Brons et al., 2019a).

A baseline maxillary dental cast of the unoperated cleft condition has been proven to be very helpful for later ascertainment of the exact diagnosis for research purposes. Due to the risk of respiratory obstructions resulting in cyanotic events or worse, maxillary impressions should only be taken by an orthodontist who is experienced in impression taking in cleft‐affected babies. The maxillary impression could be made just prior to the lip repair in the operating room (Figure 7.2). Digitization of the plaster casts will facilitate measuring models and long‐term storage. Currently, digital impression taking using intraoral scanning of babies seems to be a promising technique that is far more child and parent friendly than taking impressions (Benitez et al., 2022; Weise et al., 2022). Unfortunately, the size of the scanner tip is often too big to reach easily behind the maxillary tuberosities in a newborn, as these scanners were developed for adult use (Figure 7.3). However, it will be a matter of time before scanners come onto the market with a smaller scanner tip.

In most centers, the first surgical intervention for a child with CL ± P is surgical repair of the lip, followed a couple of months later by palatal repair. To date this is a highly controversial issue and no consensus exists among centers. After the infantile period, the CLP team follows the child on an annual basis. Speech and hearing are the main issues during this period.

Neonatal maxillary infant orthopedics

Whether neonatal maxillary infant orthopedics will be part of the treatment plan prior to surgical repair of the lip depends on the treatment protocol of the team. Ever since its introduction by McNeil (1950), it has been a controversial issue. In Europe, about 54% of the operational centers used infant orthopedics (Shaw et al., 2000), while in the USA this approach has always been less popular.

Basically, infant orthopedics aims at molding the alveolar segments into the correct anatomical position, to facilitate lip repair by an intraoral acrylic appliance. Later on, the orthodontic discipline tried to justify early intervention for other reasons. Kuijpers‐Jagtman and Prahl (2013) summarized the arguments of the proponents of the use of infant orthopedics, who state that this approach allows a more normalized pattern of deglutition while preventing twisting and a dorsal position of the tongue in the cleft, improves the arch form and position of the alar base, facilitates surgery, and improves outcome in general. Other alleged benefits that became en vogue are reduction of posterior cleft width, prevention of initial collapse after surgery, prevention of crossbites, straightening of the nasal septum, facilitation of feeding, reducing the danger of aspiration, improving speech development, nose breathing, reducing the severity and frequency of middle ear conditions, the extent of orthodontic treatment at later ages, and creating a positive psychological effect in the parents (Kuijpers‐Jagtman and Prahl, 2013). Most of these benefits could not be substantiated by scientific evidence, however, as demonstrated in several systematic reviews (Papadopoulos et al., 2012; Hosseini et al., 2017).

A wide range of appliances has been designed for these purposes, with pin‐retained active appliances at one side of the spectrum (Georgiade and Latham, 1975) and passive appliances at the other side (Hotz and Gnoinski, 1976). Arbitrarily, they fall into three main categories: active, semi‐active, and passive appliances (Kuijpers‐Jagtman, 2006). Active appliances are constructed to apply a force to the maxillary segments, to move them in the desired direction by using an active force delivery system, like springs and screws. Additional anchorage can be obtained by pins that are driven into the maxillary bone holding the plate in position. Semi‐active appliances are constructed by sectioning the dental cast and reorienting the maxillary segments into a more favorable position. The plate is fabricated on the reconstructed cast and forces the palatal segments in the predetermined direction when placed in the oral cavity (Figure 7.4). External strapping across the cleft can be part of the treatment protocol. These are the McNeil (1950) type of appliances. Passive appliances that are combined with extraoral strapping also fit into this category. They are supposed to induce arch alignment during growth by grinding away acrylic material in definitive areas of the plate, to ensure proper spontaneous development of the segments. The plate is held in position by suction and adhesion only, and no extraoral strapping is applied. The so‐called Zurich approach is the best‐known representative of this kind of neonatal maxillary orthopedic device (Hotz and Gnoinski, 1976).

Many different techniques for infant orthopedics have been described (for an overview, see Monga et al., 2022), and therefore it is difficult to compare treatment results. More importantly, treatment for a cleft patient consists of more than infant orthopedics alone. All treatment steps may have an influence on the final treatment outcome, and it is impossible to separate these steps when comparing results in retrospective research (e.g. Ross, 1987; Mølsted et al., 1992). So far, to our knowledge only two randomized control trials (RCTs) have been performed in this field, one in the Netherlands and one in India (Shetty et al., 2017; Garland et al. 2022). From the six‐year results of the Dutch three‐center trial, named DUTCHCLEFT, it can be concluded that infant orthopedics in children with a complete unilateral CLP (UCLP) with a passive appliance, as performed in this trial, is not necessary for the feeding and nutritional status of the child, parents’ satisfaction (Prahl et al., 2005), facial appearance (Bongaarts et al., 2008), facial growth (Bongaarts et al., 2009), or orthodontic reasons (Bongaarts et al., 2004, 2006). Regarding speech, a positive but very limited effect was found until the age of 2.5 years, but the speech of children with clefts remained far behind that of their noncleft peers anyway (Konst et al., 2000, 2003). Relative to the total costs of the treatment of a UCLP patient, the financial investment to reach this effect was rather limited (Konst et al., 2004). However, it is questionable whether this limited effect is important enough to justify infant orthopedics, while it should also be taken into consideration that the six‐year results for speech have not been analyzed. The last publication on the results of this comprehensive RCT was in 2015 and regarded the transverse dental arch relationship until 12 years of age (Noverraz et al., 2015). No differences between the groups with or without infant orthopedics were found. The second RCT was performed in India and compared presurgical nasoalveolar molding (PNAM) starting at three different time points after birth, which is a different type of infant orthopedics that will be discussed later in the chapter (Shetty et al., 2017).

For children with bilateral clefts, no evidence‐based data from RCTs are available about the effect of infant orthopedics. Based on clinical experience, it can be stated that with the therapy, good alignment of the maxillary segments can be obtained, which facilitates closure of the lip in one operation. An easier approach is preoperative lip taping only, without an intraoral appliance, which could also assist in reducing a prominent or deviated premaxilla and is also occasionally applied in wide unilateral clefts. Steri‐Strips™ (3M, Saint Paul, MN, USA) or specially designed commercially available strips can be used for this purpose.

A special type of infant orthopedics that has emerged during the past three decades is nasoalveolar molding (NAM). There is a distinct difference between the types of infant orthopedics described previously and the one that has been developed by Grayson and Cutting (Grayson et al., 1993; Cutting et al., 1998; Grayson and Cutting, 2001; Barillas et al., 2009). In the latter method, nasal stents are added to the molding plate. This appliance aims to address correction of the nasal deformity; that is, to improve nasal tip projection, and septal and lower lateral cartilage position, before cleft repair (Figure 7.5

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