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Research Letters |

Correction of the Secondary Bilateral Cleft Lip Deformity Encountered in Guatemala FREE

Frederic W.-B. Deleyiannis, MD, MPhil, MPH
Arch Facial Plast Surg. 2009;11(3):203-211. doi:10.1001/archfacial.2008.523.
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Published online

Cleft care in Guatemala is not standardized or centralized in distinct cleft centers. Instead, most cleft care is offered through surgical missions by multiple surgical groups likely to have diverse training and expertise. This is particularly true in rural areas of Guatemala. Owing to the lack of locally trained cleft specialists, distance, and cost, patients rarely have the option of presurgical orthopedics.1 Given these circumstances, the variety and severity of secondary cleft deformities that are encountered are distinctly different from those presently encountered in the United States.2

The purpose of this study is to describe the secondary bilateral cleft lip (BCL) deformities encountered in Guatemala during the last 6 years and the surgical techniques that I have used for correction.

From 2002 through 2007, 11 patients underwent secondary correction of BCL deformities during 6 consecutive annual cleft lip mission trips to Guatemala. Preoperatively, the secondary deformities were classified according to the abnormalities and deficiencies noted in the philtrum, white roll, vermilion, orbicularis oris (OO), and position of the premaxilla. Surgical correction was then based on this classification.

White Lip Deformities. The most common deformity encountered was an excessively wide philtrum (Table and Figure). An excessively wide philtrum was routinely associated with central vermilion and OO deficiency. Surgical repair consisted of excision of the scar and of philtral skin to narrow the philtrum; the central tubercle was recreated with recruitment of vermilion from the lateral lip elements and OO muscle plication (Mulliken-type repair).

Place holder to copy figure label and caption
Figure.

Secondary bilateral cleft lip deformities. A, Patient 1, showing misalignment of Cupid's bow). The prolabial vermilion was preserved for the central vermilion at the time of primary bilateral lip repair. B, Patient 2, showing wide philtrum, misalignment of the Cupid's bow, and orbicularis deficiency (OO). The prolabial vermilion was also used for the central vermilion reconstruction at the time of the primary bilateral lip repair. C, Patient 5, showing a wide philtrum with central vermilion and OO deficiency. D, Patient 10, showing a catastrophic deformity. The primary lip repair has completely dehisced with secondary healing behind the premaxilla. E, Patient 10, basal view. F, Patient 11, showing a catastrophic deformity and dehisced lips with severe scarring and the need for a premaxillary setback.

Graphic Jump Location

Central Tubercle Deformities. The central deformities included either a vermilion mismatch or vermilion deficiency, usually combined with an OO deficiency. A vermilion mismatch was commonly seen in those patients in whom the dry vermilion of the prolabium had been preserved for creation of the central tubercle (Figure). At the time of secondary repair the remaining prolabial dry vermilion was excised, and the dry vermilion and mucosa from the lateral lip element were then used for creation of the median tubercle.

Catastrophic Deformities. Catastrophic deformities were defined as the results seen after obvious lip dehiscence and secondary wound healing. In 2 cases the lips had healed behind a protruding premaxilla. Correction consisted of excising the scar, designing a philtrum from the unscarred central white lip, and forming the median tubercle from lateral labial elements. Two patients required a premaxillary setback to achieve a tension-free lip closure.

The principles that guide the primary repair of the BCL deformity include (1) symmetry, (2) primary OO muscular continuity throughout the vertical extent of the upper lip, (3) proper philtrum size and shape, and (4) formation of the median tubercle from the lateral labial elements. According to Mulliken,2 the philtrum is constructed on a small scale because the constructed philtrum widens remarkably and has considerable vertical growth. Because the prolabium is deficient in both central vermilion and mucosa and often lacks a distinct white roll, prolabial vermilion and mucosa should not be preserved. Following these principles, Mulliken2 observed that the primary and secondary problems that are now encountered in specialized cleft lip centers are mainly only extra mucosa in the lateral labial elements and minor vertical deficiencies of the median tubercle.

Many of the secondary BCL deformities that were encountered in Guatemala were similar to those deformities that could be described as the traditional bilateral cleft stigmata. The common stigmata that I have observed are an excessively wide philtrum, a preservation of the prolabial vermilion and mucosa, and a median tubercle with a deficiency of dry vermilion and OO muscle. To repair these deformities, I have relied on the principles outlined herein.

The size of the philtrum flap depends on race and age. In North American white individuals aged 1 to 18 years, Farkas et al3 documented normal growth of the nasolabial features. All of the labial landmarks grew rapidly, approaching approximately 90% of adult proportions by 5 years of age. Only columellar length and nasal tip projection were slow growing (ie, attaining less than two-thirds of adult size by 5 years of age). In expectations of fast growth in the labial elements, Mulliken2 recommended designing the philtrum during the primary repair on a small scale. Fast-growing features may become too long or too wide. Expanding these recommendations to what I have encountered at the time of secondary cleft lip repair, I have designed the dimensions of the philtrum flap based mainly on age. Thus, patients who were 5 years or older (most of the patients) had a constructed philtrum that was designed on a normal scale. Younger patients had their secondary philtrum designed on a slightly reduced scale.

Five of the 11 patients (45%) had partial or complete lip dehiscence after primary BCL repair. One likely reason for this high incidence of lip dehiscence is the presence of a protruding premaxilla at the time of primary lip repair. Repositioning of the protruding maxilla, either surgically or nonsurgically, permits a better repair of the lip by relieving undue tension. Nonsurgical repositioning with such devices as elastic traction, nasoalveolar molding, or active appliances (such as the Latham device4) is not practical because these devices are not readily available or affordable in Guatemala. Surgical correction thus consists of 2 possibilities: (1) a planned lip adhesion with a definitive repair staged at a later date or (2) surgical setback of the premaxilla. Because of the lack of long-term follow-up, it is doubtful that surgeons on cleft lip mission trips routinely plan a 2-staged lip repair with initial adhesion. Surgical excision of the premaxilla is not advocated because the premaxilla is recognized as the keystone of the maxillary arch. Premaxillectomy in an infant causes flattening or concavity of the midface, loss of support for the upper lip, and failure of forward growth of the nose. The risk of premaxillary necrosis is real when the premaxillary setback is performed with the labial repair. However, in my 2 patients, the premaxilla remained pink and bleeding after the setback.

Surgical setback of the premaxilla is indicated only as a final option. As demonstrated by Friede and Pruzansky,5 the premaxillary-vomer suture is the likely growth center affecting midface, nose, and maxillary arch development. Surgical setback of the premaxilla, if performed before the completion of midface growth, likely places a patient at risk for midface retrusion and a concave profile.6 However, various authors have also suggested that a protrusive premaxilla can be set back after 6 to 8 years of age without deleterious effects on midfacial growth.7

Malnutrition is endemic in Guatemala8 and presents another risk for poor wound healing and potential lip dehiscence. For example, nearly 50% of children in Guatemala younger than 5 years have low height for their age.9 Inadequate dietary intakes and high rates of infections are believed to be the principal postnatal biological factors that limit children's growth. A previous analysis of Guatemalan infants' dietary data indicated low intake of several micronutrients, especially iron and zinc, vitamin B12, and protein.10

In conclusion, patients encountered in the developing world often represent a reconstructive challenge not encountered in the United States. Most of these secondary deformities can reliably be repaired with scar excision, a Mulliken-type repair, and, if necessary, a premaxillary setback.

Correspondence: Dr Deleyiannis, Ste 6B, Scaife Hall, 3550 Terrace Ave, Pittsburgh, PA 15261 (deleyiannisfw@msx.upmc.edu).

Financial Disclosure: None reported.

Additional Contributions: I gratefully acknowledge the dedication and charitable work of Children of the Americas (COTA) Inc, a not-for-profit charitable corporation of volunteers who provide gratis medical and surgical treatment to the children of Guatemala.

Grayson  BHMaull  D Nasoalveolar molding for infants born with clefts of the lip, alveolus, and palate. Clin Plast Surg 2004;31 (2) 149- 158, vii
PubMed
Mulliken  JB Primary repair of bilateral cleft lip and nasal deformity. Plast Reconstr Surg 2001;108 (1) 181- 194
PubMed
Farkas  LGPosnick  JCHreczko  TMPron  GE Growth patterns of the nasolabial region: a morphometric study. Cleft Palate Craniofac J 1992;29 (4) 318- 324
PubMed
Latham  RA Bilateral cleft lip and palate: improved maxillary and dental development. Plast Reconstr Surg 2007;119 (1) 287- 297
PubMed
Friede  HPruzansky  S Long-term effects of premaxillary setback on facial skeletal profile in complete bilateral cleft lip and palate. Cleft Palate J 1985;22 (2) 97- 105
PubMed
Vargervik  K Growth characteristics of the premaxilla and orthodontic treatment principles in bilateral cleft lip and palate. Cleft Palate J 1983;20 (4) 289- 302
PubMed
Padwa  BLSonis  ABagheri  SMulliken  JB Children with repaired bilateral cleft lip/palate: effect of age at premaxillary osteotomy on facial growth. Plast Reconstr Surg 1999;104 (5) 1261- 1269
PubMed
Rivera  JAHabicht  JP Effect of supplementary feeding on the prevention of mild-to-moderate wasting in conditions of endemic malnutrition in Guatemala. Bull World Health Organ 2002;80 (12) 926- 932
PubMed
 Encuesta Nacional de Salud Materno Infantil, 2002.  Guatemala City, Republic of Guatemala Instituto Nacional de Estadistica, Center for Disease Control2002;
Bégin  FSantizo  MCPeerson  JMTorún  BBrown  KH Effects of bovine serum concentrate, with or without supplemental micronutrients, on the growth, morbidity, and micronutrient status of young children in a low-income, peri-urban Guatemalan community. Eur J Clin Nutr 2008;62 (1) 39- 50
PubMed

Figures

Place holder to copy figure label and caption
Figure.

Secondary bilateral cleft lip deformities. A, Patient 1, showing misalignment of Cupid's bow). The prolabial vermilion was preserved for the central vermilion at the time of primary bilateral lip repair. B, Patient 2, showing wide philtrum, misalignment of the Cupid's bow, and orbicularis deficiency (OO). The prolabial vermilion was also used for the central vermilion reconstruction at the time of the primary bilateral lip repair. C, Patient 5, showing a wide philtrum with central vermilion and OO deficiency. D, Patient 10, showing a catastrophic deformity. The primary lip repair has completely dehisced with secondary healing behind the premaxilla. E, Patient 10, basal view. F, Patient 11, showing a catastrophic deformity and dehisced lips with severe scarring and the need for a premaxillary setback.

Graphic Jump Location

References

Grayson  BHMaull  D Nasoalveolar molding for infants born with clefts of the lip, alveolus, and palate. Clin Plast Surg 2004;31 (2) 149- 158, vii
PubMed
Mulliken  JB Primary repair of bilateral cleft lip and nasal deformity. Plast Reconstr Surg 2001;108 (1) 181- 194
PubMed
Farkas  LGPosnick  JCHreczko  TMPron  GE Growth patterns of the nasolabial region: a morphometric study. Cleft Palate Craniofac J 1992;29 (4) 318- 324
PubMed
Latham  RA Bilateral cleft lip and palate: improved maxillary and dental development. Plast Reconstr Surg 2007;119 (1) 287- 297
PubMed
Friede  HPruzansky  S Long-term effects of premaxillary setback on facial skeletal profile in complete bilateral cleft lip and palate. Cleft Palate J 1985;22 (2) 97- 105
PubMed
Vargervik  K Growth characteristics of the premaxilla and orthodontic treatment principles in bilateral cleft lip and palate. Cleft Palate J 1983;20 (4) 289- 302
PubMed
Padwa  BLSonis  ABagheri  SMulliken  JB Children with repaired bilateral cleft lip/palate: effect of age at premaxillary osteotomy on facial growth. Plast Reconstr Surg 1999;104 (5) 1261- 1269
PubMed
Rivera  JAHabicht  JP Effect of supplementary feeding on the prevention of mild-to-moderate wasting in conditions of endemic malnutrition in Guatemala. Bull World Health Organ 2002;80 (12) 926- 932
PubMed
 Encuesta Nacional de Salud Materno Infantil, 2002.  Guatemala City, Republic of Guatemala Instituto Nacional de Estadistica, Center for Disease Control2002;
Bégin  FSantizo  MCPeerson  JMTorún  BBrown  KH Effects of bovine serum concentrate, with or without supplemental micronutrients, on the growth, morbidity, and micronutrient status of young children in a low-income, peri-urban Guatemalan community. Eur J Clin Nutr 2008;62 (1) 39- 50
PubMed

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