Presurgical columellar elongation and primary retrograde nasal reconstruction in one-stage bilateral cleft lip and nose repair

We present a new combined approach to primary bilateral cleft lip, nose, and alveolus repair using presurgical nasoalveolar molding combined with a one-stage lip, nose, and alveolus repair. Presurgical alveolar molding is used to bring the protruding premaxilla back into proper alignment with the lateral segments in the maxillary arch. Presurgical nasal molding produces tissue expansion of the short columella and nasal lining. A coordinated surgical approach involves a one-stage repair of the lip, nose, and alveolus. The nasal repair uses a retrograde approach in which the prolabial flap and columella are reflected over the nasal dorsum by continuing the dissection behind the prolabium up the membranous septum and over the septal angle. Tissues are dissected out from between the tip cartilages, and the domes are sutured together in the midline. This method joins a new class of bilateral cleft repairs that place the primary emphasis on correction of the deformity of the nasal tip cartilages.     

Improved treatment of cleft lip and palate is now available

When cleft lip and palate treatment was introduced at Gothenburg in 1957, the procedure used was early bone grafting (EBG). By 1965, EBG had been omitted from the regimen, bone grafting being postponed until the appearance of mixed dentition. Analysis of the results of both techniques showed maxillary retrusion of different degrees. Accordingly, this routine was abandoned in 1975, being replaced by a procedure which is characterised by delayed closure of the hard palate (DCHP). Thus, the surgical procedure comprised the following steps: 1, lip closure at 1-2 months of age; 2, soft palate repair at 6-8 months; 3, final lip-nose surgery at 12 months; and 4, closure of the left in the hard palate, and bone grafting to the alveolar process during mixed dentition at about 8-10 years of age. Follow-up has shown the majority of patients to manifest acceptable speech development during childhood, though problems may occur in some cases. Maxillary growth has been found to be improved after DCHP, and at present the need of maxillary advancement surgery has been reduced to approximately 5% of cases, as compared with the former rates of 50% of cases among those treated with EBG, and of 25% among those treated with the vomer flap procedure.     

Surgical treatment of unilateral alveolar cleft

In this study, 16 cases of unilateral alveolar cleft with cleft lip and palate were repaired with autografts of cancellous bone (13 cases) or hydroxyapatite (3 cases). The grafts were covered by reflected mucoperiosteal flaps and a mucosal flap from the upper lip. Twelve of the thirteen cases were followed up for 1-5 years. Nine of whom using cancellous bone had bony continuity of the maxilla and 7 cases erupted permanent maxillary canines within the area of autografts. None of the 3 cases using hydroxyapatite erupted a canine tooth. The results showed that autograft was better than hydroxyapatite in terms of maxillary canine eruption.     

Effect of lip adhesion on labial height in two-stage repair of unilateral complete cleft lip

A lip-adhesion procedure before definitive repair of unilateral complete cleft lip has been widely used but rarely analyzed over the past 25 years. This report is a quantitative prospective assessment of one possible benefit of lip adhesion, an increased vertical height of the cleft labial elements. Lip adhesion was performed on 43 consecutive infants with unilateral complete cleft of the primary palate. Markings for rotation-advancement repair were placed before lip adhesion and again at the time of the complete nasolabial correction. Using a standardized caliper, anthropometric measurements of the vertical height of the medial and lateral lip elements were made from these markings. The relative increase in vertical height of the medial and lateral labial segments during the mean interoperative interval of 3.2 months was calculated. Analysis of these measurements revealed a small disproportionate increase in vertical lip height of the cleft side compared with the normal side after lip adhesion. Discrepancy of the lateral lip height between the cleft and noncleft sides decreased from 3.0 to 2.7 mm (10 percent, p = 0.05), and the prolabial or medial height discrepancy decreased from 3.4 to 2.8 mm (17 percent, p < 0.01). Subgroup analysis of infants whose adhesion was done either at a young age, without premaxillary orthopedics, or with an intact secondary palate, revealed no statistically significant improvement in the labial height measures. It is arguable whether the small relative increase in height of the medial and lateral cleft elements justifies lip adhesion before definitive repair of unilateral complete cleft lip.     

Current knowledge in cleft lip and palate treatment from an orthodontist's point of view

The aim of this review was to put new clinical research findings into proper perspectives relative to previously accepted knowledge on treatment of patients with cleft lip and palate. The first part of the paper deals with various aspects of infant orthopedic treatment, such as its influence on primary surgery, maxillary arch form and dimensions, feeding, psychological situation of the parents and speech development. Following parts analyze general maxillofacial growth outcome after surgery and also maxillofacial growth in relation to particular surgical procedures (palatal repair, periosteoplasty/gingivoplasty, bone grafting). The last part of the review discuss the effects of certain orthodontic/orthopedic treatment approaches as well as the role of dental implants in treatment of cleft lip and palate patients.     

Axial ultrasonographic imaging of the fetal maxilla for accurate characterization of facial clefts

The purpose of this study was to determine whether scanning of the fetal midface in the axial plane allows accurate characterization of facial clefts. During fetal anatomic survey, facial clefts were identified in six fetuses. The midface anatomy was evaluated with ultrasonography in the coronal and axial planes, and the clefts were characterized prospectively as unilateral or bilateral and as involving the lip alone or both the lip and the palate. The integrity of the upper lip was assessed in the coronal and axial planes. The continuity of the normal C-shaped curve of the tooth-bearing alveolar ridge and the anterior six tooth sockets was assessed in the axial plane. The prospective prenatal diagnosis was correlated with postnatal findings in all cases. The clefts where characterized prospectively as unilateral cleft lip (one case), unilateral cleft lip and cleft palate (four cases), and bilateral cleft lip and cleft palate (one case). The prenatal characterization was confirmed to be correct postnatally in all cases. Prenatal sonographic evaluation of the axial view of the tooth-bearing alveolar ridge of the maxilla allows accurate determination of whether a cleft is confined to the lip or involves both the lip and the palate.     

Development of the residual cleft in the hard palate after velar repair in a 2-stage palatal repair regimen

Delayed closure of the hard palate is believed to improve maxillary growth and facial appearance in cleft lip and palate patients. However, the cleft opening in the hard palate after velar closure might impair speech development. The aim of this investigation was to study the development of the residual cleft in the hard palate after 2-stage palatal repair (TSPR) in children born with complete cleft lip and palate (bilateral [BCLP]; n = 7 or unilateral [UCLP]; n = 22) or isolated cleft palate (CP; n = 9). Moreover, we aimed to investigate whether any morphologic factors before surgery might predict development of the residual cleft. Dental casts obtained prior to velar repair (mean age 7 months) and postoperatively at 1 1/2, 3, 4, 5 and 7 years were analyzed with a Reflex Microscope regarding the width, length and area of the cleft in the hard palate. The palatal cleft varied in size both pre- and postoperatively in all 3 types of cleft patients. The width of the cleft in the UCLP subgroup showed a marked reduction immediately after velar repair, but then, on average, remained stable until final surgical closure of the hard palate. In the BCLP subgroup the initially rather narrow width of the clefts remained unchanged postoperatively. Clefts in the CP subgroup, especially in those with a complete cleft, remained large after veloplasty. In 4 of the UCLP and 2 of the BCLP patients, the cleft width increased gradually. In some other subjects, both in the UCLP and BCLP subgroups, the residual cleft closed functionally with time, but this development could not be foreseen.     

A new principle for unilateral complete cleft lip repair, the lateral columellar flap method

This article presents a different method for unilateral complete cleft lip repair. The tissue deficiency of the medial lip segment is filled with a flap from the lateral surface of the columella, which is in continuity with the lip segment. The lateral columellar flap and the medial lip segment are lowered in one piece vertically downward to such a position that the height of the arch of the Cupid's bow is equal on both sides. No lateral advancement is done. The suture line of the medial and lateral lip segments is an uninterrupted straight line, which imitates the natural line of the philtral column. The repaired lip has more fullness than the lips repaired with the conventional methods. The procedure is described in detail.     

Plasma integrated concentration of growth hormone after recombinant human growth hormone injection. Implications for determining an optimal dose

The length of the cervical spine in a series of 206 adult males with cleft lip and/or palate and 50 normal controls was measured. The patients were divided into five subgroups according to the type and extent of the cleft. The shortening of the spine was most marked in bilateral cleft lip and palate patients (complete), less marked in unilateral cleft lip and palate patients, and was slight in isolated cleft palate patients. Complete isolated cleft palate and cleft lip was not associated with a shortening of the spine. A shortening of the cervical spine in less extensive types of isolated cleft palate was suggestive of the participation of the spine in their development, while in cleft lip and palate a simultaneous exposure to a teratogenic agent or any other developmental error during early stages of embryogenesis could explain the concomitant occurrence of spine anomalies. Patients with cleft lip and palate associated with a short spine also had a shorter mandibular ramus, which could be suggestive of simultaneous damage to both structures during morphogenesis. This relationship was not demonstrated in isolated cleft palate that developed in later stages of embryogenesis. In these cases a short spine itself could not have impaired the growth potential of the mandible, yet it could have mechanically induced the development of cleft palate. These observations are in agreement with the present state of knowledge on the development of orofacial clefts as shown in experimental animals.     

Review: the Michigan cleft twin study

The combined effects of clefting of the lip and/or palate and of their surgical repair have been examined for a cross-sectional sample of 20 MZ and 25 DZ like-sexed twins discordant for clefting, in the age range of 4 to 17 years. The findings of four studies published since 1975 on that sample are explored for the primary, contiguous and pleiotropic effects of clefting. The primary effects on facial structures of repaired cleft lip appear to be minimal. However, for twins with repaired cleft of the palate (with or without cleft of the lip), the maxilla was both deficient antero-posteriorly and was positioned more posteriorly than in their non-cleft co-twins. The maxillary first molars were usually slightly less erupted in the cleft twins than in the non-cleft twins. The cleft palate only twins had larger cranial base angles than their non-cleft co-twins. A contiguous effect of the posteriorly positioned maxilla was a mandibular rotation downward and backward in the cleft palate and bilateral cleft lip and palate groups but not in the unilateral cleft lip and palate group. The slightly larger cranial base angles found for cleft palate only, although contiguous in location, are more likely pleiotropic effect. The pleiotropic effects include a deficit in height and weight for cleft subjects which appears only after puberty. Although the teeth of the cleft twins tended to be smaller than those of the non-cleft twins, the amount was not clinically significant.     

Alveolar bone grafting in unilateral complete cleft lip and palate patients

Autogenous bone graft of an alveolar cleft area has the following advantages: (1) assistance in the closure of buccoalveolar oronasal fistula; (2) provision of bony support for unerupted teeth and teeth adjacent to the cleft; (3) formation of a continuous alveolar ridge to facilitate orthodontic correction of malocclusion; (4) supporting the nostril floor and alar base to improve nasal aesthetics. It has been well accepted in most craniofacial centers as routine procedure in cleft lip and palate rehabilitation. A new surgical technique for alveolar bone grafting has been introduced to the Chang Gung Craniofacial Center since July 1991. It provided a good exposure of the alveolar cleft, primary closure of the fistula and adequate volume of bone graft. A review of 27 consecutive alveolar bone grafting procedures performed in unilateral cleft lip and palate patients from July 1991 to June 1992 was presented. Patients have been followed up for at least 6 months. The alveolar bone graft was evaluated clinically and radiologically at one week, six months and one year after the surgery. The preliminary results indicated that the new surgical technique produced less chance of recurrent fistula, good postoperative gingival height, and improvement of nasal aesthetics. Based on the results of this new study we strongly advocate the use of this new surgical technique.     

Endosseous implants for prosthetic rehabilitation in bone grafted alveolar clefts

The authors report their experience in the surgical and prosthetic rehabilitation of three patients affected by sequelae of cleft lip and palate, with residual alveolar cleft and absence of maxillary anterior teeth. The patients were treated by means of late secondary bone grafting of the alveolar cleft, followed by the insertion of endosseous titanium plasma-sprayed implants (IMZ). After a further healing period (6-12 months) fixed dental prostheses were constructed. Preliminary results from this series have shown how dental prostheses supported by endosseous implants in grafted alveolar clefts are a very reliable possibility in dental rehabilitation of this malformation.     

Repair of unilateral cleft lip: a comparison of five techniques

Repair of unilateral cleft lip is a challenging procedure with no single technique satisfactory for all types of unilateral cleft deformity. This study compares retrospectively five techniques of unilateral cleft lip repair in 72 children (45 boys, 27 girls). Twenty-two children had a Millard rotation-advancement repair, 5 children had a Davies Z-plasty, 22 had a modified Z-plasty, 10 had a Tennison-Randall triangular flap repair, and 13 had a Nakajima-Yoshimura straight-line repair. Assessment was performed clinically by a plastic surgeon not involved in the original surgery. Repairs were assessed objectively by measurement of the vertical length of both the repaired and normal sides of the lip with calipers. Subjective criteria used to evaluate the repair were the symmetry of Cupid's bow, the quality of scar, the alignment of white roll, the evenness of the vermilion, and the lip pout. The mean follow-up period was 5.4 years (range, 9 months-29 years). Sixty-five of the 72 repairs measured (90%) were within two standard deviations (SDs) of normal and thus were considered to be of "acceptable" length. Seven repairs were unacceptably short (>2 SDs) on measurement, six of which included a rotation-advancement repair for a complete cleft lip. Subjective results paralleled the objective results. The outcome following repair of unilateral cleft lip was similar for all five surgical methods assessed except for complete cleft lips repaired by the rotation-advancement technique, which tended to result in an unacceptably short lip as measured on the repaired side.     

Long-term assessment of early alveolar bone grafts using three-dimensional computer-assisted tomography: a pilot study

Fifteen patients with complete unilateral cleft lip and palate who had primary alveolar bone grafting were studied with computer-assisted tomography at a mean age of 12 years. Keeping the maxillary alveolar crest parallel to the plane of the scan, 1.5-mm cuts of the maxilla were made from the infraorbital rim to the gingival third of the crowns of the teeth. A single operator reformatted the data into three-dimensional images using the Maxiview 3200 computer workstation. This allowed examination of the position, size, and spatial relationship of the grafted area and quantification of the amount of bone coverage of root surface and bone height of the alveolus in or adjacent to the graft site. Ten patients showed a lateral incisor in the line of the cleft. The average bony coverage of these tooth roots was 76.5 percent. In the five patients in whom there was lateral incisor agenesis, the canine root had average bony coverage of 82.6 percent. The average height of bone at the lateral incisor was 8.7 mm; at the canine, 14.1 mm. In two patients in whom there was only 42 percent tooth root coverage, the teeth were still viable, stable, and without mobility. Computed tomographic (CT) scans of the 15 patients demonstrated good graft survival with adequate volume. The functional and aesthetic status of the dentition in the area of the cleft also was demonstrated.     

Oro-nasal fistula development and velopharyngeal insufficiency following primary cleft palate surgery—an audit of 148 children born between 1985 and 1997

We present an audit of primary cleft palate surgery in our unit including rates of two important post-operative complications.Multidisciplinary audit clinics ran from March 1998 to April 2002 to follow up all local patients with a cleft lip or palate who had undergone primary palatal surgery in our unit. One hundred and forty eight patients were studied. Patient ages at follow-up ranged from 3 years and 10 months to 17 years and 4 months. Two surgeons performed the primary surgery. One hundred and twenty eight Wardill-Kilner and 20 Von Langenbeck repairs were performed.We found a 4.7% rate of oro-nasal fistula development requiring surgical closure, and a 26.4% rate of velopharyngeal insufficiency (VPI) requiring subsequent pharyngoplasty. We noted that the type of cleft involved affected the rate of VPI, 16% of patients with unilateral cleft lip and palate versus 29.2% of patients with a solitary cleft palate requiring secondary surgery.Outcome of surgery was determined by a ‘Cleft Audit Protocol for Speech’ (CAPS) speech therapy assessment at follow-up clinics. Only 14.9% of all patients assessed demonstrated any degree of hypernasality.Our results compare favourably with other recent studies including the Clinical Standards Advisory Group (CSAG) report into treatment of children with cleft lip and palate.     

Maxillary distraction: aesthetic and functional benefits in cleft lip-palate and prognathic patients during mixed dentition

In the last few years, distraction techniques have been used successfully to correct the hypoplastic human mandible. In patients with cleft lip and palate, normal growth of the maxilla may be impaired by early cleft repair, and many of them do not respond to orthodontic procedures alone. Maxillary distraction is an alternative technique to correct maxillary hypoplasia during mixed dentition. In the last 3 years, the procedure was performed in 38 patients aged between 6 and 12 years; 18 patients had unilateral cleft lip and palate, 9 patients had bilateral cleft lip and palate, 7 patients had unilateral cleft palate, 2 patients had prognathism, and 2 patients had nasomaxillary dysplasia. Photographs, posteroanterior and lateral cephalograms, and dental models are obtained preoperatively (as well as an orthopantomogram) to locate the tooth buds. A subperiosteal dissection is performed exposing the anterior and lateral aspects of the maxilla, and an incomplete horizontal osteotomy is done above the tooth buds. Using a facial mask and an intraoral fixed appliance system as an anchorage, we initiate on the fifth postoperative day the application of distraction forces. Maxillary advancement between 4 and 12 mm is achieved during 3 to 4 weeks, and a satisfactory class I or II molar relationship is also obtained. A combination of forward and downward distraction forces can be used to achieve simultaneous advancement and elongation of the hypoplasic maxilla. The aesthetic results are excellent, and the nasolabial angle is increased, including a more anterior projection of the upper lip. Nasal breathing is improved as well as the air flow and patency of the nasal airway. Velopharyngeal function remains unchanged after the procedure. The follow-up in this series varied from 6 months to 3 years. No relapses have been observed.     

One-stage closure of uni- and bilateral cleft lip and palate

A preliminary report of an "all-in-one' one-staged closure of all forms of cleft lip and palate during the first year of life. The one-stage repair of complete uni- and bilateral clefts includes the anatomical reconstruction of soft palate, hard palate closure in two layers, alveoloplasty with bone grafting and lip repair. This surgical technique is described and early results presented.     

Deficient and delayed primary palatal fusion and mesenchymal bridge formation in cleft lip-liable strains of mice

During mammalian primary palate formation, the facial prominences enlarge around the nasal pit, fuse and then merge to give rise to the tissue of the upper lip and premaxillary region. The mechanisms involved in successful primary palate formation and how they are affected in the cleft lip genotype remain poorly understood. The purpose of this study was to compare morphometrically internal development and growth of the primary palate in five different strains of mice. Two of the strains, BALB/cByJ, and C57BL/6J, have normal primary palate development, and three of the strains, A/J, A/WySn, and CL/Fr, have stable frequencies of cleft lip associated with genotype. In the present study, frequencies of 4, 23, and 24%, respectively, were observed on day 13. For palatal growth analysis, embryos were collected on days 10 and 11, staged by number of tail somites (TS), and the heads were photographed and serially sectioned for measurement of primary palate components. The heights of the epithelial seam and the mesenchyme bridge between the facial prominences were measured on serial sections and areas of contact were calculated. The position or depth of the maxillary prominence was determined from the number of frontal sections from its tip to the rostral end of the nasal fin. Analysis of measurements showed that in cleft lip strains enlargement of the epithelial seam and replacement of epithelia by a mesenchymal bridge were both delayed relative to somite stages. Measurements from day 11 embryos with complete failure of contact were excluded from the growth analyses. The mesenchymal bridge formed at 12--13 TS in noncleft strains, 14 TS in the A/J strains with higher cleft lip frequency, and 15--17 TS in A/WySn and CL/Fr strains with higher cleft lip frequency. Forward growth of the maxillary prominence was highly correlated with the primary palate measurements and mesenchymal bridge formation in all strains. In both cleft and noncleft strains, the primitive choanae open at 18--20 TS and the medial nasal region narrows with advancing embryonic development. As a result, cleft lip-liable strains have a narrower window in development in which a robust mesenchymal bridge must form, thus increasing the liability to cleft lip.     

Robin sequence: obstructive sleep apnea following pharyngeal flap

OBJECTIVE: We reviewed 24 children with Robin sequence who underwent cleft palate repair. METHOD: All patients were 5 years of age or older at the time of review, allowing for accurate assessment of speech in relation to velopharyngeal function. All infants had palatal closure between 9 and 14 months of age, either V-Y repair (n = 16) or von Langenbeck repair (n = 8). RESULTS: Only 1 of 16 children who had V-Y repair had borderline velopharyngeal dysfunction (VPD). For reasons that are unclear, in the von Langenbeck repair group, six of eight children had VPD, and four of six underwent pharyngeal flap. Three additional patients with nonsyndromic Robin sequence had palatoplasty and subsequent pharyngeal flap. Six of the combined total of seven children with nonsyndromic Robin sequence developed obstructive sleep apnea and require flap take-down. CONCLUSION: Since conventional pharyngeal flap for VPD in nonsyndromic Robin sequence children resulted in a high incidence of obstructive sleep apnea, alternative management should be considered: modification of the standard pharyngeal flap, palatal lengthening (V-Y or double-opposing Z-plasty), or construction of a speech bulb.     

Two sibs with cleft palate, ankyloblepharon, alveolar synechiae, and ectodermal defects: a new recessive syndrome?

Hay and Wells in 1976 reported seven patients from four families who had an inherited condition of which the main features were ankyloblepharon, ectodermal defects, and cleft lip and palate. The inheritance pattern was determined to be autosomal dominant. This condition is known as AEC syndrome or Hay-Wells syndrome. We report a family with two sibs showing some of these features and congenital adhesions between the upper and lower jaws (alveolar synechiae). There seems to be a recessive pattern of inheritance as neither of the parents has any features of the syndrome. This could be described as a recessive form of Hay-Wells syndrome with additional features or be named as a new syndrome.