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ORIGINAL ARTICLE
Year : 2015  |  Volume : 28  |  Issue : 1  |  Page : 99-106

Patterns and management of congenital nasal clefts


1 Department of Plastic and Reconstructive Surgery, Menoufia University Hospital, Menoufia, Egypt
2 Department of General Surgery, Menoufia University Hospital, Menoufia, Egypt

Date of Submission10-Jun-2014
Date of Acceptance20-Jul-2014
Date of Web Publication29-Apr-2015

Correspondence Address:
Hossam Hassan Abd Al Raheim Fawzy
30 Mostafa Kamel Street, Al Bar Al Sharky, Shebin El Kom, Menoufia
Egypt
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1110-2098.155960

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  Abstract 

Objective
The aim of the study was to study different patterns of congenital nasal clefts and different methods of their management trying to put forward a road map for their treatment.
Background
Because of the rarity of atypical facial clefts, little is written with respect to patterns and plans of management. This study will spot light on atypical facial clefts affecting the nose (atypical nasal clefts) with respect to different patterns and plans of management.
Patients and methods
This was a retrospective and short prospective study that was conducted in the Department of Plastic Surgery, Menoufia University, from 2001 to 2014. The study included 26 patients with atypical nasal clefts. Those patients were classified according to Tessier classification into Tessier type 0, 1, 2, and 3. All patients were evaluated for the type of facial clefts, pattern of nasal involvement, and associated congenital anomalies. In all, 20 of them underwent surgical repair in one or more stages according to complexity of the deformity. The study describes different plans of reconstruction used for management.
Results
The statistical analysis of data was descriptive and focused on description of different patterns of Tessier type. It showed that the highest incidence was Tessier 0, which included 17 cases found in eight patterns, followed by five cases in Tessier 1 in three patterns, two cases in Tessier type 2 in two different patterns, and two cases in Tessier type 3 in two patterns. The analysis of data showed that the most common associated congenital anomalies were cleft lip and hypertelorism. With respect to management, analysis of data showed the variation in number of operations, timing of first operation, and type of surgical intervention according to complexity and pattern of deformity.
Conclusion
Atypical nasal clefts can be found in a very wide range of patterns, ranging from simple esthetic deformity to complete agenesis or bifidity of the nose. Management of these cases requires good analysis of the pattern of nasal cleft and we provided a treatment algorithm for each pattern.

Keywords: Atypical facial clefts, congenital nasal clefts, craniofacial clefts, nasal deformities, nose reconstruction, tessier clefts


How to cite this article:
Ghareeb FM, Farghaly AA, Al Barah AM, El Sheikh YM, Fawzy HH. Patterns and management of congenital nasal clefts. Menoufia Med J 2015;28:99-106

How to cite this URL:
Ghareeb FM, Farghaly AA, Al Barah AM, El Sheikh YM, Fawzy HH. Patterns and management of congenital nasal clefts. Menoufia Med J [serial online] 2015 [cited 2019 Nov 15];28:99-106. Available from: http://www.mmj.eg.net/text.asp?2015/28/1/99/155960


  Introduction Top


Congenital clefts of the nose whether isolated or associated with more severe craniofacial clefts are fortunately rare; hence, not surprisingly, little is written with respect to operative management in long run and follow-up in patients with nasal clefts. The exact incidence of rare craniofacial clefts is unknown. However, they have been estimated at 1.4-4.9 per 100 000 live births. Comparing rare craniofacial clefts with common clefts, their incidence seems to approximate 9.5-34 per 1000 [1]. Several theories have been proposed to explain the etiology of facial clefts. The most widely accepted theory at this time is the mesodermal penetration theory, which states that clefts occur along potential lines of fusion as lack of mesodermal penetration. Without this reinforcement, epithelial breakdown and separation occur [2].

The most widely accepted classification of craniofacial clefts is Tessier classification, which is proposed by Paul Tessier in 1976 [Figure 1]. This classification is based on specific axes (0-14) along the face and the cranium. The orbit divides the cranial clefts (9-14) from facial clefts (0-7), and facial clefts have cranial extensions. Tessier clefts involving the nose are types 0, 1, 2, and 3 with their cranial counterparts [3]. Patterns of nasal clefts are very variable ranging from simple notch or asymmetry of alar margin to complex complete craniofacial cleft involving the lip, nose, eyelid, brow, forehead, and underlying bones [3].
Figure 1: Tessier classification on the skull and face.

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Because of its rarity, there are no established standardized methods for correction of congenital nasal clefts, and management is tailored for each case using the basic principles of management. Principles of management include precise clinical and radiographic diagnosis. One must first know the normal anatomy and the sense of proper facial proportions and landmarks [1]. Each component of the nose, skin, mucous lining, and support (both bony and cartilaginous) must be evaluated in a qualitative and quantitative manner. Staged reconstruction of patients with complex craniofacial clefts frequently requires multiple-staged reconstructive procedures. Replace like with like, for example, bone defects repaired with bone grafts (calvarial, rib, ilia, etc.). Free cartilage grafts or chondromucosal flaps are used to repair cartilage defects according to size. Adequate nasal lining is also essential to achieve functional and predictable esthetic units; in addition, optimal lining will help vascularity of reconstructed area for small area. Local mucosal flap can be used for larger defects and mucoperichondrial grafts can be used [4].

Hence, the aim of this work was to study different patterns of congenital nasal clefts and different methods of their management trying to put forward a road map for their treatment.


  Patients and methods Top


This was a retrospective and short prospective study that was conducted in the Department of Plastic Surgery, Menoufia University, to study patterns of congenital nasal clefts and different modalities of management. The study included 26 patients who attended our Plastic Surgery Clinic over the period from 2001 to 2014. Inclusion criteria included atypical facial clefts involving the nose (Tessier types 0, 1, 2, and 3) [Table 1]. Exclusion criteria were other congenital nasal deformities such as common cleft lip nasal deformities and maxillonasal dysplasia and other noncongenital nose deformities such as traumatic or neoplastic. The patients were divided into four groups according to the Tessier classification as shown in [Table 1].
Table 1: Classification of the cases under study according to Tessier classification

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All patients were evaluated for type of facial cleft, pattern of nasal involvement, and associated anomalies. In all, 20 of them underwent repair of nasal cleft and associated anomalies if present in one or more stages according to complexity of the deformity.

Clinical evaluation included detailed history including perinatal history and analysis of patient's complaint (or his guardian) to determine whether the problem is esthetic only or functional and esthetic. Family history was also taken to detect similar conditions in the family. Clinical examination included general examination to detect associated congenital anomalies. Local examination included description of nose deformity at level of dorsum (either absent, hypoplastic, broad, or bifid), alae (absent, hypoplastic, depressed, notched, or widely separated), tip (absent, broad, or bifid), septum (absent, hypoplastic, depressed, or bifid), and columellar (absent, depressed, or bifid). The study describes also the associated congenital anomalies such as cleft lip, cleft palate, hypertelorism, and so on. Investigations by radiological examination were performed by three-dimensional computed tomography to assess nasal bones, facial bony clefts, alveolar clefts, or hypertelorism. Other radiological investigations were performed to detect associated congenital anomalies such as echocardiography. Routine laboratory investigations were also performed to assess patient's general condition.

Surgical management

Number of operations and type of surgical intervention vary widely according to pattern and complexity of the deformity. Surgical intervention aims for restoration of nasal skeleton, both bony and cartilaginous, skin coverage, and mucous lining to regain normal nasal structure giving normal esthetic look and normal function (airway).

Restoration of skin coverage was performed by either local flaps (V-Y or Z plasties), expanded forehead flap, or skin trimming and refashioning. The planning of skin flap depends on the amount of skin needed for skin coverage and mucous lining aiming for moving skin from areas of excess to areas of deficiency. Reconstruction of nasal bone was performed by either bone graft or medialization of bifid nasal bone during hypertelorism repair. The preferred donor site for bone graft in this study was iliac bone graft due to its easy harvesting, hidden donor site, and bulkiness. The harvested bone graft was shaped according to pattern of deformity as in cases of absent the bone graft shaped as pyramidal shape of nasal bone. Cartilaginous framework was reconstructed by cartilage grafts either costal or conchal according to the amount of cartilage needed and site to be positioned in - for example, if rigid cartilage is needed for columellar strut costal cartilage is used and if rim of cartilage is needed for alar shaping conchal cartilage can be used. Mucous lining was restored using folding of local skin flaps. In each operation, usually reconstruction of one or more elements is performed. Repair of associated congenital anomalies was performed side by side to repair nasal clefts - for example, repair of cleft lip or hypertelorism.

In the study, we spot light also on the management of hypertelorism associated with atypical nasal cleft because it is considered a stage in management of atypical nasal cleft. In this study, we used two methods for hypertelorism repair. The first is orbital translocation, which is performed through bicoronal incision, dissection to expose both orbits followed by osteotomy of all four walls of both orbits, and repositioning of the orbits is then performed. During this repair, correction of the bifid nasal bones is performed [Figure 4].

The second method is orbital medialization, which is performed also through bicoronal incision and dissection to expose both orbits followed by osteotomy only of the interorbital segment followed by medialization of two medial orbital walls and repositioning of the interorbital segment to decrease interorbital distance. In addition, correction of bifid nasal bones is performed during this repair [Figure 3].

Follow-up of the patients was performed over a period of 6 months from the last operation. It included guarding against complications of nose or donor site, such as hemorrhage and infection. Assessment of results was performed esthetically by patient's and doctor's satisfaction and functionally by assessment of patent nostril airways.


  Results Top


The data from the 26 patients were either collected retrospectively from hospital records, photographic records, and patient's history or collected prospectively by clinical assessment, surgical management, and follow-up of the patient. These data underwent statistical analysis descriptive type.

The statistical analysis included the incidence with respect to the sex and presence of risk factors such as perinatal problems or drug intake. Thereafter, we come to study patterns of atypical nasal clefts after the patients were divided according to Tessier classification into Tessier 0, 1, 2, or 3. Each type was studied with respect to patterns of nasal cleft; each pattern describes the deformities present in dorsum, tip, septum, alae, and columellar [Table 2]. The study also spots light on associated congenital anomalies and their incidence.
Table 2: Patterns of each type of nasal clefts

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The methods of surgical intervention were classified into skin management, and we describe different methods used to deal with skin deficiency or excess, bone management to describe options used to restore bony skeleton, cartilage management also to restore cartilaginous framework, and options for providence of mucous lining was needed. Finally, it also included the surgical intervention for associated congenital anomalies. Follow-up results were esthetically with respect to patient's satisfaction and doctor's satisfaction and functionally by patency of nostrils.

The plan of management differs according to the pattern of nasal cleft. The treatment algorithm described in [Table 3] describes the management plan for each pattern of congenital nasal cleft [Figure 2],[Figure 5],[Figure 6],[Figure 7] and [Figure 8].
Figure 2: Case 1 presentation: female patient with Tessier 0 in the form of broad tip and depressed columellar. This case was treated by costal cartilage graft to augment nasal tip and columellar lengthening by V– Y flap and columellar strut. (a) Preoperative frontal view; (b) postoperative frontal view.

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Figure 3: Case 2 presentation: female patient with Tessier 0 in the form of hypoplastic bifi d dorsum hypoplastic alae associated with hypertelorism. This case was treated by orbital medialization. (a) Preoperative frontal view; (b) preoperative measuring of intercanthal distance (=50mm); (c) three-dimensional (3D) computed tomography (CT) shows the hypertelorism and bifi d hypoplastic nasal bones); (d) intraoperative view: exposure of orbits and nasal bones through bicoronal incision; (e) intraoperative view: osteotomy of the interorbital segment); (f) postoperative frontal view; (g) postoperative intercanthal distance (=30 mm); (h) postoperative 3D CT shows decrease interorbital distance and correction of nasal bone bifidity.

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Figure 4: Case 3 presentation: male patient with Tessier 0 in the form of bifid dorsum and widely separated hypoplastic alae corrected in three stages: first, by Z plasty to approximate two halves of nose; second, correction of hypertelorism by orbital translocation; and third stage was bone grafts to augment nasal bone. (a) Preoperative frontal view; (b) approximation of separated alae by Z plasties); (c) after approximation of two nasal halves; (d) planning of hypertelorism repair by orbital translocation); (e) orbital translocation and medialization of nasal bones; (f) after hypertelorism repair and bone graft.

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Figure 5: Case 4 presentation: female patient with Tessier 0 in the form of absent nasal dorsum with hypoplastic alae associated with median cleft lip and alveolar cleft. Repair was performed in two stages: first, by iliac bone graft to create nasal dorsum and second, by costal cartilage grafts to create cartilaginous framework of the nose. (a) Preoperative frontal view; (b) preoperative lateral view; (c) three-dimensional computed tomography shows the hypoplastic nasal bone and alveolar cleft; (d) intraoperative view: during repair of cleft lip creation of a pocket was done to hold iliac bone graft; (e) intraoperative view: harvesting iliac bone graft pyramidal in shape; (f) postoperative after fi rst stage; (g) intraoperative view: harvesting costal cartilage graft); (h) intraoperative view: using the cartilage grafts to create cartilage framework by dividing it into segments for both alae and columellar strut; (i) postoperative after the second stage frontal view; (j) postoperative after the second stage lateral view.

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Figure 6: Case 5 presentation: male patient with Tessier 0 associated with frontoethmoidal meningocele repaired by excision of meningocele by double approach, external and intracranial, and closure of the defect by iliac bone graft and excision of the abnormal skin followed by refashioning of remaining skin around gathering ring. (a) Preoperative frontal view; (b) preoperative lateral view); (c) coronal computed tomography show the frontoethmoidal meningocele; (d) intraoperative view: the external approach; (e) intraoperative view: the intracranial approach; (f) intraoperative view: the bone defect in the roof of the nose; (g) intraoperative view: closure of the defect by bone graft and excision of abnormal expanded skin; (h) intraoperative view: refashioning of remaining skin around a gathering ring; (i) postoperative frontal view.

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Figure 7: Case 6 presentation: male patient with nasal cleft Tessier 2 in the form of hypoplasia of right ala and ipsilateral side of dorsum with notch in medial 1/3 of ala. This case was managed in seven stages in the form of local Z flaps and repeated bone and cartilage grafts. (a) Preoperative frontal view; (b) preoperative occipitomental view; (c) intraoperative view: show local flap Z plasty to correc t skin defi ciency; (d) intraoperative view: after positioning of the local fl aps and input of iliac bone graft); (e) postoperative view after repeated bone and cartilage grafts to restore normal aesthetic anatomy; (f) postoperative oblique view.

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Figure 8: Case 7 presentation: female patient with Tessier 3 nasal cleft in form of complete hypoplasia of the right half of nose associated with anophthalmia managed in six steps in form of expanded forehead fl ap and repeated bone and cartilage grafts. Anophthalmia has been treated by creation of a pocket for artifi cial eye. (a) Preoperative frontal view; (b) After insertion of tissue expander in the forehead; (c) intraoperative view : using the expanded forehead fl ap to create skin coverage and mucous lining for left half of the nose; (d) intraoperative view: positioning of iliac bone graft to create dorsum of the nose after separation of the forehead flap; (e) postoperative view: after the stages of forehead flap and bone graft; (f) postoperative after other three stages of bone and cartilage grafts to restore normal aesthetic anatomy of the nose.

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Table 3: Treatment algorithm for different patterns of congenital nasal clefts

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  Discussion Top


Congenital craniofacial clefts are abnormal disfigurements of the face and cranium with deficiencies, excesses, or even a normal (but separated) amount of tissue occurring along linear regions. Of all congenital facial anomalies, craniofacial clefts are among the most disfiguring. They may be seen in a variety of patterns and varying degrees of severity [3]. In the largest series reported, Ortiz Monasterio [5] reviewed 6500 clefts treated over 20-year period; only 2.2% (146 patients) had nasal clefts corresponding to types 0, 1, 2, and 3 of Tessier classification, distributed as follows: 92 patients had Tessier 0 (63%), 16 patients had Tessier 1 (10.9%), 23 patients had Tessier 2 (15.7%), 15 patients had Tessier 3 (10.3%).

In this study that was conducted in Menoufia University Hospital over 11 years (2003-2014), the incidence of atypical nasal clefts (including Tessier 0, 1, 2, and 3) was 26 cases, whereas the average incidence of all craniofacial clefts was 495 cases during the period of study. This means that incidence of atypical nasal clefts is 5.2% [5].

The 26 cases of atypical nasal clefts were divided according to Tessier classification as follows: 17 cases in the Tessier 0 group, five cases in the Tessier 1 group, two cases in the Tessier 2 group, and two cases in the Tessier 3 group. The study showed that 34.6% of the studied group was men and 65.4% was women. The study showed some risk factors during perinatal period; five cases gave history of drug intake (three analgesics and two vitamins), two cases gave history of prolonged labor, and three cases had similar conditions in the family. The majority of cases showed no definite etiology during perinatal period.

The study showed that each type of Tessier clefts has variable patterns as follows: in Tessier 0 there were group were 17 cases. They showed the following variation: two cases of broad tip only, three cases of broad bifid dorsum and depressed flattened alae associated with hypertelorism, two cases of hypoplastic bifid dorsum, three cases of bifid dorsum and separated hypoplastic alae, one case of hypoplastic bifid dorsum and frontoethmoidal meningocele, one case of absent nasal bone with normal tip and alae, and two cases of absent nasal bone with hypoplastic widely separated alae. In Tessier 1 group there were five cases, which varied as follows: two cases of notch in medial 1/3 of alae with normal dorsum, one case of notch in medial 1/3 of alae with hypoplasia of ipsilateral dorsal bone, and two cases notch in medial 1/3 of alae with broad dorsum. Tessier 2 group included two cases, one of them had hypoplasia involving one alae and ipsilateral dorsum with notch in middle 1/3 of alae and the other case had hypoplasia of one alae and ipsilateral dorsum. Tessier 3 group included two cases, one of them showed absent one side of nose and the other case showed complete hypoplasia of one side of nose [Table 2].

The incidence of associated congenital anomalies in the cases under study was:

  1. Associated typical clefts: seven cases had cleft lip, four cases had cleft palate, and five cases had alveolar clefts;
  2. Associated eye anomalies: unilateral anophthalmia in one case;
  3. Other craniofacial skeletal anomalies: hypertelorism (in eight cases), frontal bone hypoplasia (in one case), bone defect with meningocele (in four cases); and
  4. Eyelid defects: epicanthal fold (one case), coloboma of medial 1/3 of eyelid (in three cases), and ptosis (in two cases).


The number of operations ranges according to complexity of the deformity, associated congenital anomalies, and patient and doctor satisfaction. The number of operations ranges from 1 to 7 (during the period of study); the mean number of operations was 2.35 for atypical nasal cleft repair. Timing of first operation ranges from 2 to 18 years according to severity of the deformity and patient complaint. For example, patients with bone defect absent or hypoplastic management initiated early during first 5 years of life in form of bone graft to expand the nasal skin to allow further modeling and reconstruction of nose.

After determining the type and form of deformity, we decide the plan of management and sequence of reconstructive steps; the goals of reconstruction are to restore the normal esthetic anatomy and normal function of the nose; the reconstruction involves skeletal framework of the nose (bony or cartilaginous), skin coverage, and mucous membrane lining. Repair of congenital nasal clefts cannot be performed regardless of associated anomalies such as hypertelorism or cleft lip. For example, in repair of hypertelorism the medialization of nasal bone is performed, which is considered an important step in repairing nasal cleft.

Types of interventions for skin reconstruction were in the form of local flaps in seven cases, as V-Y flap in three cases, and Z plasties in four cases. These flaps are designed to move skin from areas of skin excess to areas of skin deficiency. In cases in which the skin deficiency is large as in cases of anaplasia of half nose (Tessier 3), the repair of skin management is performed by expanded forehead flap, which was performed in two cases. Inner lining can be made by folding of local flap used for skin coverage in two cases or folding of forehead flap in two cases. Nasal bone cleft may be in the form of anaplasia or hypoplasia, if one or both nasal bones or bifid nasal bones or hypoplastic and bifid nasal bones are present. In cases with bone defect either hypoplasia or anaplasia, bone grafts were performed in seven patients. We preferred in all the seven cases the donor to be iliac bone as it is characterized by bulkiness and hardness so that the harvested bone segment can be easily modeled to take the desired shape for missed bone from the nose. In addition, harvesting iliac bone graft is characterized by easy access and hidden scar. In cases with bifid nasal bone without hypoplasia, the deformity can be corrected with medialization of nasal bone and there is no need for bone graft, and if this is associated with hypertelorism the medialization of nasal bone is considered a step in hypertelorism repair operation. Cartilaginous part of nasal framework is frequently involved in the atypical nasal cleft in the form of notch in alar cartilage or in form of anaplasia or hypoplasia. Restoring normal esthetic anatomy usually needs cartilage graft; the costal cartilage was the donor in eight cases and conchal cartilage was the donor in two cases. In most cases, the atypical nasal cleft was associated with short columella. The columellar lengthening is performed either by V-Y flap alone in two cases or V-Y flap with cartilage graft in six cases or composite conchal graft in three cases [Table 3].

The management of associated craniofacial anomalies is closely related to management of atypical nasal cleft. Hypertelorism management was performed by orbital translocation in three cases and by medialization of orbital wall in one case. During hypertelorism repair, medialization of nasal bone is performed. Correction of frontoethmoidal meningocele is performed, corrected in same operation of correction of nasal cleft by double approach, intracranial and nasal approaches; bone grafts are used to close the defect and for excision of the expanded skin. Cleft lip repair during repair of median cleft lip columellar lengthening is performed and labial flaps are used to close nasal floor.

The esthetic outcome of surgical interference was assessed by patient's satisfaction and doctor's satisfaction. According to patient's satisfaction, 17.6% had excellent result, 70.6% had good results, and 11.7% had poor results. According to doctor's satisfaction, 17.6% had excellent results, 58.8% had good results, and 17.6% had poor result.


  Conclusion Top


On the basis of this study, the congenital nasal clefts have variable patterns ranging from simple to complex deformities. To restore normal esthetic anatomy of the nose, this needs proper evaluation of the deformity and accordingly planning the management. We come to a treatment algorithm to facilitate decision of plan of management for each pattern of congenital nasal clefts.


  Acknowledgements Top


Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Kawamoto HK Jr. Rare craniofacial clefts, plastic surgery. Philadelphia, PA: Saunders; 1990. 4 :2922-2973.  Back to cited text no. 1
    
2.
Shewmake KN, Kawamoto HK. Congenital clefts of the nose: principles of surgical management. Cleft Palate Craniofac J 1992; 29 :531-539.  Back to cited text no. 2
    
3.
Tessier P. Anatomical classification of facial, cranio-facial and latero-facial clefts. J Maxillofac Surg 1976; 4 :69-92.  Back to cited text no. 3
    
4.
Drake R, Vogl AW, Mitchell AWM. Nasal cavity, Gray′s anatomy for students. 2 nd ed. Elsevier; 2007. 8 :965-985.  Back to cited text no. 4
    
5.
Ortiz Monasterio F, Fuente del Campo A, Dimopulos A. Nasal clefts. Ann Plast Surg 1987; 18 :377-397.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]
 
 
    Tables

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