CLEFTS

CLEFTS OF THE LIP

One of the most common abnormalities that is presented during birth, and comes to disrupt the joy that the arrival of a child brings to parents, is the cleft of the lip, of the palate or both.

CLEFT LIP

Cleft lip is called the discontinuation of the lip and perhaps of the alveolus, namely the segment of the maxilla that holds the teeth together.
If it separates the lip entirely, the cleft is called “complete”, while if it only divides a segment of the lip it is called “incomplete”.
It may be unilateral (single) or bilateral (double), namely on one or on both sides, while very rarely it is precisely in the middle.

clefts 1

FREQUENCY

It is the most frequent congenital abnormality of the face and it is presented once in about every 600 births. In Cyprus it is estimated that approximately 15 children are borne with a cleft lip palate per year where as in Greece it is estimated that the number reached  200-250 children per year.
The cleft may constitute a part of a more severe condition (of a syndrome), but most times it is not accompanied by other problems.
The mental development of a child is normal and when the rehabilitation in appearance and functionality of the problems is complete, the child is integrated into the community without any problems whatsoever.
In contrast with more severe syndromes, the simple cleft lip does not follow the rules of heredity, but there is often a relative in the family, even a remote relative, with the same problem.
It is important to know that: the functional and aesthetical rehabilitation of clefts is absolute, with the prerequisite that there will be uninterrupted cooperation of the parents with a group of specialists on clefts, who will provide the child and its family with constant support, monitoring and guidance for a complete treatment.

DENTITION

The dentition of children with a cleft lip and especially in instances of clefts of the lip and the alveolus, usually presents certain abnormalities in regards to the arrangement of the teeth. These problems are very effectively corrected with orthodontics, with the prerequisite that the orthodontist who will take on the treatment will have experience on the subject of clefts.
An essential prerequisite is the retention of the deciduous teeth in good condition with frequent visits to the paediatric dentist (something that should apply not only for children, but for the general population as well). Regular check ups and fluoridations will be performed until the time comes for the orthodontic treatment to begin. The orthodontic treatment usually begins after the passing of the sixth or seventh year of age, but the first evaluation must be done much sooner.

clefts 2

clefts toxo new

 

If you have any questions or need additional information about the treatment of clefts, please call +357 25761010 or send us an e-mail at info@ccmfc.com.cy.
The Cyprus Cranio Maxillofacial Surgery Center cooperates with the Cyprus Center for Clefts &  Facial Deformities,
MEDICLEFT where a team of specialists monitor patients with similar problems. www.medicleft.com

 


SURGICAL REHABILITATION OF CLEFTS

The rehabilitation of a child with a cleft must be taken on from the beginning by a group of doctors of various specialties, who are entirely specialized on the whole spectrum of the treatment of clefts.  The initial procedures are of crucial importance for the appearance and functional integrity of the child for the rest of its life.
The child must be constantly monitored, and at frequent intervals, as the lack of constant monitoring is responsible for the creation of problems with severe consequences. The plastic surgeon that carries out the initial procedures usually has the immediate supervision and monitoring of the child and following the specified time-schedule of treatment refers the child for evaluation by other specialties such as E.N.T.s, paediatric dentists, speech and voice pathologists, orthodontists etc.
The time-schedule of the surgical treatment of clefts may vary from team to team. Here we present to you our personal preference.

CORRECTION OF THE CLEFT OF THE LIP

The correction of the single cleft of the lip is usually done at the age of three months.
In instances of wide complete single or double cleft, the first surgical procedure may be the agglutination of the lip, which is done at about the age of three months old and it constitutes the simple lip approximation without the attempt of aesthetic correction. The aim of this procedure is the relaxation of the tissues of the lip to facilitate the next and determinant correction of the lip, which will follow in approximately three to four months later.
The scars that occur follow the natural direction of the filter of the lip and most times they become almost invincible with the passing of time.

 

In the double cleft the problem is more complex. The principles of rehabilitation are the same as with the single cleft of the lip, but more procedures might be needed depending on the cleft.

clefts 5

If you have any questions or need additional information about the treatment of clefts, please call +357 25761010 or send us an e-mail at info@ccmfc.com.cy.
The Cyprus Cranio Maxillofacial Surgery Center cooperates with the Cyprus Center for Clefts &  Facial Deformities,
MEDICLEFT where a team of specialists monitor patients with similar problems. www.medicleft.com


BONE GRAFT ON THE ALVEOLUS

In instances where there is a cleft in the area of the alveolus, an essential prerequisite for the supplementation of the orthodontic rehabilitation is for a bone graft to be done in the area of the cleft, namely to fill in the gap with a bone that is taken from the pelvis, so that the permanent bicuspid  can rise.
This procedure is usually done between the ages of nine and eleven and it may be combined with the correction of the scoliosis of the diaphragm and corrections of imperfections in the appearance of the lip that are still likely to remain.
If it is a double cleft, it is possible that the graft might not be able to be done on both sides simultaneously and two procedures may be needed to be done.
It is obvious that in order to achieve the best possible aesthetic and functional outcomes, the constant and uninterrupted monitoring of the child is essential from its birth until puberty, as well as the execution of a series of procedures which are interdependent with each other on the basis of a clearly specified time-schedule.
Dealing with clefts outside of specialized centres typically leads to bad outcomes whose correction requires additional procedures and unnecessary hardship for the child and its family.

 

.


DEVELOPMENT OF THE MAXILLA

In instances where the cleft of the lip co-exists with the cleft of the alveolus, the development of the maxilla may fall short relative to the mandible, either due to the use of a traumatic technique in previous procedures or due to a diminished force of development. One more procedure is then needed for the rehabilitation of the child to be completed. (See chapter “Clefts of the palate”)


Text and photo source: Greek Craniofacial Center. The GCC was founded by the late Dr. Alexander Stratoudakis and is directed by Dr. Kostas Alexandrou Craniofacial Surgeon, a member of the Cyprus Center for Clefts & Facial Deformities, MEDICLEFT.

 


 

CLEFTS OF THE PALATE

The cleft of the palate is the separation of the roof of the mouth, namely of the palate.  The cleft of the palate may be presented on its own or in combination with the cleft of the lip and alveolus.  In the instance where the cleft of the palate is not accompanied by a cleft of the lip it runs down the midline, while when it co-exists with a cleft of the lip and alveolus, the cleft of the palate is on the same side as the cleft of the lip and alveolus.
The palate is made up of two segments, the hard and the soft palate. On the soft palate there is a muscle sheath that secures its mobility, which is necessary for its proper function (speech, swallowing). It is easy to understand that the cleft of the palate interrupts the muscle sheath and it renders its normal function impossible.
The cleft of the palate may only pertain to the soft tissues or also extend towards the front and include the bones of the hard palate.
The breadth (width) of cleft palate shows large variations, from very narrow, to baggy.

clefts 6


CORRECTION OF THE CLEFT OF THE PALATE

The correction of the cleft of the palate is usually done between the ages of 10 and 12 months old and it usually includes the correction of the soft palate and the hard palate until the area of the alveolus (where the teeth grow). But in instances of wide clefts, the surgical correction is transferred later, without this having any negative effect.
The technique of the correction of the palate must be very delicate and cause the minimal possible injury of the soft tissues so that the dynamic/force of the development of the maxilla is not over-damaged.
During the correction of the cleft of the palate, it is essential for the muscle sheath to be corrected with preciseness, so that the soft palate will acquire normal mobility.
The rehabilitation of the area of the alveolus is done later, during the execution of the bone graft. The technique of the correction of the cleft of the palate plays a significant role in the smooth development of the maxilla afterwards. A bad technique may have as a consequence severe disturbances in the development of the maxilla.


PROBLEMS CAUSED 

EARS

It is also important for the parent of a child with a cleft of the palate to know that there are high possibilities for their child to have a distinct sensibility of the ears.
More precisely, in children with clefts of the palate, the Eustachian tube, namely the tube through which the instrument of the ear communicates with the pharynx is closed off, as a result of which fluid is accumulated in the middle ear.
If this fluid is superinfected it can cause acute otitis, which if not dealt with effectively, it might lead to a rapture of the dram.
If the fluid remains for a long period of time, it becomes sticky and it hinders the transmission of sound, as a result of which damage to the hearing is created. The continuous monitoring of the ears from the paediatrician or the E.N.T. is therefore essential.
Overall, if the existence of fluid is noted, that does not subside after treatment or if the child suffers from more than three otitides per year ventilation tubes will have to be placed in the drams, with a very simple procedure, which will allow the unrestrained entry of air in the middle ear and will hinder the accumulation of fluid.
The repetitive and continuous administration of antibiotics not only does it not constitute an alternative solution, but it is believed that it might have a harmful effect on the teeth, especially on the permanent first molars, which might become unsound and break easily when the child bites into something hard.

EUSTACHIAN

SPEECH

It is obvious that the speech of a child with a cleft of the palate, that has not been surgically corrected, is not possible to be normal as the development of speech entails entirety of the anatomical elements of the mouth and pharynx. The surgical correction of the cleft creates the essential conditions for the proper development of speech.
In a small percentage, even after the correction of the palate, problems of the speech might remain, which are at first dealt with, with speech therapy and if needed with a small procedure called “pharyngyoplasty”

PHARYNGOLASTY

DEVELOPMENT OF THE MAXILLA

In some instances, especially when a cleft of the alveolus co-exists with the cleft of the palate, despite the compliance to the rules of the proper technique during the correction of the cleft, the final development of the maxilla falls short relative to the development of a normal maxilla.   This is due to the force of the development of the maxilla which might be decreased in instances of clefts. In this case, when the development is completed, at the end of puberty, the maxilla will be in a posterior position relative to the mandible. This problem is corrected with a follow-up procedure (osteotomy of the maxilla), with which the placement of the maxilla in the right position is achieved relative to the mandible (in more severe instances the method of the distraction osteogenesis is used and thus the circle of procedures for the rehabilitation is completed.


Sub-mucosal Cleft palate

In the sub-mucosal cleft the separation of the palate is not complete. The distinct feature in the presentation of the sub-mucosal cleft is the (double) uvula.(The cleft pertains mainly to the muscle sheath of the palate, which is separated, and to the hard palate, which often has a small gap on its posterior segment.

FUNCTIONAL PROBLEMS

The functional problems in the sub-mucosal cleft are the same that are observed in the full forms of the cleft of the palate, namely:

  • Nasal speech
  • Frequent incidents of otitis

It must be noted that these problems are usually mild or might not even exist at all, so it is possible that the diagnosis might never occur.

HOW IS THE DIAGNOSIS DONE?

The diagnosis is done exclusively with the medical examination during which the double uvula is observed. With a gentle palpation of the palate a small gap (shortage) is ascertained on the hard palate.
The doctor suspects the existence of a sub-mucosal cleft when a child presents inexplicable nasal speech or has frequent incidents of otitis.

WHEN IS THE SUB-MUCOSAL CLEFT OPERATED ON?

In indication for a surgical correction only exists when nasal speech or even frequent instances of otitis as well are observed.
It must be noted however, that the outcomes following the surgical correction, for the improvement of speech or the reduction of the frequency of the incidents of otitis, are not always completely satisfactory. They are also perhaps even less satisfactory than in cases of full cleft of the palate.

If you have any questions or need additional information about the treatment of clefts / deformities, please call +357 25761010 or send us an e-mail at info@ccmfc.com.cy.
The Cyprus Cranio Maxillofacial Surgery Center cooperates with the Cyprus Center of clefts &  Face deformities,
MEDICLEFT where a team of specialists monitor patients with similar problems

Text and photo source: Greek Craniofacial Center. The GCC was founded by the late Dr. Alexander Stratoudakis and is directed by Dr. Kostas Alexandrou Craniofacial Surgeon, a member of the Cyprus Centre of cleft & Face Deformity, MEDICLEFT.


 

Craniofacial clefts

WHAT ARE CRANIO-FACIAL CLEFTS?
Craniofacial clefts are big clefts, which are likely to affect the face, the cranium or both. They are much rarer than the simple clefts of the lip and of the palate and are presented sporadically without conforming to any rule of heredity.
Their gravity varies significantly from the smallest cut on the lip or on the nose or from a “scar” on the cheek, to a dramatic separation of the tissues of one segment of the face.

FREQUENCY
Despite being rare, their actual frequency is difficult to be calculated, because the more mild forms might not be diagnosed.

RANKING
The craniofacial clefts follow certain axis on the face, whose course has been numbered for sorting and filing purposes. The orbit is taken as a benchmark and the numbers follow an anti-clockwise direction, so that the smaller numbers have a “southern” direction and pertain to the face, while the bigger numbers have a “northern” direction and pertain to the cranium.
It is possible for more than one cranio-facial clefts to co-exist and then the clinical image is respectively more severe. Different areas of the face are affected depending on the cleft. For example cleft number 0 (Midline craniofacial dysgraphia) causes an expansion of the bridge of the nose, while clefts number 1 and 2 affect the side view of the nose. Cleft number 3 separates the nose from the buccal region and usually affects the ipsilateral orbit and the ifraorbital region on the same side. Cleft number 4 may also imply absence of the eye and orbital deformity in the homonymous side. Cleft number 5 is rare and affects the maxilla and the lip. Clefts number 6, 7 and 8 affect the area of the zygomatical bone and cause deformities of the side segment of the face and of the angle of the mouth as well as the lower eyelid. Clefts 11 through 14 affect the area of the forehead and might even cause the protrusion of the brain by a deficiency in the area of the forehead.

SURGICAL REHABILITATION
The correction of cranio-facial clefts requires complex surgical procedures with the cooperation of various specialties and according to a predetermined time-schedule. In general, the surgical rehabilitation starts at the infant age and may extend up until puberty. The careful programming of the essential procedures will secure the best possible outcomes.

 

If you have any questions or need additional information about the treatment of clefts / deformities, please call +357 25761010 or send us an e-mail at info@ccmfc.com.cy.
The Cyprus Cranio Maxillofacial Surgery Center cooperates with the Cyprus Center for Clefts &  Facial Deformities,
MEDICLEFT where a team of specialists monitor patients with similar problems

 

Text and photo source: Greek Craniofacial Center. The GCC was founded by the late Dr. Alexander Stratoudakis and is directed by Dr. Kostas Alexandrou Craniofacial Surgeon, a member of the Cyprus Centre for Clefts & Facial Deformities, MEDICLEFT.

 


CRANIOSYNOSTOSIS

NONSYNDROMIC (SIMPLE) CRANIOSYNOSTOSIS
CRANIAL SUTURES AND PREMATURE CRANIOSYNOSTOSIS

The premature fusion of two or more bones of the cranium is called craniosynostosis.
The newborn’s skull is made up of bones which have not yet been joined together, but are held together with flexible joints which are called “cranial sutures”.
Because in the initial stages of a person’s life the brain has an intense distinct development, the bones, giving in to the pressure of the developed brain pull away one from the other, thus allowing the smooth development of the brain and of the cranium.
When the development of the brain stops, the cranial sutures lose their functional significance and they no longer have a reason to exist, therefore they close and the bones are fused together.
The premature synostosis, meaning the premature union of the bones, hinders the normal development of the skull and of the brain and causes morphological (changes in the normal shape of the head), functional, but also developmental problems to the child.
If a suture between two bones closes prematurely then the cranium cannot grow properly and the bones that still have open sutures will develop more disproportionately (countervailing), so that the development of the size of the brain is not confined.  This has as a result the further deformed development of the cranium. The premature synostosis of every suture causes distinctive deformity. (See below)

deformities 1

THERE ARE FOUR GENERAL CATEGORIES OF NON-SYNDROMIC PREMATURE SYNOSTOSIS

1. Plagiocephaly
The term “plagiocephaly” is a broad sense for the description of the asymmetrical formation of the cranium and it is divided into two categories: the frontal plagiocephaly and the occipital plagiocephaly.

2. Frontal plagiocephaly
The term “frontal plagiocephaly” defines the creep of the asymmetry of the forehead, which is due to the premature occlusion (premature synostosis) of the left or of the right side of coronary suture.
The appearance is distinctive:

  • On the side where the problem exists the forehead is wide (flat) or even concave
  • The root of the nose is drawn towards the side of the problem
  • The orbit is raised in relation to the orbit of the opposite side
  • The eyebrow is usually in a higher position
  • The ear is also located on a more anterior position
  • On the opposite side the forehead is projected more, a phenomenon which is caused by the pressure that the brain applies in its attempt to ensure the necessary space for its development.

deformities 2

3. Postoperative Appearance
The diagnosis is essential to be done during the first months of an infant’s life, because dealing with the frontal plagiocephaly synostosis is always surgical and ideally it must be carried out between the 6th and 12th month, in order for the higher possible benefit to be ensured.
The deformity, which is considered trivial by a lot of parents, actually evolves into a serious problem, because as time passes by it worsens and in an older age the surgical rehabilitation is significantly harder.
The rehabilitation of the frontal plagiocephaly is done by a surgical team with a specialization in craniofacial problems. In these particular cases the craniofacial/ plastic surgeon and the neurosurgeon work together.
During the procedure, the deformed area of the forehead and of the orbits is removed and with various handlings the normal shape in the bone area is attributed, which constitutes the highest segment of the orbits.
The transfer of the bone segments follows so that a new and symmetrical forehead will be formed.
The patient’s stay in the intensive care unit for 24 hours, following the completion of the procedure, is essential in order to ensure their excellent recovery, and the total stay in the hospital usually does not exceed 5- 7 days.

4. Occipital plagiocephaly
The occipital plagiocephaly is defined as the asymmetrical creep of the posterior segment of the head (occipito- parietal region).
Most instances of occipital plagiocephaly are due to the application of external pressure on the cranium (intrauterine or after birth), while a small percentage existing due to the premature occlusion (synostosis) of the lambdoid suture.

deformities 3

For the full description of the occipital plagiocephaly, see separate chapter.Dealing with instances of occipital plagiocephaly by a premature synostosis of the lambdoid suture is surgical. The suitable surgical procedure constitutes a cranioplasty of the posterior segment of the cranium, to which an as much of a normal as possible shape is attributed with the transfer of bone segments. Thus the disappearance of the pressure, which is applied on the subjected segment of the brain, is ensured.


BRACHYCEPHALY

The deformity that is caused if both sides of the coronary suture are synostosed/ prematurely, diminishing the frontal-posterior development of the cranium is called brachycephaly.


TRIGONOCEPHALY

The trigonocephaly is caused by the premature synostosis of the frontal (mid-frontal) suture (which is a suture in the midline of the frontal bone) where the forehead develops in a triangular way.deformities 4


SCAPHOCEPHALY

Scaphocephaly is caused by the premature synostosis of the sagittal suture. This suture heads, from the front, towards the back on the top of the cranium and when it closes prematurely it usually becomes palpable, while visually a relatively elongated head is observed.

deformities 5

In some instances, where the back segment of the sagittal suture is prematurely synostosed the shape of the head somewhat differs. The forehead is big and arched, while the vault of the cranium presents an inclination from the front and upwards towards the back and downwards.


DIAGNOSIS OF THE CRANIOSYNOSTOSIS

Craniosynostoses might be obvious during birth or they might be observed later. The diagnosis is done through the physical examination, usually in combination with diagnostic illustrations.
During the examination the full medical history is received and the necessary measurements are taken.
There is also the possibility that supplementary special illustrative examinations will be ordered, such as C.T.s or M.R.I.s, for a more detailed estimation.


SURGICAL REHABILITATION

Every incident is evaluated individually based on the following parameters:

  • Age
  • State of health and bodily weight
  • Type and extent of craniosynostosis

Therefore the plan of rehabilitation of every incident is entirely personalized based on the individual needs of the child.
Usually the rehabilitation is feasible only with a surgical procedure. The aim is the correction of the shape of the head, so that the further normal development of the brain and of the shape of the head will be allowed, as well as the decrease of the intracranial pressure, if it exists.
In the past the surgical treatment of the craniosynostosis constituted the simple removal of the synostosed suture with the hope that this would have, up to the point of success even, the smoothness of the shape of the cranium, with the results typically being non-satisfactory.
Only after having realized that the dura mater (the sheath that surrounds the brain) had to be released in a wide range by its adhesions in the crania and that a normal shape had to be given to the deformed segments of the cranium, during the initial procedure, the surgeons started to achieve satisfactory outcomes.
Nowadays specialized surgical cranio-plastic procedures are executed, which have been especially designed for the deformed areas of the cranium and the skeleton of the face to be rehabilitated.
These surgical procedures are typically executed during the first months of a person’s life so that the infant will benefit from its tissues’ (periosteum and the dura mater) increased ability to form bones after the surgical procedure.
The cranio-plastic procedures must be carried out by specialized cranio-facial teams in centres that are staffed with scientists and doctors with a lot of experience in this kind of diseases.


PROBLEMS THAT ACCOMPANY THE CRANIOSYNOSTOSES

Phenomena of pressure in the brain
In the area of the synostosis the brain is being pressurized, by the lack of space that is created, and as a consequence this area might not be supplied with enough blood. As a consequence of this may be the lacking creation of synapses between the cells of the nerves that are necessary for the normal conformation of the nervous system.

Intracranial hypertension
The pressure that is applied on the brain by the supernatant deformed segment of the cranium may cause an increase of the intracranial pressure, meaning generalized intracranial hypertension, or it may be applied locally only on the segment of the brain that is located below the deformed bone.
This problem is more frequent than it used to be believed it was. The highest percentages are not observed in the premature occlusion of the sagittal suture (scaphocephaly).

Progressive premature occlusion of other sutures
Progressive premature occlusion of the coronary suture has been observed following the surgical correction of scaphocephaly. This problem is prevented with the simultaneous removal of the coronary suture during the correction of the scaphocephaly.

Eye problems
Of the non-syndromic craniosynostosis, the frontal plagiocephaly is very often accompanied by vision/ eye problems . The most frequent are:

  • Strabismus, meaning the eyes don’t see one image but two, as a consequence of which one image gets neutralized and amblyopia is created in one eye. The eye that is ailing is usually on the healthy side and not on the side of the damage. This problem is entirely curable, as long as the diagnosis is done on time, and it is almost exclusively observed in the frontal plagiocephaly.
  • The swelling of the eye sockets is a sign of intracranial hypertension

It is therefore obvious how essential is the eye examination of children with premature synostosis of the cranial sutures.

Cognitive and learning disorders
They are observed, in high frequency, in children with non-syndromic synostoses and they manifest during the pre-school ages. It has not been made clear if they are caused by the craniosynostosis itself or if they are simply due to the same gene that causes the craniosynostosis.
An evaluation by a developmental paediatrician is recommended once a year from the age of two years old and onwards, so that if there is a suspicion of this problem the early therapeutic intervention will be done on time.


PROGNOSIS

The prognosis of the non-syndromic craniosynostosis is generally good as long as the indicated procedure is done on time and the child continues to be monitored regularly by the craniofacial team. The children are expected to live a normal life without restrictions with the same allocation as that of the general population.


 

Click HERE to see our CASES

 

If you have any questions or need additional information about the treatment of clefts / deformities, please call +357 25761010 or send us an e-mail at info@ccmfc.com.cy.
The Cyprus Cranio Maxillofacial Surgery Center cooperates with the Cyprus Center for Clefts &  Facial Deformities,
MEDICLEFT where a team of specialists monitor patients with similar problems