Upper lip measurements at the time of surgery and follow-up after modified rotation-advancement flap repair in unilateral cleft lip patients

The purpose of this prospective study was to determine whether unilateral cleft lip repaired by the rotation-advancement flap will grow short on the repaired side. This study involved 56 patients with nonsyndromic unilateral cleft lip (31 with complete and 25 with incomplete cleft lip) who underwent a rotation-advancement flap repair by a single surgeon between 1989 and 1997. Eleven patients were lost to follow-up. Forty-five patients have been followed for a varying period of between 8 and 84 months (mean = 37 months). The upper lip was measured immediately after the lip repair and follow-up using calipers. The growth ratios of vertical, horizontal, and nostril sill dimensions were compared between the cleft side and the noncleft side of the same face. Statistical analysis was performed to compare the growths between the cleft and noncleft sides. There was not a significant difference in the growth ratios of vertical (Wilcoxon signed rank test, p = 0.85) and horizontal dimensions (Student's t test, p = 0.18) between the cleft and noncleft sides. There was, however, a statistically significant difference in the growth ratios of nostril sill width between the cleft and noncleft sides (Student's t test, p = 0.02). Our findings indicated that a repaired unilateral cleft retained the vertical and horizontal dimensions determined at the time of the initial repair.     

Experience with the functional cleft lip repair

The first 12 functional cleft lip repairs performed on unselected consecutive patients immediately following the completion of training by the author are presented. Previous reports on this cleft lip repair have shown excellent results but have always been based on patients operated on by the originator of the procedure. This report gives credence to the ease with which a cleft lip repair that gives reproducible good results can be taught and learned even by plastic surgeons with limited experience. It reviews the technical steps of the procedure, which emphasizes wide undermining and release of the orbicularis oris muscle on the lateral side of the cleft to allow redraping and lengthening of the lip skin, step-by-step layered closure of the mucosa, muscle, and skin, and further vertical lengthening of the lip with a Z-plasty skin closure. Three elements that are difficult to achieve or restore with cleft lip revision are evaluated: (1) achievement of a good skin scar, (2) maintenance of the alar-facial groove, and (3) achievement of adequate lip height without sacrificing horizontal lip length. Ten of the 12 patients had a satisfactory scar, 9 patients had a good alar-facial groove, and all patients had a normal-appearing horizontal lip length. Nine patients required secondary surgery; however, in six patients, this included correction of the nasal deformity that was not corrected at the time of cleft lip repair.     

A long-term, controlled-outcome analysis of in utero versus neonatal cleft lip repair using an ovine model.

Successful open repair of a cleft lip in utero has the advantage of scarless wound healing in the fetus. Unfortunately, no long-term outcome studies have been performed to evaluate the efficacy of these repairs. Moreover, no study to date has compared the long-term results of an in utero cleft lip repair to a similar, control-matched, newborn cleft repair. This study was performed to evaluate the 9-month outcome of in utero cleft lip surgery compared with an identical cleft lip repair performed on infant lambs. In utero epithelialized cleft lips were created through an open hysterotomy in sixteen 65-day-old fetal lambs (term = 140 days) using methods described by Longaker et al. Eight of 16 animals underwent subsequent in utero repair of these clefts at 90 days gestational age. The repair of the remaining eight animals was delayed until 1 week postpartum. At 9 months, the animals were analyzed for changes in lip contour and for the degree of scarring by hematoxylin and eosin and Masson's trichrome collagen staining. Two animals in each group died from preterm labor. Of the animals that survived to term, all repaired lips had some degree of abnormality postoperatively. One of six lips repaired in utero dehisced before delivery. Three of six neonatal repairs dehisced in the first postoperative month. In the remaining animals with intact lip repairs, the vertical lip height on the repaired side was an average of 9 to 12 mm shorter than the normal lip in both the in utero and neonatally repaired animals. Phenotypically, the postnatally repaired animals had more lip distortion and visible notching. Histologically, the in utero repair was scarless and the neonatal repairs had scar throughout the entire vertical height of the lip with an associated loss of hair in this region. Maxillary growth was also evaluated. There was no inhibition of maxillary growth in the animals that underwent in utero cleft lip repair. However, in the neonatal repair group, significant maxillary retrusion was evident. Compared with the cleft side of the maxilla, horizontal growth was decreased by 11 percent (p = 0.01). Compared with the intrauterine repair group, there was a 17-percent decrease in horizontal maxillary width (p = 0.01). Straight-line in utero repair of a cleft lip produces a better long-term result in terms of maxillary growth than a similar repair performed postnatally in the ovine model. There was no diminution in maxillary growth in the animals treated in utero. Histologically, in utero repair of clefts was indeed scarless. However, both lip repairs produced lips that were significantly shorter than their contralateral noncleft sides. This degree of lip shortening would require a secondary lip revision, thereby defeating the purpose of performing an intrauterine repair. Comparisons now need to be made between in utero and neonatal repairs using a Millard-type rotation advancement technique before intrauterine treatment can be considered to be more beneficial than our current treatment modalities.     

The Tennison Lip repair revisited

Tennison presented his method for the repair of the single cleft. He was the first to recognize and to preserve the cupid's bow by lowering the peak in the margin of the cleft. He incised the medial side of the cleft and filled the space with a triangular flap from the lateral side. We have modified the Tennison repair based on the vertical height of the normal side, as did Randall and Hagerty. We make the lip 1 mm shorter in the vertical height than the normal side because some of our repairs were too long. Other modifications include a 1-mm offset at the vermilion, and in certain lips that are too long in the newborn, a triangle is excised beneath the alar base to shorten the vertical height. The details for planning the incisions and accomplishing the surgery are given. Certain patients required a V-to-Y procedure to augment a vermilion deficiency, but none of these patients required a secondary procedure. This emphasizes the need for careful planning to get it right at the primary repair. The operation is indicated for the incomplete cleft to the very wide cleft, and in no patient was a lip adhesion required. We no longer operate on the nose at the primary repair.     

Muscle reconstruction in cleft lip repair

This article presents a method of muscle reconstruction in the cleft lip patient that grossly reproduces the interlacing muscle anatomy in the central portion of the upper lip and thereby addresses both the static and the functional requirements of cleft lip repair. This lip repair procedure is combined with paranasal muscle reconstruction, preoperative nasoalveolar molding, and postoperative nasal stenting to constitute a comprehensive approach to cleft lip/nose repair.     

A comparative study of the skin envelope of the unilateral cleft lip nose subsequent to rotation-advancement and triangular flap lip repairs

The secondary nasal skin envelope asymmetries were studied after unilateral cleft lip repair using the original (obsolete) rotation-advancement (Millard I) and the triangular flap techniques (Bardach's modification). Secondary correction of the nasal deformity was not performed in either group. Our findings indicated that in both groups, vertical asymmetries of the nasal skin envelope were similar. The alar dome on the cleft side was depressed, the columella was shorter on the cleft side, and there was hooding at the nostril apex. The principal difference between the two lip repairs was observed in the horizontal dimension of the nasal skin envelope. The position of the alar base was more normal following the Millard I repair, while the triangular flap repair left the alar base laterally displaced. When considered together with flattening of the cleft alar dome, a horizontal skin-envelope deficiency from middome to lateral alar crease was produced in the Millard I group. More lateral positioning of the alar base after the triangular flap technique minimized this horizontal skin deficiency. The triangular flap technique produced a secondary nasal deformity that looked worse but was easier to correct. The clinical implications of these findings are discussed.     

A model for fetal cleft lip repair in lambs.

Fetal wounds heal without inflammation and scar formation. This phenomenon may, in the future, be applicable to human cleft lip and palate repair. However, extensive experimental work must first be done to document the benefits of in utero repair. We developed a large animal model for creation and repair of a complete cleft lip and alveolus using fetal lambs. The cleft lip and alveolus deformity was created in eight 75-day-gestation fetuses (term = 145 days) and either repaired in three layers or left unrepaired. There were four sham-operated fetuses, and all animals were alive at harvest. Repaired, unrepaired, and control fetuses were harvested at 7, 14, 21, and 70 days following surgery. The unrepaired fetuses demonstrated a complete cleft lip and alveolus with an oronasal fistula. The maxilla was asymmetrical, with the greater segment deviated toward the cleft and with decreased anterior maxillary width. In contrast, repaired cleft lip and alveolus animals showed no scar, normal thickness of the lip, and a symmetrical maxilla. Histologic analysis of the repaired wounds showed evidence of tissue regeneration without scar formation. The results of this preliminary study indicate that the fetal lamb cleft lip and alveolus model is technically feasible with an excellent survival rate. Healing occurs without scar formation. In the repaired animals, the maxilla was symmetrical. This model will be used to document facial growth following in utero repair of a cleft lip and alveolus.     

Growth of the cleft lip following a triangular flap repair

This study involves 50 children who had a triangular flap repair for unilateral cleft lip. They have been followed for 5 to 14 years by serial measurements, photographs, and clinical evaluation. During the first 5 years, the lips were designed to allow for future growth. The expected growth did not occur. During the second 5 years, the lip length was designed equal to the normal side with the belief that unequal growth does not occur. In this group, the results supported the premise that deviation from the correct lip length would now be equally divided between too long and too short. None of the entire group has a lip which started too short becoming equal or starting equal to become too long. It is the conclusion of the authors that a repaired unilateral cleft lip retains the configuration and length determined at the time of the initial repair.     

Unilateral cleft lip repair

The marking of the medial lip segment of the Millard rotation advancement procedure for repair of the unilateral cleft lip has been altered in the uppermost portion by utilizing tissue from the columellar base. Once adequate length has been obtained, cutback is utilized at approximately 90 degrees. With adequate full-thickness release of this medial lip segment and subsequent rotation into the proper position, the C flap is advanced into the donor defect of the columellar base and is also used to lengthen the shortened columella on the cleft side. This results in placement of a scar that will closely simulate the "mirror image" of the noninvolved philtral column. Fifty-seven patients with unilateral cleft lip have been repaired utilizing this technique during the past 14 years. Several of these children have required secondary surgeries because of mucosal irregularities or residual nasal deformities, but none has required additional surgery because of inadequate rotation of the medial lip segment or for correction of any donor-site defect at the base of the columella.     

Lip height and lip width after extended Mohler unilateral cleft lip repair

The purpose of this study was to evaluate the symmetry in lip height and lip width after extended Mohler unilateral cleft lip repair, with long-term follow-up monitoring. In the Mohler repair procedure, Millard's C-flap is used to fill the entire defect created by the downward rotation of the medial lip element. Because a lateral advancement flap is not transposed into this defect, Mohler repair is often expected to produce a short lip. In a retrospective study of 120 patients, anthropometric measurements were made on black-and-white photographs. Of those patients, 49 met the study criterion of having a set of photographs taken 13 months or less postoperatively and another set taken at least 2 years postoperatively. The distance from the Cupid's bow peak to a line tangent to the base of the columella (lip height) and the distance from the Cupid's bow peak to the ipsilateral commissure (lip width) were measured with a Vernier caliper. The medial intercanthal distance was also measured, for standardization of all measurements. All values were normalized to the mean intercanthal distance at age 6, as reported by Farkas. Matched-pair test analyses were used to assess the statistical significance of differences in cleft-side versus non-cleft-side measurements for each group, as well as changes with time. No statistically significant difference in cleft side versus non-cleft-side lip height for the two groups or with time was observed (< or =13 months, p = 0.28; >2 years, p = 0.08; change with time, p = 0.69). Statistically significant differences in lip width between the cleft side and the non-cleft side were observed for both time groups. The average difference in lip width at 1 to 13 months was 8.6 percent (p < 0.001). The average difference in lip width at 2 years or more postoperatively was 5.8 percent (p < 0.001). In comparisons of early versus late measurements, it was noted that lip width significantly increased with time (mean, 0.91 mm; p = 0.035). The findings suggest that extended Mohler repair does not produce a short lip. Interestingly, lip width was observed to be significantly smaller on the cleft side in the immediate postoperative period. However, this deficiency was observed to decrease significantly during long-term follow-up monitoring.     

Further refinements on the triangular flap closure of the cleft lip

Refinements in the triangular flap closure of the unilateral cleft lip are presented. Randall's mathematical interpretation of the Tennison repair has been extended by using a series of arcs to determine the crucial points which form the triangular flap. Using this easily taught method, an isosceles triangular flap can be plotted which will interdigitate into the noncleft side of the lip. Two symmetrical vertical distances on either side of the cleft are thus formed. This allows for a standardized repair which may be more readily taught than the rotation-advancement technique. In addition, flaps are created which are turned medically toward the cleft and are used to accentuate the philtral pout, close the nostril floor, and reinforce the lip repair, allowing closure of even extremely wide clefts in one stage. These refinements in using the triangular flap closure for the repair of the unilateral cleft lip are diagrammatically presented. We believe that these refinements enhance the results of this closure.     

Outpatient cleft lip repair

The emphasis on cost reduction and increased efficiency in health care delivery has prompted an increase in outpatient (ambulatory) surgical procedures. A retrospective review of the perioperative management of patients undergoing cleft lip repair at two urban tertiary pediatric hospitals was performed to assess the safety of outpatient cleft lip repair. The hospital database at Childrens Hospital Los Angeles was searched to find all patients who had been operated on for cleft lip repair during calendar years 1999 and 2000. Two groups were identified from Childrens Hospital Los Angeles: the outpatient cleft lip repair group (patients discharged the same day as the operation; n = 91) and the inpatient cleft lip repair group (n = 14). A data set was acquired from the Royal Children's Hospital in Melbourne, Australia, using the same criteria, for fiscal years 1998 to 2000 (n = 50). All patients from Royal Children's Hospital had operations as inpatients. Parameters considered for each group were age, sex, race, ethnicity, length of hospital stay, preexisting medical conditions or diagnoses, complications, and readmissions or presentation to the emergency department within 4 weeks of operation. The Childrens Hospital Los Angeles outpatient group had three readmissions that were considered to be complications of the operation. The Childrens Hospital Los Angeles inpatient group had one readmission attributable to a complication. The Royal Children's Hospital group also had one readmission for a complication. There was no significant difference in the complication rate of the Childrens Hospital Los Angeles outpatient group and the Royal Children's Hospital group (p > 0.05). There was also no significant difference in the complication rate of both of the Childrens Hospital Los Angeles groups compared with the Royal Children's Hospital group (p > 0.05). This study indicates that cleft lip repair performed in an outpatient setting may be a safe alternative to the inpatient operation. Certain preexisting medical conditions, however, may dictate the need for inpatient hospitalization after repair.     

Bilateral cleft lip and nasal repair

SUMMARY: The bilateral cleft lip and nasal repair has remained a challenging endeavor. Techniques have evolved to address concerns over unsatisfactory features and stigmata of the surgery. The authors present an approach to this complex clinical problem that modifies traditional repairs described by Millard and Manchester. The senior author (H.S.B.) has developed this technique with over 25 years of surgical experience dealing with the bilateral cleft lip. This staged lip and nasal repair provides excellent nasal projection, lip function, and aesthetic outcomes. Lip repair is performed at 3 months of age. Columellar lengthening is performed at approximately 18 months of age. A key component of this repair focuses on reconstruction of the central tubercle. A triangular prolabial dry vermilion flap is augmented by lateral lip vermilion flaps that include the profundus muscle of the orbicularis oris. This minimizes lateral lip segment sacrifice and provides improved central vermilion fullness, which is often deficient in traditional repairs. The authors present the surgical technique and examples of their clinical results.     

New technique for correction of the microform cleft lip using vertical interdigitation of the orbicularis oris muscle through the intraoral incision

A microform cleft lip has three major components: (1) a minor defect of the upper vermilion border with loss of the mucocutaneous ridge; (2) a narrow ridge of tissue, resembling an exaggerated philtral column extending to the nostril sill; and (3) a deformity of the nostril. To attain the muscle continuity without an external scar on the upper lip, the author introduced a new method for the correction of a microform cleft lip deformity using vertical interdigitation of the orbicularis oris muscle through the intraoral incision to create the philtrum. Through the intraoral incision, a full-thickness incision is made down to the mucosa and the posterior portion of the muscle. Then, the remaining portion of the muscle is dissected. The medial and lateral muscle flaps are also detached from the oral mucosa and completely exposed and split into two leaves. The upper leaf of the lateral muscle flap is sutured to the dermis on the philtral dimple and base of the upper leaf of the medial muscle flap. Two leaves of each muscle flap are sutured together to create a vertical interdigitation to increase the thickness of the philtral column and to provide continuity of the muscle. A total of 12 patients with microform cleft lip were treated between August of 2001 and October of 2002. Seven of the patients were male and five were female, with an age range of 1 to 43 years. The follow-up period ranged from 6 months to 15 months, with an average follow-up of 9 months. The results of vertical interdigitation of the muscle were examined. All patients were satisfied with their results. The orbicularis oris muscle provided continuity and preserved good function. In all cases, the operation scar was not visible on the depressed philtral groove on the cleft side. Correction of cleft lip nasal deformity was performed in four patients and alar base advancement was performed in two patients. The advantages of the proposed procedure include the creation of an anatomically natural philtrum without an external visible scar through the intraoral incision, preservation of the continuity and function of the muscle, and sufficient augmentation of the philtral column by the vertical interdigitation of the muscle.     

Functional cleft lip repair: a sequential, layered closure with orbicularis muscle realignment

A lip repair based on anatomic and electric stimulation studies of the orbicularis muscle in unilateral cleft lip has previously been reported by one of the authors. Following some early modifications, this technique has been used on 125 primary lip repairs. The details of the present technique are described here. The advantages of the procedure, in addition to its functional reconstruction of the orbicularis muscle, are in its applicability to clefts of all widths and superior scar formation. The sequential nature of the procedure and freedom from commitment to a fixed, measured pattern at the outset makes the teaching of the method easier and the achievement of a pleasing result more predictable.     

In utero cleft lip repair in the mouse without an incision

In utero cleft lip repairs were done by full-thickness approximation of the cleft edges by 11-0 sutures in the A/J mouse on day 17 of gestation after Dilantin was administered on day 10. On day 20, a cesarean section was performed, the repair inspected, and the lip sectioned for histologic study. One-thousand one-hundred and thirty-nine mice were bred, and surgery was performed on 48. At least one fetus was present that had a cleft lip in 21 mice, and repairs were done on 16 fetuses. Ultimately, 9 viable fetuses were studied. In all cases, lip continuity was present where the suture had coapted the edges. There was little or no evidence of the lip repair despite no incision being performed. Histologic examination revealed epithelial and mesenchymal continuity with an occasional notch noted in the epithelium and soft-tissue asymmetry in complete clefts. The implications of these findings are discussed.     

A comparative analysis of healing of surgical cleft lip corrected in utero and neonates.

We studied the healing process in surgically created cleft lips in fetal mice and compared it with that in newborn mice with cleft lips. Our purpose was to determine the time for optimal healing, defined as minimal scarring, for a repaired cleft lip. Full-thickness paramedian lip incisions were made in NMRI mice in utero, in 2- and 4-day-old neonates, and in adults (n = 10 in each experimental and control group). The healing process was studied by biochemical analysis of hyaluronic acid and hydroxyproline content in the repaired cleft tissue. We found that the production of hyaluronic acid remained stable during the healing period and was similar in all experimental groups. However, there was an unexplained but consistent depression in the hyaluronic acid content of fetal tissue 2 days after repair. Hydroxyproline was present in the fetal healing tissue, but in a low concentration, starting 4 days after surgical incision of the lip. The production of hydroxyproline in 2-day-old neonates was similar to that in the fetuses throughout the healing period (p less than 0.0005). However, the production of hydroxyproline increased in 4-day-old neonatal and adult tissues. In conclusion, we found an optimal healing period for mice with minimal collagen production in the late fetal stage, and this lasted 2 days after birth.     

Lip pressure changes following lip repair in infants with unilateral clefts of the lip and palate

The present study was designed to quantitatively assess lip pressure changes following cleft lip repair in infants with unilateral cleft lip, alveolus, and palate. Lip pressure measurements were taken using an electronic transducer system developed especially for this study. Lip pressure was monitored from 3 months (preoperatively) through 2 years of age in cleft and normal control children. Findings from the present study confirm the hypothesis that lip repair in infants with unilateral cleft lip and palate significantly increases lip pressure and that increased lip pressure remains significantly higher than in normal control children for the 2-year duration of the study. Thus increased lip pressure when the palate is unrepaired has to be considered as a factor modulating subsequent craniofacial growth.     

The fetal cleft palate: III. Ultrastructural and functional analysis of palatal development following in utero repair of the congenital model

The role of fetal surgery in the management of congenital anomalies and intrauterine abnormalities is appropriately restricted on the basis of feasibility and risk-to-benefit analyses of intrauterine intervention. Recently, the authors demonstrated that in utero cleft palate repair of the congenital caprine model is technically feasible and results in scarless healing of the mucoperiosteum and velum, with subsequent development of a potentially functional bilaminar palate with distinct oral and nasal mucosal layers, following single-layer repair of the fetal mucoperiosteal flaps. A slight indentation at the site of repair was the only remaining evidence of a cleft. At 6 months of age, normal palatal architecture, including that of mucosal, muscular, and glandular elements, was seen grossly and histologically. The present work investigated the ultrastructural and functional aspects of the palate following in utero cleft repair to determine what benefits might be derived from fetal intervention. Six goats pregnant with twins were gavaged twice daily for 10 days (gestational days 32 to 41; term, 145 days) with dry, ground Nicotiana glauca plant delivering between 2.4 and 14 mg/kg per day of anabasine, doses that were adjusted in response to mater-nal toxicity. At 85 days' gestation, six fetuses underwent in utero palatoplasty using a modified von Langenbeck technique with elevation of bilateral mucoperiosteal flaps and lateral relaxing incisions. A single-layer repair of the mucoperiosteal flaps was performed using interrupted 6-0 Vicryl sutures. Six fetuses remained as unrepaired clefted controls. Six months after in utero palatoplasty, each group of goats underwent nasoendoscopy to evaluate palatal function; two unclefted 6-month-old goats served as controls. Subsequently, soft palate muscle was harvested from each of the goats and was evaluated by light and electron microscopy. Velar muscle was also harvested from the unclefted control goats and was similarly studied. Nasoendoscopy demonstrated functional palates capable of dynamic velopharyngeal closure following in utero cleft repair; this motion was similar to that observed in unclefted animals. Unrepaired clefted goats did not demonstrate any evidence of velar motion or velopharyngeal closure. Soft palate muscle from this group demonstrated evidence of myofibril degeneration, atrophy, and loss compared with unclefted control velar muscle. Ultrastructural changes included sarcomere "scalloping, " partial Z-line degeneration and loss, and progressive I-band degeneration and loss. Repaired clefted soft palate muscle was remarkably similar to unclefted control muscle. Significantly less myofibril, Z-line, and I-band degeneration and loss were observed with minimal evidence of sarcomere scalloping. In utero cleft palate repair results in a functional soft palate with restoration of ultrastructural architecture of the velum. These findings were attributed to reconstitution of the velar muscular sling, which is disrupted during the clefting process and remains abnormally inserted into the posterior edge of the palatal bone and along the bony cleft. Although repaired velar muscle does demonstrate some evidence of ultrastructural change compared with control muscle, these findings are significantly less pronounced than those observed in the unrepaired clefted muscle.     

Primary repair of bilateral cleft lip and nasal deformity.

LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. List five principles that guide synchronous repair of bilateral complete cleft lip and nasal deformity. 2. Explain how different growth rates for the principal nasolabial features are applied during primary repair. 3. Describe two approaches for positioning the alar cartilages to form the columella. 4. Discuss the influences on referral patterns for a newborn with bilateral cleft lip. --Traditional repair of bilateral cleft lip focused on labial closure but accentuated the nasal deformities, which were addressed later. By the end of the past century, single-staged labial closure had replaced the old multistaged procedures and the technical emphasis had begun to shift from secondary to primary nasal correction. Now, presurgical maxillary orthopedics sets the bony foundation for synchronous nasolabial repair and for closure of the alveolar clefts. The study of normal nasolabial growth and the typical stigmata of the conventional methods provides the necessary foreknowledge to guide surgical sculpture in three dimensions and to anticipate the fourth dimension. The convergence of several forces are changing referral lines for children born with bilateral cleft lip. These include affirmation of centers of excellence, surgeons' self-regulation, prenatal diagnosis, economics of health-care delivery, and increasing parental sophistication. These pressures are not necessarily in conflict. Care by a subspecialized plastic surgeon and experienced team is in the best interests of the child and the third-party payer.