Repair of Bilateral Cleft Lip

Thesis submitted for fulfillment of M.Sc Degree In surgery

By

Dr. Dawlat Emara Gomaa MB.BCh Cairo University

Under supervision of

Prof. Dr. Ahmed Gamil El-Sharkawy Professor of Plastic surgery

Cairo University

Prof. Dr. Ahmed Tarek Atta Professor of Plastic surgery

Cairo University

Prof. Dr. Mamdouh A. Aboul-Hassan Assistant Professor of Pediatric surgery

Cairo University

Faculty of Medicine Cairo University

2009

 

2

بسم االله الرحمن الرحيم

و قـالوا سبحانك لا علم لنا إلا ما علمتنا إنك أنت العليم الحكيم صدق االله العظيم

 

3

Acknowledgement

First of all, I would like to thank Allah who gave me the strength to accomplish this work. I have been honored and delighted to work under the supervision of such distinguished and eminent professors. I would like to express my feelings of gratitude and respect that I carry to Prof. Dr. Ahmed Gamil El- Sharkawy, Professor of Plastic and General surgery, Faculty of Medicine, Cairo University, who had patiently guided and supervised my work. It has been a pleasure to proceed with this thesis under his supervision and guidance. Words would not convey my feelings of appreciation and gratitude towards Prof. Dr. Ahmed Tarek Atta, Professor of Plastic and General surgery, Faculty of Medicine, Cairo University, for his continuous advice and close supervision. I would like finally to thank and am grateful to Ass. Prof. Dr. Mamdouh A. Aboul Hassan, Professor of Pediatric surgery, Faculty of Medicine, Cairo University, for his patience, sincere advice, valuable assistance and spending with me much of his precious time to help me finish and to reach the best of this work.

 

4

Abstract

Approximately one in 1000 children is born with a cleft palate, cleft lip, or with both anomalies. Although bilateral complete cleft lip and palate is the least common of the upper lip clefts, it has severe consequences for both the physical and psychological progress of the infantile development.

A multidisciplinary team approach is essential to the care of a child with a cleft lip. The team should consist of a surgeon, pediatrician, geneticist, otolaryngologist, speech therapist, orthodontist & audiologist. A detailed history includes the family history of cleft lip and/or palate, prenatal maternal exposures (alcohol, smoking & drugs).

There are different approaches for the management of bilateral cleft lip and palate. Presurgical orthodontic nasoalveolar molding, lip adhesion, one stage or two stages repair. There is a lot of debate about the ideal treatment for bilateral cleft lip.

Care of patients with bilateral cleft lip and palate needs a dedicated team, presurgical nasoalveolar molding facilitates the lip closure and improves nasal projection and symmetry and should be tried whenever possible. Long term follow up and educating of the parents for the deformity and its consequences is necessary to achieve optimum results.

Key Words:

Nasoalveolar molding.

Multidisciplinary team.

Long term follow up.

Family history.

 

5

List of Contents

Title Page

Introduction and Aim of work 1

Embryology of head and neck 2

Anatomy of the lip 5

Anatomy of bilateral cleft lip 12

Epidemiology 18

Preoperative assessment 22

Historical surgical aspects 31

Principles of surgical repair 33

Bilateral cleft lip repair methods 37

Secondary deformities of cleft lip and nose 47

Patients and methods 59

Results 67

Discussion 78

Summary 84

References 85

Arabic summary

 

6

List of figures

Figure No

Page

1. Early embryo 4 weeks. 2

2. Embryo in 5 weeks. 4

3. Muscles of the face. 6

4. Arrangement of the fibers of the Orbicularis Oris. 8

5. Bilateral incomplete cleft lip only. 13

6. Bilateral asymmetrical lip and alveolus. 14

7. Noordhoff’s point (point 6). 15

8. Bilateral complete cleft lip and palate. 17

9. Cleft plan. 23

10. Nasoalveolar molding device. 25

11. Manchester repair. 39

12. Modified Tennison repair. 40

13. Millard rotation advancement approach for the incomplete

bilateral cleft lip.

41

14. Millard repair in complete bilateral cleft lip. 42

15. Mulliken repair. 44

16. Noordhoff repair. 45

17. Nasoalveolar molding. 48

18. Malalignment of vermilion. 49

19. Repair of vermilion deficiency. 50

20. Repair of whistle deformity. 51

21. Repair of short prolabium. 54

22. Abbe flap technique. 55

23. Lip adhesion. 61

24. Reference points & incision markings for bilateral cleft lip repair. 62

25. Incisions performed. 63

 

7

26. Nasal floor and mucosal layer from lateral lip segment are closed. 64

27. Muscle layer closure. 64

28. Final skin closure. 65

29. Wound infection. 67

30. Case 1 incomplete bilateral cleft lip. 69

31. Case 2 complete bilateral cleft lip. 70

32. Case 3 Asymmetrical bilateral cleft lip. 71

33. Case 4 Asymmetrical bilateral cleft lip after nasoalveolar

molding.

72

34. Poor results. 73

35. Case 5 Asymmetrical bilateral cleft lip. 74

36. Case 6 complete bilateral cleft lip after lip adhesion. 76

37. Case 7 complete bilateral cleft lip after lip adhesion. 77

 

8

List of tables

Table no

Page

1. Table 1 19

2. Table 2 20

3. Table 3 68

4. Table 4 75

 

9

INTRODUCTION

 

10

Introduction

The treatment of patients with bilateral cleft lip is more difficult than

treatment of patients with unilateral cleft lip, especially when a bilateral cleft lip is accompanied by bilateral cleft of the alveolus and palate (Salyer 1991). It is often quoted that compared to unilateral cleft “bilateral cleft is twice as difficult, and the results are half as good”. The new generation emphasis on the primary correction of the cartilaginous deformity that is present in bilateral cleft lip and nose (Cutting 2007). The old staged labial repairs, one side and later the other, have been replaced by simultaneous lip closure. For nasal correction, most surgeons no longer believe that the columella is deficient, and thus there is no need to recruit tissue from the lip or nostril sills as a secondary procedure. (Mulliken 2003) The columella is concealed in the nose. The new strategy is to construct the columella and nasal tip by anatomic positioning of the alar cartilages and sculpting the investing skin. Furthermore, nasal correction is done at the time of bilateral labial repair and, whenever possible, the alveolar clefts are closed as well. (Mulliken 2001) Aim of the work It is to evaluate the outcome of repair of patients with bilateral cleft lip.

 

11

EMBRYOLOGY OF HEAD AND NECK

 

12

Embryology of Head and Neck

The cranial end of the human embryo undergoes precocious development

beginning in the middle of the third week. This development of the cranial

portion of the embryo results in the head which constitutes nearly one half of

total body length from the fourth to eighth week of development. During the

fourth week of development, the cranial region of the human embryo resembles

a fish embryo at a comparable stage. ( Nomina Embyrologica 1989)

Branchial Apparatus:

The term branchial is often used to describe the embryonic arches. Early

in the fourth week, branchial arches begin to develop from the connective and

muscle tissue elements of the neural crest. Differentiation of those neural crest

cells that migrate ventrally and caudally is induced by contact with the

pharyngeal endoderm. The pharyngeal endoderm and initial mesodermal core

surround the six aortic arch arteries. Contact between the pharyngeal endoderm

and migrating neural crest cells results in a series of mesenchymal swellings

that constitute the branchial arches. The initial mesodermal core of each arch

gives rise to muscle myoblasts, while the neural crest cells give rise to skeletal

and connective tissues. (Fig. 1)

Fig. (1) Early embryo 4 weeks. (Peter Williams 1980).

 

13

Each branchial arch contains four essential tissue components:

1- Cartilage: this is a central rod that gives origin to bony, cartilaginous and

ligamentous structures.

2- Aortic arch arteries: these arteries course through the pharynx, joining

the heart, which is ventrally located, to the aorta, which is dorsally located.

3- Nerve: these compromise both sensory and motor fibers from the

respective cranial nerves.

4- Muscle.

The branchial arches are separated by a series of clefts, which are termed

“branchial grooves” on their external aspect and “pharyngeal pouches” on their

internal aspect. With the exception of the dorsal end of the first branchial

groove, all remaining branchial grooves are obliterated during embryonic

development. The second, third, and fourth branchial grooves are obliterated

within the sinus, which develops as a result of caudal overgrowth of the second

branchial arch. The cervical sinus appears as an ectodermal depression caudal to

the third branchial arch. During the fifth week the second branchial arch

overgrows the caudal arches, leaving a small opening into the cervical sinus.

The second to fourth branchial grooves and the cervical sinus are obliterated by

the end of the seventh week, producing a neck with a smooth contour.

(Arun 2007)

Facial development

The branchial arches are largely responsible for the formation of the face,

neck, nasal cavities, mouth, larynx, and pharynx. The first branchial arch

contributes to the maxillary and mandibular prominences and the anterior

portion of the auricle. The paired maxillary and mandibular prominences

derived from the first arch form the lateral and caudal borders of the stomodeum

(primitive mouth), respectively. The frontonasal prominence, a central process

formed by proliferation of the mesenchyme ventral to the forebrain, forms the

cranial boundary of the stomodeum (Fig. 2)

 

14

Facial development largely occurs between the fourth and eighth weeks, and

the face has a clearly human appearance by age 10 weeks. Fusion of the medial

nasal, lateral nasal, and maxillary prominences produces continuity between the

nose, upper lip, and the palate. (Moore 1988)

Fusion of the medial nasal and maxillary prominences results in separation

of the nasal pits from the stomodeum and subsequent separation of the oral and

nasal cavities. Merging of the nasal prominences forms the philtrum and

Cupid’s bow region of the upper lip, the nasal tip, the premaxilla and primary

palate, and the nasal septum. The lateral nasal prominences form the nasal alae.

The Lack of fusion of the lateral nasal and maxillary prominences results in an

oblique facial cleft which tracks the nasolacrimal groove. The maxillary

prominence forms the major portion of the upper lip (excluding the philtrum)

and the upper cheek regions. Bilateral cleft lip results from failure of fusion of

merged medial nasal prominences with the maxillary prominence on either side.

As a result, the merged medial nasal prominences (globular process) are often

quite prominent, as they are not restrained by attachment to the maxillary

prominences laterally. This manifests at birth, as in a patient with a complete

bilateral cleft lip and anterior over projection of the premaxilla and prolabium.

Laterally, the maxillary and mandibular prominences join at the lateral

commissure of the mouth. (Arun 2007)

Fig. (2) Embryo in 5 weeks (Peter Williams 1980)

 

15

ANATOMY OF THE LIP