**3. Clinical signs and symptoms**

A variety of clinical signs may be seen in an operated cleft lip and palate patient as well as in unoperated cases. We are usually faced with a wide variety of signs and symptoms in a repaired cleft lip case with or without primary rhinoplasty. In this section the clinical signs of cleft nose deformity will be discussed.

#### **3.1. Unilateral cleft lip nose deformity**

Clinical features of cleft nose deformity from a cosmetic point of view have varied from minor to severe (Figure 1).

It is not easy to accurately describe the anatomic pathology of secondary CLND. Components of the nasal deformity include defects of all layers of skin, cartilages, septum, entire nasal pyramid as well as hypoplasia and mal positioning of the maxillary segments and the anatomic

**Figure 1.** Basal view of minor and two difficult cases of unilateral cleft nose.

and functional deformity of the orbicularis muscle. It is accepted that patients who undergo appropriate primary repair for cleft lip will have secondary deformities [8]. Recently many three dimensional studies have been performed on cleft lip nose deformity patients; thanks to advances in technology we can define the details of the anatomic and functional deformity of each component [10]. The cleft deformity is not restricted to the skin and cartilage. Hogan represented the unilateral cleft nasal deformity as a tilted tripod (Figure 2). **Figure.1: Basal view of minor and two difficult cases of unilateral cleft nose.** It is not easy to accurately describe the anatomic pathology of secondary

CLND. Components of the nasal deformity include defects of all layers of

**Figure 2.** Tilted nasal tripod in CLND technology we can define the details of the anatomic and functional

CLND presents a unique challenge due to complexity and combination of aspects and certain

Primary rhinoplasty means performing rhinoplasty simultaneously with repair of cleft lip and secondary rhinoplasty means performing rhinoplasty at an early age i.e. during school going age(5-6 years), early adolescence (10-12 years) or later on in life (above 16 yrs. in women and age 18 yrs. in men). But as a working diagnosis, primary rhinoplasty in adults with CLND means the first attempt of surgical intervention on the nose and secondary rhinoplasty means

The best time to attempt correction of CLND is still controversial. With improvement in cleft lip surgery, there is an increasing interest for correction of the nose at the time of lip repair. Some authors strongly recommend a primary rhinoplasty and believe if the procedure is performed correctly it does not adversely affect the growth of the paranasal region. Primary rhinoplasty improves nasal symmetry in patients with unilateral cleft lip deformity. This does not exclude the possibility of later revisional surgery although there is tendency to doing an appropriate primary repair of cleft lip deformity but the fact is, small defects that are left after primary repair are amplified with the growth process and affect adjacent structures. It is become clear that primary and secondary rhinoplasty at the same time of lip repair or at age 7-8 can lead to some kind of deformities in adulthood. The adult deformity is related not only to the original embryological mesodermal deficiency and diminished growth potential, but also to the pattern of primary surgery, the degree of interceptive surgery during growth and the level of orthodontic skill practiced within a particular treatment. In our center most cases

A variety of clinical signs may be seen in an operated cleft lip and palate patient as well as in unoperated cases. We are usually faced with a wide variety of signs and symptoms in a repaired cleft lip case with or without primary rhinoplasty. In this section the clinical signs of

Clinical features of cleft nose deformity from a cosmetic point of view have varied from minor

It is not easy to accurately describe the anatomic pathology of secondary CLND. Components of the nasal deformity include defects of all layers of skin, cartilages, septum, entire nasal pyramid as well as hypoplasia and mal positioning of the maxillary segments and the anatomic

techniques that may be more suitable than others in individual cases [1, 2].

**2. Primary or secondary rhinoplasty**

786 A Textbook of Advanced Oral and Maxillofacial Surgery Volume 2

revision rhinoplasty or second operation on the nose.

are referred for primary rhinoplasty in adulthood [3-9].

**3. Clinical signs and symptoms**

cleft nose deformity will be discussed.

**3.1. Unilateral cleft lip nose deformity**

to severe (Figure 1).

Because of the lack of skeletal support, the alar base on the cleft side can, in some cases, become retro positioned with growth, even following an appropriate primary correction [9]. In most cases however good the primary correction, the patient is left with asymmetry of the nasal base and nares [11]. deformity of each component [10]. The cleft deformity is not restricted to the skin and cartilage. Hogan represented the unilateral cleft nasal

The ala on the cleft side is lengthened vertically and lies below the alae on the non- cleft side. The lower lateral cartilage is depressed and spread across the cleft. The nasal tip is deviated toward the left side. The columella on the left side is shortened significantly, as compared with the non-cleft side. The columella is obliquely oriented. With its base deviated to and located in the non-cleft side away from the midline. Bilateral alar bases are asymmetric, with the cleft side alar base inferiorly and posteriorly displaced. [12] deformity as a tilted tripod [**Figure 2**].

The deformities such as a deficient tubercle, vermilion deficiency, irregularities, the short upper lip, long upper lip, tight upper lip, and unfavorable scars may be common seen in the repaired lip [9]. Also the severity of septal deformity is variable. Typically, the septum is dislocated from the maxillary crest towards the non-cleft side resulting in a septal deflection towards the cleft side commonly causing nasal obstruction on that side. In addition, the inferior turbinate on the cleft side is also frequently hypertrophic, further adding to nasal obstruction on that side. [13] Lee [8] described seven cardinal deformities in unilateral cleft lip nose deformity include: Discontinuity of the orbicularis oris muscle


Although Huffman and Lierle in 1949 published the most detailed descriptions of the cleft lip nasal deformity, over time it has changed; the typical clinical features of the unilateral cleft nasal deformity (Figure 3) is characterized as follow:

**Figure 3. Typical deformity in unilateral cleft nose deformity Figure 3.** Typical deformity in unilateral cleft nose deformity


repaired lip [9]. Also the severity of septal deformity is variable. Typically, the septum is dislocated from the maxillary crest towards the non-cleft side resulting in a septal deflection towards the cleft side commonly causing nasal obstruction on that side. In addition, the inferior turbinate on the cleft side is also frequently hypertrophic, further adding to nasal obstruction on that side. [13] Lee [8] described seven cardinal deformities in unilateral cleft lip nose

Discontinuity of the orbicularis oris muscle

Although Huffman and Lierle in 1949 published the most detailed descriptions of the cleft lip nasal deformity, over time it has changed; the typical clinical features of the unilateral cleft

**Figure 3. Typical deformity in unilateral cleft nose deformity**

**1.** The tip of the nose and caudal septum are deviated towards the non-cleft side.

**3.** The convexity of the septum on the side of the cleft impinges on the airway.

**4.** The angle between the medial and lateral crura on the cleft side is excessively obtuse.

**6.** The interior of the cleft-side nostril, from its apex down the cephalic margin of the alar cartilage to the pyriform aperture, is bowed by a linear contracture—the vestibular web.

1. The tip of the nose and caudal septum are deviated towards the non‐

2. The base of the columella also deviates towards the non‐cleft side.

Although Huffman and Lierle in 1949 published the most detailed descriptions of the cleft lip nasal deformity, over time it has changed; the typical clinical features of the unilateral cleft nasal deformity [**Figure 3**] is

**•** Caudal deflection of the nasal septum to the non-cleft side

Long or short lip deformity

Absence of the philtral column

deformity include:

**•** Deviation of the nasal dorsum **•** Low setting of the medial crus

**•** Long or short lip deformity

**•** Absence of the philtral column

**•** Tethering deformity of the lateral crus

**•** Discontinuity of the orbicularis oris muscle

788 A Textbook of Advanced Oral and Maxillofacial Surgery Volume 2

nasal deformity (Figure 3) is characterized as follow:

characterized as follow:

cleft side.

**5.** The dome of the alar cartilage on the cleft side is depressed.

**7.** The lateral crus is caudally displaced on the cleft side.

**2.** The base of the columella also deviates towards the non-cleft side.

**Figure 3.** Typical deformity in unilateral cleft nose deformity


#### **3.2. Bilateral cleft nose deformities**

Adult patients with bilateral cleft lip nose deformity (BLCND) show some asymmetry. Typical characteristics of BLCND include: a short columella, a short, depressed, thick nasal tip, flat– appearing nasal tip, sometimes notched in the midline, large diverging nostrils, wide nostril floors, an obtuse columella-labial angle, wide alae, a short nasal bridge, and a wide nasal root, lateral displacement of both alar domes with bilateral dislocation of the lateral crura from the septum, hooding of the alar rims and flaring alar bases.The short columella is the most common problem in bilateral cleft nose deformity. Although the residual deformity in bilateral cleft is symmetrical correction of nasal tip widening, the retro- positioning of the alar cartilage and shortening of the columella are all encountered and difficult to treat. The secondary deformity involves the nose and the lip as well as the facial profile (Figure 4) [12, 15, and 16]. and difficult to treat. The secondary deformity involves the nose and the lip as well as the facial profile (**Figure 4)** [12, 15, and 16].

**Figure 4.** Preoperative views A: Frontal view B: lateral view

**4‐ Diagnosis**

## **4. Diagnosis**

**Figure 4: Preoperative views A: Frontal view B: lateral view** In order to treat CLND, clinical diagnosis and complete knowledge of anatomy, pathology and physiology of the nasal pyramid, maxilla, and lip is imperative. Clinical examination

grown adult we need to evaluate:

1‐The nose and lip

2‐ Midface deficiency

3‐ Oro‐ nasal fistula

4‐Occlusion and

In order to treat CLND, clinical diagnosis and complete knowledge of

anatomy, pathology and physiology of the nasal pyramid, maxilla, and lip is

imperative. Clinical examination consists of a careful examination of the

bony and cartilaginous skeleton, anterior rhinoscopy evaluating the

appearance of the nasal mucosa and the position of the anterior part of the

septum is necessary. The problems present in a patient with a cleft lip nasal

deformity must be recognized just as any other rhinoplasty patient and

clearly defined in order to formulate a successful treatment plan. In fully

grown adult we need to evaluate:

**4‐ Diagnosis**

consists of a careful examination of the bony and cartilaginous skeleton, anterior rhinoscopy evaluating the appearance of the nasal mucosa and the position of the anterior part of the septum is necessary. The problems present in a patient with a cleft lip nasal deformity must be recognized just as any other rhinoplasty patient and clearly defined in order to formulate a successful treatment plan. In fully grown adult we need to evaluate: 2‐ Midface deficiency 3‐ Oro‐ nasal fistula 4‐Occlusion and

**e 4: Preoperative views A: Frontal view B: lateral view**

In order to treat CLND, clinical diagnosis and complete knowledge of

anatomy, pathology and physiology of the nasal pyramid, maxilla, and lip is

imperative. Clinical examination consists of a careful examination of the

bony and cartilaginous skeleton, anterior rhinoscopy evaluating the

appearance of the nasal mucosa and the position of the anterior part of the

septum is necessary. The problems present in a patient with a cleft lip nasal

deformity must be recognized just as any other rhinoplasty patient and

clearly defined in order to formulate a successful treatment plan. In fully


Significant improvement in growth, function and esthetics has been achieved by almost normal reconstruction of alveolar clefts.To establish the nasal skeletal base, three dimensional reconstruction of alveolar defects with bone grafting has been advocated by clinicians; different approaches at the different stages of life have been suggested (Figure 4). the nasal skeletal base , three dimensional reconstruction of alveolar defects with bone grafting has been advocated by clinicians; different approaches at the different stages of life have been suggested [**Figure 4**].

**Figure 4:**

**Intraoperative**

**Figure 5.** Intraoperative view of 3D reconstruction of alveolar cleft

Proper jaw relationship plays an important role in the skeletal base of the nose; obviously, final rhinoplasty must be postponed until completion of orthognathic surgeries. Although some authors suggested performing orthognathic surgery on growing cleft patients when mandated by psychological and / or functional concerns; but, because of postsurgical outcome the consensus of most clinicians is to delay orthognathic surgery until growth is completed. Different kinds of distraction procedures such as intraoral and extraoral devices may be used as an alternative to the orthognathic approaches in cleft patients; a proper position of the maxilla is mandatory before performing rhinoplasty in adults with cleft lip and nose deformity. Supposedly, the skeletal support enhances the projection of the lip and nose on the cleft side.

CT scan of paranasal sinuses in axial and coronal views may be helpful to define the defor‐ mation of the septum as well as other intranasal structures. It is important to identify both aesthetic and functional problems associated with the cleft nose deformity. Each component of the deformity must be addressed in an orderly manner including the skeletal base, nasal dorsal bone and cartilage, nasal tip cartilage, and, finally, the skin envelope [1, 8, 11, 17-21].
