Bilateral complete wide cleft lip with short, protruded or deviated prolabium

Bilateral complete wide cleft lip with short, protruded or deviated prolabium

Amin M. Saleh, MD; Mohammad Ahmad AI Ekrashy, MD;

Hany Mohamed  Hassan, MD

Pediatric Surgery Unit, Zagazig University, Egypt.

The aim was evaluation of 5years experience in correction of cases of bilateral wide complete cleft lip with short, deviated or protruded prolabium and promaxilla.

Patients and methods:  20 children with wide complete cleft lip were corrected at the age of

3-6 months. Anterior palate was closed in all corrected children in two layers. The anomalies associate cleftlip as deviated prolabium and promaxilla was centralized by fracturing the vomer; the protruded prolabium and promaxilla were pushed backward after submucous excision of a bony triangle  from the vomer and the short prolabium was elongated by tissues transferred from both sides. Facial appearance and complete healing  was assessed in all corrected patients.

Results: the 20patients were corrected with excellent cosmetic appearance and no remaining anterior fistulas. The anterior palate was closed easily before lip repair without any remaining fistula, this palatal repair supported lip repair and helped in prevention of lip dehiscence. The function was good in all corrected children.

Conclusion: Wide cleft lip with protruded, short prolabium can be safely repaired with good cosmetic and  functional outcomes.  The anterior palate should be closed  with lip correction while it is better to delay closure of the posterior palate to another operation.

Key words:  Wide cleft lip and palate, protruded prolabium, short prolabium, deviated promaxilla.

Introduction:

Patients   with   bilateral   complete   wide cleft lip with short, protruded or deviated prolabium represent a great challenge for surgeons  as this  combination  of anomalies is difficult to manage in only two operative stages and needs several steps for correction and restoration of shape and function.

Cleft palate is associated with 85% of the

bilateral  lip defects as compared to 70% of the unilateral cleft population.

Construction   of  the   cleft   muscle   ring

system of the  lip as early as possible is the base for normalization  of disturbed function in newborn with cleft lip. Repair of cleft lip alone in cases with complete cleft will leave anterior fistula that will be difficult to close and make a problem for most of the surgeons. Repair of anterior palate can be done easily

before  lip closure  and allows  correction  of

associated anomalies as deviated or protruded prolabium and promaxilla.

Timing for surgery also causes a dilemma, where  orthodontists  prefer  delaying  surgery not  to  affect  facial  skeletal  growth  while speech   therapists   prefer  early  surgery   as the development of speech abnormalities is difficult to correct even with speech therapy.

From 2007 to 2012 patients with bilateral

wide cleft lip were repaired at the age of 3- 6 months. Closure  of anterior palate was done easily before lip closure to support lip repair, the associated  lip anomalies  were corrected. Construction of the posterior palate was performed at delayed age (9-12 month).

The aim was the improvement of the development  of  peripheral  hearing,  speech and growth ofbony structures in patients with wide cleft lip and palate with protruded, short

or deviated prolabium.

Am-Shams] Surg 2014; 7(19):1-10

Patients and  methods:

From Jan 2007 to Jan 2012 children with wide cleft lip and palate with short, protruded or deviated prolabium had been corrected.

Surgical techniques: During the first part of the operation an endotracheal tube was placed oral and central and fixed to the center ofthe lower lip, the child's  head was positioned on the surgeon's knees, two strips of gauze placed as  packs  around  the  tube,  opening  of  the mouth was done by using tongue  depressor. The cleft anterior palate was closed in two layers before lip closure. Incision was made in the mucosa over the vomer and two mucosal flaps was designed, deviated prolabium was corrected   by   fracturing   the   bony   vomer, the protruded prolabium were corrected by excising a wedge from the bones of the long vomer to allow the protruded promaxilla and prolabium to be pushed backwards. Incision was made in the oral mucosa 1-2 ml. medial to the  gingival margin in the anterior  palate lateral, anterior  and then in the medial edge of  the  cleft.  Closure  of  the  anterior  palate was performed  in two  layers, nasal mucosa to nasal mucosa then oral mucosa to oral mucosa before lip repair.

The short prolabium was left adherent to

promaxilla and two triangles were elevated from its sides by the covering mucosa and transferred  to form the floor of both nostrils. Incision  was then  made  in the  red  line  of the  prolabium  to  separate the  mucosa  from the skin and prepare a bed for the flaps that were to be transferred to elongate the short prolabium.

Incision was made to separate and mobilize

the lip from the maxilla, then lateral incision was done under the ala of the nose, a second incision was designed to prepare a triangular flap that passed to side of the prolabium and labial flap that were to elongate the short prolabium Figures (2-5).

Incision  was made in the red  line of the

remaining  part of the prolabium to separate the mucosa  of the short prolabium from the skin.

The    short    prolabium    was    elongated by  transferring   two  triangular   flaps  from both  sides  of  the  clefts,  after  incising  and

mobilizing the remammg mucosa of the prolabium that turned backward to be used as a mesentery for the triangles  transferred from both edges.

The short prolabium was kept adherent to

the promaxilla and the separated mucosa was used as a mesentery to the transferred  labial flaps, by suturing  to mucosa  of labial  flaps that  used  to  elongate  the  short  prolabium. The final step in lip repair was suturing of muscles oftransferred labial flaps together in the midline, The lip muscles were dissected, and mucosa sutured to mucosa, skin to skin in  the  midline  then  sutured  to  skin  of  the free  edge  of  the  short  prolabium  on  both sides to elongate it. The floor of both nostrils were covered by the two triangular  skin and mucosal flaps.

The  anterior  palate  were  closed  easily

before lip repair with excellent results and without any residual anterior defect.

Results:

The 20 patients with wide complete cleft lip were repaired during the period between

2007 and 2012 and showed excellent cosmetic appearance and no remaining anterior fistulas. The anterior palate was closed  easily before lip repair without any remaining fistula, this palatal repair supported lip repair and helped in prevention of lip dehiscence. The function was good in all corrected children.

Discussion:

Controversial issues in correction of cases of cleft lip and palate are more abundant as regard most aspects of treatment  , timing of hard palate closure, early or delayed, closure of cleft lip alone, or closure oflip and anterior palate, and delayed closure of the posterior palate in another time, or closure of both lip and palate in one operation.

Louise C.l stated that these combined procedures are generally performed at 2.5 to three months  of age. In these young  babies, airway  monitoring  is  essential.  The  size  of the airway is drastically changed after lip closure and nostril reshaping. There is more resistance  to  breathing,  and  the  child  has to  adapt  to  it.  Furthermore,  the  baby  has

Figure {1): Children with wide cleft lip and palate with protruded, deviated prolabium.

Figure {2): The children after repair of the lip and anterior palate.

Figure  {3):  Two  triangles  elevated   from sides of The short prolabium by the covering mucosa and transferred to form the floor of both nostrils.

never expetienced mouth closure before the procedure and will not spontaneously open his mouth to breathe if the nose is obstmcted (obligatmy nasal breathing in young children). The  developed  postoperative  edema  may reduce the airway; another factor to consider IS    the  partial  obstmction  of   the  normal

Figure {4): Incision is then made in the red line of the prolabium to separate the mucosa from the skin. Then lateral incision is done under the ala of the nose.

nostril due to the septal cartilage deviation, which is particularly severe in wide clefts. Continuous oxygen saturation monitming is recommended for at least 24 h, and narcotics should be used spruingly.

Venkatesh M. et al,2   stated that: timing

of cleft  palate repair  has significant effect

Figure (5): A second incision designed to prepare a triangular flap that will pass to side of the prolabium and labial flap that will be transferred to elongate the short prolabium.  

on  speech. It has been argued that earlier repair  benefits the speech  development as the speech process in some children begins at 1 year of age. Conversely, the late repair theoretically allows for a proper maxillo­ facial growth because the transverse facial growth is not complete until 5 years of age. This has led to a vatiety of timing protocols at different institutions and the optimal time of palatoplasty remains scientifically unproven. However, the best speech results are obtained when palate is closed near the time of the infant's initiation of language acquisition; thus, a p1imruy palatoplasty before 2 yeru·s of age has become the nonn.

Al-Kassaby et al,3 ru·gued that There is conelation  between the highly significant change in anteroposterior projection, observed in presurgical mthodontics and lip repair phases, and reduced time prior to lip reprur

We  have  perfonned  our  procedure between 3 to 6 months aged infants to avoid respiratmy  and  cru·diovasculru·  hazru·ds of repair in eru·lier ages and before development of speech abnormalities.

One ru·gument against the complete cleft closure dming the first yeru·oflife, repeatedly discussed in most meetings of cleft, is the negative effect on maxillruy growth which is not noticed or seen.

In a study  for  Venkatesh M. et al,2 the postoperative fistula had developed in 31% (4/13) of the patients with bilateral cleft lip and palate.

50% of patients with bilateral cleft lip and palate  needed  phru·yngeoplasty  in  a  study done by Bicknell et al.4

Closure of the antetior palate was done easily in all patients before lip closure and without any remaining antetior fistula. Delayed  closure   of   the  posterior  palate after 3-6months allowed its elongation and excluded the need for phruyngeoplasty.

In   cases   with   protruded   or   deviated prolabium these abnmmalities can be conected easily after excision ofbony hiangle from the vomer without any effect on growth of facial bones. In these cases after removal of  the  bony n·iangle the vometian  mucosa apperu·s redundant and both nasal cavities can be closed easily.

The results of the current study ru·e relatively better than that showed by a study for Al-Kassaby et al3 on bilateral complete cleft lip and palate by doing presurgical mthodontics (pso) and only bilateral lip repair by Millru·d technique, their study showed that patients in group R (mdimentru·y promaxilla) showed a highly significant reduction in the anteropostetior projection of the premaxilla in all phases of orthodontics and after lip repair. This could be atu·ibuted to the small size of the promaxilla and the more flexible attachment between the promaxilla and vomer observed in this group. On the other hand, in the group P (prominent), reduction in the anteropostetior projection was significant only during and after orthodontics and the change was of lower significance after lip repair. This could be attlibuted to the more firm attachment between the promaxilla and the vomer in this group. They assured that ren·opositioning of the promaxilla, especially in the alveolar part, is a desired effect of lip repair, especially in group P.

Holland et aP suggested that speech impairment  increases  with  delaying  repair of cleft palate for a long time. Fried9 stated that there  is no relationship  between timing of hard palate repair and mid facial growth disturbances.

Al-Kassaby   et   aP   have   recorded   the relation between lip repair and the following palatal changes; reduction in the most anterior arch width and intercanine width were only significant in group R after lip repair. This could be explained by the direct transmission of   compressive   muscular   forces   exerted by the  lip on the  lateral  sides  of the  upper arch  after  lip repair  (where  the  promaxilla is retracted posteriorly in-between the two palatine  shelves  giving  a  U-shaped  arch). On the  other  hand,  the  change  in  anterior arch  width  was  of  lower  significance   in group  P (where  change  is seen  one  month after lip repair).  This could be attributed to the  presence  of  the  prominent  promaxilla that is locked infront of the palatal shelves preventing lateral forces from being directly transmitted   to   palatal   shelves.   Regarding the changes in the maxillary posterior arch width, there was only low significant change in group R one month after lip repair. There was a highly significant difference at the end of three months after lip repair as compared to Ml.  Change in posterior  arch width  was of no significance in group P. This could be attributed to position of the promaxilla in relation to the palatal shelves and transmitted lip pressure after lip repair.

Santdoli and Cordaro6  stated that there are many reasons, the most important is financial outcome for the application of one stage correction  of cleft lip and palate in the first year of life. We found that delaying closure of the soft palate for another operation allows its elongation with better functional outcomes.

Conclusion

The anterior palate should be closed with lip correction during repair of wide complete cleft lip and palate with short protruded prolabium,  and it  is better  to  delay  closure of the posterior palate to another operation. Excision of bony triangle from the vomer allows pushing back of protruded prolabium and allows centralization of deviated prolabium. Incision done in the red line of the short prolabium allows its elongation by flap formed skin, mucosa and muscles transferred from adjacent lips.

Reference

1-   Louise C L: Unilateral cleft lip and palate: Simultaneous early  repair  of   the  nose, anterior palate and lip. Can 1Plast  Surg.2007; 15(1): 13-18.

2-   Annigeri VM,  Mahajan JK,  Nagarkar A, Singh SP: Outcome analysis of palatoplasty in various types of cleft palate. 1Indian AssocPediatrSurg2012; 17(4): 157-161.

3-   El-Kassaby MA,  Abdelrahman NI, AbbassIT: Premaxillary characteristics in complete bilateral cleft lip and palate: A predictor for treatment outcome. Ann  Maxillofac  Surg 2013; 3(1): 11-19.

4-   Bicknell  S,  McFadden LR,  Curran  JB: Frequency of pharyngeoplasty after primary repair of cleft palate. 1Can Dent Assoc.2002; 68(11): 688-{)92.

5-   HollandS, Gabbay JS, Heller JB, O'Hara C, Hurwitz D, Ford MD, Saunder AS, Bradly JB: Delayed closure of the hard palate lead to speech problems and deleterious maxillary growth. Plastic  Reconst  Surg  2007;  119:1302-1310.

6-   Santdoli L, Cordaro L: One stage repair of unilateral complete cleft lip, alveolus and palate. 6 years follow up presentation. Nr308 at the 7th international congress of cleft palate and related craniofacial anomalies. P Roadbeach Australia 1993.