AUTHOR AND EDITOR INFORMATION

Section 1 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

INTRODUCTION

Section 2 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Although surgery for the bilateral cleft lip has undergone many advances, correction of the nasal deformities associated with this congenital malformation remains one of the greatest challenges in plastic surgery. Various single- and multiple-stage procedures have been used. Deformities may become apparent after further growth and development of the nose, making the bilateral cleft lip nasal deformity a 4-dimensional problem.

History of the Procedure

Early attempts to repair the cleft lip nasal deformity in conjunction with the lip component resulted in scarring and corrections that did not last. This led to the beliefs that primary repair may interfere with growth of the nasal cartilages and that the nasal deformity should not be corrected until nasal growth is complete. Soon, evidence began to refute these beliefs, and some have claimed that early surgery may assist growth. Unilateral staged procedures were reported first, and these gave way to bilateral staged procedures. Currently, some authors advocate simultaneous bilateral lip and nose repair.

Problem

Surgical correction of nasal deformities associated with bilateral cleft lip is challenging because deformities may become apparent as the nose undergoes further growth and development.

Frequency

The incidence of cleft lip with or without cleft palate in the United States is estimated to be 1 case in 1000 individuals. This varies by race. Caucasian persons have an incidence of 1.34 cases per 1000 individuals, African American persons have an incidence of 0.41 cases per 1000 individuals, and Japanese persons have an incidence of 2.1 cases per 1000 individuals. Approximately 10-25% of cases are bilateral. The incidence of cleft lip with or without cleft palate is also affected by sex; males are affected in 60-80% of cases.

Etiology

The cause of cleft lip with or without cleft palate is still under debate. In the past, the multifactorial/threshold model was believed to be the best model for transmission of cleft lip and palate. This model describes a threshold at which a phenotype is expressed in an individual based on genetic susceptibility and the environment. It was developed because common mendelian inheritance patterns were not present with familial clusters of clefts and other birth defects. However, more recently, some studies suggest that in some situations, major gene loci may be operating.

Cleft lip can be described as either syndromic or nonsyndromic. Race appears to play an important role in nonsyndromic clefting. Individuals of Asian descent generally have underdeveloped derivatives of the median nasal process (MNP), leading to an increased incidence of clefting, whereas African American persons have well-developed MNP derivatives and experience a rate of clefting one fourth that of Asian persons. Animal studies have also demonstrated that nasal cavity width and epithelial activity in the approaching processes also may be involved. In addition, any factors altering the electron transport chain, including hypoxia, possibly phenytoin, and carbon monoxide, can increase the incidence of cleft lip. An increased incidence of cleft lip also may exist among children with fetal alcohol syndrome.

Approximately 14% of cleft lips, with or without cleft palate, are considered syndromic. Trisomies 13 and 21 both affect MNP development, leading to an increased incidence of clefting. One percent of persons with clefts may present with Waardenburg syndrome, in which neural crest cells fail to develop properly. This syndrome is associated with deafness and patchy pigmentation absence. Another rare cause is van der Woude syndrome, which manifests with lip pits due to abnormal salivary glands. The cleft in this instance may be due to failure of the epithelial lining of the facial processes to regress, preventing fusion.

Pathophysiology

The face develops from 5 processes in the human embryo, the single frontonasal process and the paired maxillary and mandibular processes. These 5 processes consist mainly of neural crest tissue and are derived from the first pair of pharyngeal arches at approximately the fourth week of gestation. The frontonasal process begins to develop median and lateral nasal prominences around an olfactory pit at approximately the 33rd day of gestation. The maxillary processes continue to develop medially toward the median and lateral nasal processes of the frontonasal process. The maxillary processes compress the MNPs, and the epithelium is believed to stretch and break down, allowing the processes to fuse in the normal fetus.

A cleft lip may result through processes described by the classic theory, the mesodermal penetration theory, or, more likely, a combination of both. The classic theory of His postulates a failure of fusion, thought to be more important for the development of clefts of the secondary palate.

The mesodermal penetration theory of Pohlmann and Veau emphasizes the importance of mesodermal migration and support of the fusing structures, without which the epithelial layers break down. This theory may be more important for the development of clefts of the primary palate. Schendel et al investigated the muscle at the site of cleft lips by performing a biopsy, the results of which demonstrated nonneurogenic atrophy and disorganization of the muscle fibers at the cleft margin, yielding a metabolic explanation for poor mesenchymal migration due to abnormal local mitochondria. These findings lend more support to the mesodermal penetration theory at the site of primary palatal clefts.

Clinical

See Relevant Anatomy for a list of defects affecting individuals with bilateral cleft lip nasal deformity.

INDICATIONS

Section 3 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

The indication for surgery is any visible nasal deformity associated with a bilateral complete or incomplete cleft of the lip with or without clefting of the palate.

RELEVANT ANATOMY

Section 4 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Nasal anatomy

The bony structure of the nasal pyramid includes the 2 nasal bones, the frontal processes of the maxilla, the nasal processes of the frontal bone, and the bony septum. The osseous septum is formed by the vomer and perpendicular plate of the ethmoid. This osseous framework is supplemented by an extensive cartilaginous component also essential for nasal form and function: the paired upper lateral cartilages (ULCs); lower lateral cartilages (LLCs), or alar cartilages; and the cartilaginous septum. The LLCs are made up of 2 crura. One is lateral, providing support to the superior portion of the nostril, and one is medial. The paired medial crura form the internal structure of the columella. The septum adds support to the external structure of the nose by preventing the ULCs and LLCs from collapsing into the face.

The nasal cavity begins at the nares, or nostrils, extending posterior to the choanae. The nasal floor consists of the palatine process of the maxilla and the horizontal plate of the palatine bone. The nasal roof extending toward the choanae also includes the cribriform plate of the ethmoid and the body of the sphenoid. The nasal conchae project from the lateral wall into the cavity. Just inside the nares lies the vestibule, the squamous epithelia-lined caudal portion of the nasal cavity. The medial wall consists of the nasal septum and is composed of membranous, cartilaginous, and osseous components.

The nose, lip, and alveolar cleft components comprise clefting of the primary palate. Clefting behind the incisive foramen comprises the hard and soft palate or secondary palate.

Anatomy of the bilateral cleft lip nasal deformity

The following deformities of the bilateral cleft lip nose have been well described by many authors:

• Shortened columella (apparent vs true)
• Broad flat nasal tip with an obtuse mediolateral crural angle
• Inferolaterally displaced alar dome cartilage (ie, bucket handle)
• Caudally displaced medial crus
• Caudally, laterally, and dorsally displaced lateral crura
• Splayed alar domes (increased separation between the genua, with interposed soft tissue)
• Subluxed LLCs off their overlapping ULCs
• Hypoplastic alar cartilages, accessory cartilages, and lobular fat
• Laterally displaced alar base
• Obtuse alar nasi-cheek angle
• Bridge of tissue (Simonart band) that may span an incomplete cleft (ie, nasal floor deficits)
• Lack of nasal sills
• Elongated and buckled lateral crura (M shape vs normal C shape)
• Drooped flared nostril rim (cat's knees)
• Vestibular web due to protrusion of the caudal margin of the LLC into lateral vestibule
• Lack of flare at the base of the columella
• Splayed alar domes and laterally displaced alar bases leading to nostrils that have a horizontal orientation
• Protrusion of the premaxilla, which contributes to the deformity by decreasing the nasolabial angle (not specifically part of the nose)
• Maxillary hypoplasia, which increases the nasolabial angle
• Paucity of alveolar bone at the cleft, leaving the tip and ala to collapse without a foundation

Recently, some authors have questioned whether the columella is truly shortened or if it appears short due to the splaying of the medial crura. They contend that the columellar tissues reside in the nasal tip and that the distal end of the columella has been unzipped due to the splaying of the medial crura.

The nasal deformity can vary considerably. In patients with bilateral incomplete clefts, little, if any, detectable deformity may be present. In patients with severe bilateral clefting, the deformity may be profound. Clefting of varying degrees may occur on each side, leading to asymmetry of the nasal deformity and rotation of the premaxilla.

CONTRAINDICATIONS

Section 5 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Any anomalies that compromise the patient's intraoperative or postoperative course may be contraindications to surgery. Diseases that may interfere with normal growth and development may impact the final repair. The child should undergo a complete physical examination to determine the extent of any concurrent medical illnesses and should be eating and gaining weight normally. Marsh and Martin¹ choose to perform repairs after 8 weeks, when the infant has fully switched to extrauterine circulation and is more tolerant of nasal obstructions. Even a small amount of blood loss is always a concern in surgery of children with very small total blood volumes. This also may help guide surgical timing, even more so for secondary palatal surgery in which larger amounts of blood loss may be expected.

Millard suggests the infant be free of respiratory infection for at least 2 weeks and without any skin infection at the time of surgery. He adds that chest radiographs be obtained and preoperative and intraoperative antistreptococcal antibiotics be used for every patient. A complete blood cell count should demonstrate a hemoglobin value of at least 10 g/100 mL and a hematocrit value of 35%. The rule of tens also applies: the patient should be aged at least 10 weeks, have a hemoglobin value of 10 g/100 mL, weigh at least 10 lb, and have a WBC count of less than 10,000/µL.

WORKUP

Section 6 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Lab Studies

• Expected results of a complete blood cell count are as follows:
• • Hemoglobin value of at least 10 g/100 mL
  • Hematocrit value of 35%
  • WBC count of less than 10,000/µL

Imaging Studies

• Chest radiographs are used preoperatively to confirm the absence of a respiratory infection or any other pulmonary anomalies that may compromise the patient's intraoperative or postoperative course.

TREATMENT

Section 7 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Surgical Therapy

See Intraoperative details for a list of surgical procedures.

Preoperative Details

A protruding premaxilla places tension across a bilateral cleft lip repair. Because of this, presurgical orthopedic appliances are often applied. Two categories of appliances are used, passive and active.

Passive appliances maintain the distance between the 2 maxillary segments while external force is applied to the premaxilla to reposition it posteriorly. This external force can be external taping, a head cap with elastic straps across the prolabium, or a lip adhesion.

One of the most commonly used active appliances is used by Millard and Latham.² It consists of elastic chains, screws, plates, and a pin through the premaxilla. It actively expands the maxillary segments and shifts the premaxilla posteriorly into the gap between them. This may be accomplished as early as 3 weeks into treatment.

Presurgical orthopedic devices are widely advocated by most but may not be used because of lack of experience of the prosthodontist, a lack of family compliance or family distance from hospital or clinic, the use of lip adhesions, or the minor nature of the maxillary defect.

Intraoperative Details

Principles of technique

Various techniques have been devised. Through his experiences, Mulliken has elucidated some of the principles he believes are necessary in the treatment of the bilateral cleft lip with nasal deformity.³ They are as follows:

• Maintain symmetry.
• Secure primary muscle union.
• Select the proper prolabial size and configuration.
• Form the median tubercle and mucocutaneous ridge from lateral lip tissue.
• Construct the nasal tip and columella by anatomic placement of the alar cartilages.

LaRossa and Donath⁴ emphasize the following:

• The maxilla also must be managed presurgically.
• The LLCs should be released from any soft tissue attachments, including the vestibular lining, to prevent any tendency for the cartilages to return to their previous positions.
• The alar bases must be repositioned and the nasal floor repaired.
• Bolstered sutures or stents are involved in the final cartilage shape.

Broadbent and Woolfe's simultaneous primary repair of lip and nose

Broadbent and Woolfe were among the first to describe a method of primary repair of the lip and nose simultaneously.⁵ They claimed that early surgery actually may assist growth and believed it certainly did not retard growth. Their experiences taught them that a good primary repair endures; however, any deformities not fully repaired do not improve with time.

Their method consists of making an incision in the superior buccal-labial sulcus extending between the ULC and LLC to the tip. The superior border of the LLC is undermined, and the skin of the nasal tip and over the ULCs is freed. The LLCs are relocated by pulling them upward and medial. Sutures are placed between the ULCs and the LLCs. The first fixes the superior edge of the LLC to the ipsilateral ULC and septum. This effectively repairs the caudal rotation of the medial and lateral crura by fixing them to the ULC. A midline incision of the nasal tip is made, and the domes of the LLCs are sutured together after removing any intervening tissue, achieving lengthening of the columella and improving tip projection. The alar bases are also rotated medially after the cartilages are in place, thus narrowing the widened alar bases.

Mulliken procedures

In 1985, Mulliken published a 2-stage procedure.³ The first stage involved banking forked flaps from the prolabium in the nasal floor at the time of lip repair. They then were released later and placed intranasally into an intercartilaginous incision. He later found this to be unnecessary, and he began removing the forked flaps at the time of lip repair and performing the lip and nasal repair as a single procedure.

He begins by excising all of the prolabial skin except for a single central flap for reconstruction of the philtrum, Cupid's bow, and philtral columns. An incision is also made around the alar bases. A single vertical incision is placed over the tip of the nose and along the rims. The LLCs are freed on their anterior surfaces through the nasal incisions. The nasal floor, orbicularis oris, and lip repairs are then completed. The domes of the LLCs are then elevated and sutured together. The lateral portion of each dome is suspended to the ipsilateral ULC near the septum. The freed alar bases are held to the prolabium medially via a mattress suture from alar base to base. Excess tissue in the soft triangle is excised. When necessary, a ridge of vestibular lining is excised. Labial closure is then completed.

Mulliken later found that the central nasal tip incision was unnecessary because he could suture the domes together through the rim incisions.

This technique lengthens the columella and reconstructs the nasal tip by suturing the domes together. It also addresses the subluxation of the LLCs off the ULCs by suspending them to the ULCs with sutures. This aids in reorienting the nostrils in a more vertical rather than transverse position. The drooping of the nostril rim is also corrected through suspension of the LLCs to the ULCs and through suturing the domes together. The alar bases are brought more medially, decreasing the width between them and making the alar nasi-cheek angle more acute. Excision of nasal mucosa removes any excess and increases support of the lateral crura, correcting vestibular webbing.

McComb procedure

In 1986, Harold McComb published a 10-year follow-up study of repairs he had accomplished using a 2-stage procedure.⁶ It involved lengthening the columella with forked flaps taken from the prolabium when the patient is aged 6 weeks, with repair of the lip and nasal deformity performed 6 weeks later. Five years later, he began to notice complications associated with this type of columellar lengthening and abandoned this technique in favor of another 2-stage procedure (see Complications). In describing his new procedure, he noted that embryologically, the prolabium belongs to the lip and therefore should not be used to reconstruct the columella.

McComb's current technique, after preoperative orthopedic appliances are used, involves nasal floor repair and long lip adhesions when the patient is aged 6-8 weeks. A V-shaped incision is made above the nostril rim with the tip of the V ending over the dorsal columella. Undermining of the nasal skin over the LLCs is accomplished through the incision in the buccal sulcus. The alar bases are mobilized. The soft tissue between the domes is removed, and temporary suspension sutures are placed through the LLCs. The domes are then sutured together, and the nasal flap is closed in a V-Y advancement fashion, lengthening the columella by approximately 5 mm. Mattress sutures over bolsters are placed to eliminate any dead space in the tip. At the second stage, 1 month later, the prolabium is lifted and mucomuscular flaps are sutured behind it, completing the lip repair.

McComb's technique addresses the short columella through suturing the alar domes together. The skin is closed with a V-Y advancement to allow for a lengthened columella while leaving a dorsal nasal scar. In addition, suturing of the domes improves nasal tip projection, eliminating the broad flat nasal tip. Wide undermining is used to correct displacement and deformity of the lateral crura.

Salyer procedure

Salyer performs his lip repair procedure, along with a limited nasal repair, when the patient is aged approximately 3 months.⁷ Initial surgery entails bilateral superiorly based prolabial flap elevation developed around the flap to be used for philtral reconstruction. These are rotated into the nasal floor after bilateral vertical intranasal alar and alar base incisions allow freeing of the LLCs and rotation of the bases medially. The lip repair is then completed.

Further nasal reconstruction is achieved when the patient is aged 12-15 months. Incisions are made below each alar base running toward the midline. Bilateral horizontal infrasill incisions meet in the midline and extend up the columella. Through this incision, the alar cartilages and columellar skin are freed. The alar cartilages are advanced, and the domes are sutured together. This lengthens the columella and redefines the tip through advancement of nasal floor tissue. No permanent sutures are described between ULCs and LLCs. Instead, Dacron bolsters are placed and left for 6-7 days to promote LLC support, nasal tip projection, and lateral crus stabilization, which is thought to eliminate vestibular webbing.

Noordhoff procedure

In 1986, Noordhoff published a 2-stage procedure for lip and nasal repair.⁸ Because of compliance issues, routine preoperative orthopedic appliances are not part of his treatment plan. The initial lip and primary nasal repair is performed when the patient is aged 3 months.

He begins by marking one central flap and 2 lateral fork flaps on the prolabium; the fork flaps vary in width according to the width of the prolabium. He then elevates a buccal mucosal L flap. The alar cartilages are then freed through incisions extended from the buccal mucosal flap up into the vestibule between the ULC and LLC. The prolabial incisions are made, and the prolabial flaps are freed from the premaxilla. Prolabial vermillion becomes mucosal premaxillary lining. The domes are held in place with temporary suspension sutures while the LLC is sutured to the contralateral and ipsilateral ULC, correcting the subluxation of the LLCs. The intercartilaginous incision is covered by the buccal mucosal flap, taking tension off the repair as opposed to the previous Mulliken fork flap placement. Reconstruction of the nostril floor is then accomplished.

Noordhoff introduces further nasal floor augmentation with lateral forked flaps rotated into the columellar incisions from excess lip tissue. These flaps are optional based on lip tissue availability. The prolabial forked flaps are then rotated to lie horizontally across the nasal sill in the whisker position. When no lateral forked flaps are developed, the prolabial forked flaps still take their whisker positions. The lip reconstruction is then completed.

When the patient is aged 1-6 years, Noordhoff lengthens the columella using a technique described by Cronin and Upton.⁹ Small triangles of tissue are removed from the nasolabial fold, and the nasal floor tissues are rotated into the columella via a confluent bilateral infrasill and midcolumellar incision. A composite ear graft may be used if inadequate tissue is available for advancement. The alar bases and orbicularis are sutured to the nasal spine to attempt to prevent columellar drift. The domes are also sutured together at the tip to improve tip projection.

Millard procedure

Millard maintained that the columella is actually shortened because the dislocated alar cartilages have not stretched the columella properly, and he designed a repair with this in mind.¹⁰ He begins by making periosteoplasties on the alveolar segments and then turns his attention to the nose. He warns that using nasal tip tissue results in both the columella and prolabium being too short. He points out that the columella and prolabium sit side by side in the 30-day embryo and gives this as his reason for taking prolabial forked flaps and banking them in the whisker position. If insufficient prolabium is available, lip adhesions may be used to enlarge the prolabium.

When the patient is aged 4 years, the fork flaps are freed and advanced into the columella. The LLCs are well developed, and open rhinoplasty and intercartilaginous and rim incisions are used to free them. The medial crura are sutured together and to the septum. Further support is added with sutures between the ULCs and the LLCs. Sutures bring the alar bases toward the midline.

This correction incorporates forked flaps to lengthen the columella while improving the nasal tip projection and narrowing the tip through suturing the medial crura together. The widely spaced alar bases are also addressed. Further complications associated with forked flaps are detailed in Complications.

Cutting and Grayson procedure

In 1993, Cutting and Grayson described a prolabial unwinding flap method as a single-stage reconstruction of the bilateral cleft lip and nasal deformity, relying on presurgical orthopedic appliances and sufficient prolabial size.¹¹ The repair involves an incision beginning at one side of the columellar base and continuing inferiorly and medially. When the prolabium and premaxilla are separated, the portion inferior to the incision is swung out and around so it is in line with the proximal end of this newly created flap. This flap ultimately forms the inferior columella and philtrum and wraps around the orbicularis muscle layer that is initially repaired behind it. Before it is placed, it is lifted, and the nasal repair begins.

The nasal repair is similar to McComb's original method. The columella is advanced along the membranous septum and sutured in place. The alar bases are sutured medially. Advantages of this method are the absence of external nasal scars, the use of a single-stage reconstruction of the columella and central upper lip, and the absence of a lip-columellar junction scar (see Complications).

More recently, Grayson has been popularizing the use of nasoalveolar molding (NAM).¹² This is a very eloquent but work-intensive molding device that is anchored to the presurgical orthopedic device, pressing up on the collapsed nasal vestibule. It is felt to remodel the cartilage and even create increased tissue for the subsequent less-involved surgical repair of the nose.

Authors' choice of procedures

Delaire¹³ and Schendel¹⁴ have emphasized the physiologic reconstruction of cleft lip and nasal deformities with focus on the muscle reconstruction not only of the lip but around the nose. The author tries to adhere to these physiologic techniques.

The procedure used by the primary author varies according to the severity of the cleft and the associated nasal deformity. The author believes that no single procedure can successfully repair clefts of various severities. The ultimate goal of the surgery should be to achieve the best possible result by the time the child is ready to attend school, with a view to final open rhinoplasty, as necessary, at craniofacial maturity.

If the cleft is not wide with a sufficiently sized prolabium, primary nasal repair is recommended. With this approach, the lip repair is performed and the nasal repair is accomplished firstly by dissection within the columella between the medial crus of the LLCs and up over the domes after elevation of the prolabium and through the lateral elements in the subcutaneous plane over the lateral portion of the LLCs to the domes. In order to free up the transverse nasalis musculature and achieve narrowing of the ala, this lateral dissection is extended over the lateral nasal wall region with subsequent subperiosteal dissection in the same region. The orbicularis oris muscle is freed from the lateral element skin and underlying periosteum. The prolabial vermilion is rolled down into the vestibule and lip mucosal repair is done.

The transverse nasalis is then sutured to the caudal septum in the region of the nasal spine, correcting the transverse alar base positions with nonresorbable sutures, and the orbicularis repair is completed beneath the prolabium. Resorbable Vicryl sutures are used to resuspend the LLCs to the ULCs, to narrow the nasal tip as an interdomal suture, at the alar cheek junction, and to hold a silastic stent in the nostrils. These sutures redrape the skin and subcutaneous tissue and narrow and elevate the tip while lengthening the columella.

In a wide cleft with severe nasal deformity, NAM is likely the state of the art, especially for these difficult bilateral cleft noses. However, in the primary author’s practice, it is not yet available. The authors feels that, with the potential for tissue expansion and early cartilaginous remodeling, NAM offers a minimally invasive presurgical advantage in the shape of the vestibule and tip and may increase the amount of columellar tissue. The primary author's practice does offer all wide clefts elastic bonnet or intraoral acrylic alveolar trays, adjusted weekly for the most compliant families, with good results at the nasal base, which makes the intraoperative repair easier with less dissection. 

The second choice, especially for patients who are hundreds of miles from the treatment center, is a bilateral lip adhesion with subsequent lip and nasal repair about 3 months later. During the subperiosteal dissection of these wide clefts, the tissue is sometimes elevated around the inside of the piriform aperture and onto the maxilla to allow enough medial movement of the alar bases. In addition, depending on the amount of tissue needed, the medial edge of the lateral elements may be used along with the lateral portions of the prolabium for nasal floor and sill reconstruction.

When the patient is nearing school age, he or she is reassessed for lip and nasal revisions. At this age, the author may use cadaveric rib cartilage to augment the nasal tip projection and lengthen the columella. Revising widened lip scars, broad philtrums, or dehisced orbicularis muscle helps bring the alar bases to a more normal position.

A final open rhinoplasty technique at craniofacial maturity, using septal, ear, or rib cartilage, can provide further improvements that may have been lost with the child’s growth. This is the opportunity for a permanent change in the nasal form. Revision of the nose can be achieved through an infracartilaginous incision, which may be connected to a transcolumellar incision, allowing better access to the nasal tip structures. The alar domes and medial crura are sutured together through the open technique under direct vision. At this time, additional cartilage can be added to the tip to improve nasal tip projection, or, a columellar strut may be added for extra support.

The authors have attempted to detail the methods and findings of some of the most experienced cleft lip surgeons in the area of primary bilateral cleft lip nasal deformity repair. This is by no means an exhaustive list of techniques, but it should provide the reader with an understanding of the challenges involved.

Postoperative Details

Postoperative care includes 2 weeks of syringe feeding and arm bolsters with nothing placed in the mouth or over the lip and nasal base (ie, no soothers or bottles). Patients without secondary palatal clefts who were breastfeeding prior to lip and nasal repair are allowed to breastfeed immediately. The repair is reviewed after 2 weeks, and the nasal stents are removed if they have not already fallen out. The wound should be assessed for any signs of infection or dehiscence.

Follow-up

Patients should be observed at 3 months and 1 year after the operation. Any residual deformities are apparent at these times. Residual deformities should be assessed to determine if any further surgery is necessary.

These patients demand a long period of follow-up care to observe growth over time and detail final outcomes.

COMPLICATIONS

Section 8 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

Many of the complications of cleft nasal surgery are described as occurring after the forked flap technique. McComb has extensive experience with the forked flap. Originally, he lengthened the columella with forked flaps taken from the prolabium. He found that 3 deformities developed as patients approached adolescence: (1) the columella was excessively long compared to control subjects, which led to larger-than-normal nostrils; (2) the base of the columella tended to drift inferiorly onto the upper lip, creating a more obtuse nasolabial angle and allowing the scars from the forked flap to drift downward and become more noticeable; and (3) the nasal tip remains broad as the cartilages continue to splay at the domes.

Cutting and Grayson describe another potential problem with using forked flaps.¹¹ When forked flaps banked in the whisker position are rotated into the columella, the rotation can cause the upper lip to bunch up. Additional tissue excision and reopening of old scars may be necessary to correct this complication. Additionally, it rotates a noticeable midline scar into the columella.

A further complication that may be encountered in single-stage procedures is the loss of a portion of the prolabium. This may occur if its blood supply is compromised by simultaneously separating the prolabium from the premaxilla and performing a nasal tip dissection.

Cutting and Grayson point out that procedures using prolabial skin exclusively to reconstruct the columella and the lateral lip segments to reconstruct the entire lip place tension on the lip and maxilla.¹¹ This tension can lead to midfacial retrusion. They also emphasize the 50% revision rate of their initial 8 patients with prolabial unwinding procedures. The very asymmetric design of the prolabial flap, which allows this repair to be performed with improved blood supply, is the root of the frequent postoperative asymmetry and deviation of the columella to one side. This was thought to be corrected by lowering the flap base off the columella at the initial procedure. Z-plasty was used to correct the asymmetry when it occurred, and this added a horizontal scar to the columellar lip junction.

OUTCOME AND PROGNOSIS

Section 9 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

McComb's experience highlights growth as the fourth dimension in an already complex problem. He found few unfavorable outcomes until the patients reached adolescence. Very few surgeons have the experience of large numbers of patients whose conditions were corrected by a single technique and who were observed over their entire growth phase. Outcome analyses can truly remain valid only in such instances. Therefore, those surgeons with documented long-term results, whether good or bad, are invaluable to those beginning their surgical careers who are faced with bilateral cleft lip nasal deformities.

FUTURE AND CONTROVERSIES

Section 10 of 11  

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

The number and variety of methods for treatment of the bilateral cleft lip nasal deformity indicate the difficult nature of the problem. Perfect anatomic reconstruction is impossible, even if completed within the first few months of life. The possibility of fetal detection and correction of bilateral cleft lip is intriguing, both for the possibility of a scarless repair and for its possible impact on the nasal deformity.

Before the deformity can be repaired in utero, it first must be recognized. Because of the 2-dimensional nature of previous scans, a severe bilateral cleft may be more difficult to recognize on ultrasound images than a unilateral cleft. A protruding premaxilla makes the deformity difficult to recognize. However, Wyberg et al found that an echogenic paranasal mass, which corresponds to the premaxilla, can be seen even before 20 weeks’ gestation. Recently, a 4-dimensional (ie, 3-dimensional in real time video graphics) ultrasound has become available in the authors’ center, and, in the authors’ experience, more prenatal diagnoses are being made.

For fetal surgery to become plausible, the benefits of the surgery must outweigh the risks. For a procedure that is largely cosmetic, the risks must be extremely low. Currently, hysterotomy is considered for life-saving surgery, but the risks incurred are significant. Advanced endoscopic techniques and equipment are being developed and may help reduce the risks of this surgery to an acceptable level. Endoscopes 1 mm in diameter are in use for surgery on the vas deferens. Kirsch and Zhu have developed nonpenetrating microclips that would lend themselves well to this form of surgery.¹⁵

Oberg et al state that for unilateral clefts of the primary and secondary palate, fetal repair offers the possibility of halting the bony deformation. This would lead to a more normal anatomy at birth and a less complicated reconstruction. This could also be applied to bilateral clefts. Repairing the lip in utero may prevent protrusion of the premaxilla. Less shortening of the columella would occur, and a more normal nasal anatomy would be present at birth, allowing for an easier repair.

The timing of the repair also must be considered. Animal studies appear to demonstrate that when the skin is more immature at the time of repair, less scaring is present when the fetus is born. The possibility of inducing preterm labor through surgery must be considered because this would be fatal at an early gestational age.

REFERENCES

Section 11 of 11

• Authors and Editors
• Introduction
• Indications
• Relevant Anatomy
• Contraindications
• Workup
• Treatment
• Complications
• Outcome and Prognosis
• Future and Controversies
• References

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Article Last Updated: Aug 14, 2007