To avoid problems with a short and tight soft palate, surgeons began realizing that velum had to be released from the posterior hard palate (Braithwaite and Maurice 1968). This idea was supported by studies of Kriens (1970), showing that if there is a cleft of the soft palate, the velar muscular complex on both sides is running in an abnormal, anterior-posterior (A-P) direction. Therefore, it seemed logical, not only to detach but also to redirect those muscles to their normal transverse direction. To get access to the attachment of the velar muscles, mucosal/mucoperiosteal flaps were dissected from various positions at or within the posterior hard palate. The muscles were then cut from the palatal shelves, reoriented to a transverse course, and could be joined in the midline in a more posterior position than before. The procedure would be enhanced by addition of a posterior vomer flap (Malek and Psaume 1983), which was sutured to the nasal mucosa of the anterior soft palate (Fig. 18.2). With anterior velum attached to the lower edge of the posterior nasal septum, the soft palate was lifted up to the level of the palatal shelves, which, during healing, helped reduce the size of the remaining cleft of the hard palate.

Regarding HPR, Schweckendiek (1955, 1978) did not suggest any particular method for this surgery and only mentioned that, preferably, it should be delayed until around puberty, i.e., when most of maxillary growth was completed. With such late repair of the residual cleft, the chosen method was not as crucial, as if this surgery had been performed at an early age. When later on some surgeons elected to close the remaining cleft already during development of the primary dentition, or sometimes even earlier, different repair methods were utilized. Without any direction from the originators of the two-stage protocol, most surgeons chose to use the same surgical method at HPR, as they were accustomed to in a one-stage palatal procedure. Examples of methods varied from use of uni- or bilateral mucoperiosteal flaps according to methods of Veau, Wardill-Kilner, von Langenbeck, Delaire, or others. Particularly, after use of methods where extensive mucoperiosteal flaps were shifted medially to cover the palatal cleft, areas of bone in the hard palate were left denuded. Growth-restricting palatal scars would then develop, which, depending on the position and size of these scars, had a varying negative effect on maxillary development.

With delay of HPR, the residual cleft usually would narrow considerably (Owman-Moll et al. 1998), which the surgeon should have taken advantage of. The reduced width of the remaining cleft would, in many cases, allow primary repair of the residual opening in the hard palate after mobilization of the cleft edges without leaving any palatal bone denuded. In wider residual clefts, the repair could be accomplished with a turnover vomer flap or by use of bilateral flaps taken from the thin palatal mucoperiosteum close to midline. According to Delaire, inclusion of the thick palatal mucoperiosteum more laterally would cause bare bone in areas with increased risk for development of growth impairing scars (Markus et al. 1993). If the remaining cleft in the hard palate was very wide, it was suggested to postpone HPR until age 2 or 3 years. These surgical details were decisive factors for maxillary development during subsequent growth.

Timing of repair of the two palatal procedures has also been characterized by great variation. Reports from literature have suggested ages varying from 3 to 24 months for SPR, and for HPR, different papers have proposed ages ranging from 6 months to 16 years. The timing preferences of the surgeon and the cleft team most often have been guided by subjective estimations of how the operations might affect speech and/or maxillary growth outcome.

Speech development, sometimes appraised before HPR, was judged as inferior to what was expected. These young children had significantly poorer articulation skills than their noncleft peers. Posterior substitutions had often developed and so had frequent VPI (Cosman and Falk 1980; Jackson et al. 1983; Noordhoff et al. 1987). From a methodological point of view, it has to be remembered that these evaluations were clinical, live judgements with no possibility for control of the data. If we believe that, even so, these early assessments were valuable, we suspect that many of the speech errors might have had their origin in missing important surgical details at closure of the soft palate. For instance, one of the papers stated, “the soft palate was closed directly with only minimal division of nasal mucosa and palatine aponeurotic fibers” (Cosman and Falk 1980). With no definite separation between the soft and hard palate, we suspect the surgeon had been unable to bring back the repaired velum to a position needed for achievement of velopharyngeal competence (VPC) on a regular basis. In other studies (Noordhoff et al. 1987), it was mentioned that SPR had been performed according to the original method of Perko (1979), but nothing was reported about use of a posteriorly based vomer flap. Omission of this crucial surgical step is likely to have caused reduced velar length in many cases and also a wider residual cleft in the hard palate due to less narrowing during early palatal growth. Tentatively, the short soft palate would increase the risk for VPI and posterior substitutions, such as pharyngeal and/or glottal articulations. In the report by Noordhoff et al. (1987), all patients treated with the two-stage method were said to have increased articulation errors, particularly those individuals with wide remaining cleft of the hard palate. A later follow-up paper (Liao et al. 2010) confirmed increased hypernasality and compensatory articulation disorders in these patients.

More recently, a number of teams have, in particular, reported the patients’ maxillary growth outcome with limited focus on their speech development. The majority of the papers described favorable midfacial growth (Noverraz et al. 1993; Tanino et al. 1997; Nollet et al. 2005, 2008; Sinko et al. 2008; Liao et al. 2010), while a few of the reports did not find any maxillary growth advantage of the two-stage protocol (Gaggl et al. 2003; Mølsted et al. 2005; Holland et al. 2007; Stein et al. 2007). The first group of papers generally advocated methods for SPR and especially for HPR, where closure of the cleft resulted in minimal denudation of bone in the palate. Examples of such procedures comprised employment of a turnover vomer flap, suturing of the cleft edges, mobilization of mucoperiosteal flaps dissected close to the cleft, or use of a modified von Langenbeck operation. On the other hand, in studies describing no maxillary growth benefits or inferior maxillary development, the methods used in these patients had created growth-restricting scars, mostly from HPR. Examples of surgical methods included employment of a Veau pedicle flap, a mucoperiosteal pushback procedure, or use of “unipedicled mucoperiosteal flaps.” All of them will give rise to significant areas of bare palatal bone, which will heal secondarily, leading to scar tissue development. Thus, these different opinions about definite growth advantage or insufficient maxillary development after the two-stage palatal protocol can be explained by the performed repair methods and, only to a limited extent, by when surgery was done, as described above. Such explanations are easier to embrace than speculation about effects, e.g., from cleft team organization (Shaw et al. 1992a, b) or surgeons’ different operating skills (Ross 1987a, b), as reasons for the patients’ growth results. However, we have to remember that acceptance or rejection of the two-stage regimen might be influenced not only by how well the repaired maxilla will develop but also by other factors, such as increased risk for fistula formation, poor speech development, and need for more velopharyngeal flaps (VPFs); etc.

In Zürich, Switzerland, the cleft team has used their version of the two-stage palatal repair protocol since the late1960s. It was built on a systematic coordination between maxillary orthopedics and surgical interventions from early infancy (Hotz and Gnoinski 1976; Hotz et al. 1986). The team initiated preoperative maxillary orthopedic treatment shortly after birth, not only to help feeding but also for growth guidance of the maxillary segments. After lip repair at age 5–6 months, the infant continued with maxillary orthopedics until SPR, which was carried out at around 18 months. This surgery included reorientation of the velar muscles after their separation from the posterior hard palate (Perko 1979). The covering flaps of the oral mucosa were dissected from the posterior third of the hard palate, and, to reduce risks for maxillary growth impairment, the dissections were made supraperiosteally. A posterior vomer flap was also raised, and after turning it backward, the flap was sutured as part of the anterior nasal layer at SPR (Hotz et al. 1986). With the nasal mucosa not separated from the posterior hard palate, inclusion of the vomer flap would not help elongate the soft palate, and therefore, a midline Z-plasty was added. At age 5–6 years, HPR was performed. Due to narrowing of the residual cleft, closure was accomplished by use of a turnover vomer flap for the nasal layer, and for repair of the oral layer, a mucoperiosteal flap was shifted medially from the noncleft side.

In 1996, our cleft team published a detailed report how the two-stage protocol was practiced in Gothenburg up to 1995 (Lilja et al. 1996). At soft palate surgery, incisions began around the posterior part of the maxillary tuberosities and then followed a zigzag route at the posterior border of the hard palate (Fig. 18.5). A posteriorly based vomer flap was dissected. Anteriorly, the incision was placed behind the vomero-premaxillary suture, and the flap had its base close to the junction between vomer and the cranial base. Mucosal flaps in the soft palate were raised by blunt dissection. Hamulus was identified but not broken. The insertions of velar muscles, including their attached nasal mucosa, were cut at the posterior border of the hard palate. A flap with the muscles connected to the nasal layer was then dissected free and mobilized to a posterior position. After that, the muscles including the levator were reconstructed to a transverse course, where suturing could be performed without tension. The vomer flap was raised and the nasal layer of velum was closed anteriorly to the level of the muscular sling by use of the vomer flap. In this way, the vomer bone became connected to the anterior velum. The palatal closure was then continued over to the oral side, where a pushback procedure was performed within the oral layer of the soft palate. Inclusion of the vomer flap as well as the pushback surgery helped to increase the length of the repaired velum.

The HPR was delayed until the patient had reached the stage of early mixed dentition (7–9 years), because, thereby, the procedure could be combined with bone grafting to the alveolar cleft. Surgery began with gingival incisions along the neck of the teeth on the palatal side and in part also labially (Fig. 18.6). In the area of the cleft, incisions were made along the cleft edges, and gingival and palatal mucoperiosteal flaps were raised. On the labial side, a back-cut was made in the cleft-side molar area. This facilitated mobilization of the gingival flap as well as the dissection in the cleft area. When the cleft had been dissected completely free on both palatal and labial side, suturing in the ­midline began in the nasal layer and continued orally in the midline palate. Cancellous bone was then grafted to the cleft in the alveolar process and covered with gingival and palatal flaps. The operation was completed with suturing the gingival and palatal mucoperiosteal flaps together in some interdental spaces. We consider the palatal incisions along the necks of the teeth very important. Because of the reduced width of the residual cleft, suturing back of the combined palatal flaps along the dental arch could be done with none or only minimal palatal bone exposed close to the teeth. In 1996, timing for HPR was modified in an effort to prevent development of typical retracted oral speech deviations noted in some patients during preschool and early school age. The repair was then changed to be carried out around 3 years, which meant an extra operation, because HPR could no longer be performed together with the bone grafting procedure. If the residual cleft of the hard palate was narrow or of average size, it was closed in one layer by use of a simple turnover vomer flap (Fig. 18.7). This approach did not work so well for wider residual clefts of the hard palate due to increased risk for fistula development. In these cases, a two-layer repair was necessary. A vomer flap was then sutured to the nasal mucosa on the cleft side, and the suture line was closed with a mucoperiosteal flap from the cleft side of the palate (Fig. 18.8).

Over the years, the Gothenburg cleft team has reported several follow-up studies, both regarding maxillary growth and speech development. An early growth comparison at age 7 years, before HPR in the two-stage protocol, demonstrated significantly improved results in comparison to what was achieved with our previous regimen (Friede et al. 1987