ESPRAS Quadrennial Congress 2022, Porto, Portekiz, 5 - 07 Ekim 2022
Median
cleft lip
İntroduction : complete or partial lip clefts in the midline of
the upper lip are defined as median clefts of the upper lip. 1 ın 1974, tessier
numbered the craniofacial clefts from 0 to 14 Counterclockwise in a study
involving 336 patients. Upper lip midline clefts are named tessier 0 clefts.
2,3 despite the diversity in classifications, upper lip midline cleft are rare.
Its incidence Among cleft patiens varies between 0.43-0.73 Percent. 4 ıf the
more common forms of cleft Lip and palate are not taken into account, the İncidence
of atypical cleft is estimated at 1/10 5 . 5 median upper lip cleft can be
defined As a cleft that passes vertically through the Midline of the upper lip.
These clefts ocur Due to incomplete or complete Nondevelopment of the medial
nasal Prominences. 6 In this study, we will share a case Diagnosed prenatally
and referred to our clinic For cleft lip repair.
Case: A 55
days old patient who was Diagnosed with cleft lip anomaly in the 20th Week of
intrauterine life was admitted to our Clinic. It was learned that she was born
at 37 Weeks of age as the third live birth from the Third pregnancy of a 36
year old mother who Had no teratogen exposure or drug use History. There was no
consanguinity in the Parents and no other family history of cleft lip And
palate. In our examination, cleft lip with Vermillon defect in the upper lip
midline and Bifid frenulum structure were observed. It was Observed that the
columella was short and the Nose tip was flat. Intercanthal distance was Measured
to be minimally increased and that Was consistent with hypertelorism. The total
Lenght and width of the vermillon were Considered normal. (photography 1) In
the preoperative period, brain mrı Ve ecocardiography were performed fort he Diagnosis
of additional anomalies such as Other midline defects. While no problem was Observed in the patient’s ecg, corpus Callosum
agenesis and colpocephaly was Detected in brain mrı of the patient. BrainMrı
showed no sign of encephalocele and Meningocele. The departmant of pediatric Cardiology
reported that the patient did not Need endocarditis prophylaxis. The Departmant
of pediatric nefrology also Examined the patient and the department did (photography
1, preoperative examination) (photography 2, intraoperative examination) Not
make any further recommendations. The patient whose preoperativePreparations
were completed and reached 9200gr, was operated on by us when she was 8 months
and 27 days old. In the operation, local anesthetic Substance containing
lidocaine+adrenaline Was infiltrated on the prolabium and cleft lip Margins.
Prolabium was elevated as a Quadriangular flap in order to eliminate Shortness
of the columella and flatness of theTip. Incision was extended in the wet mucosa
Of the upper lip to the basis of the frenulum. When it was seen that the lenght
of the wet Mucosa was not sufficient, a small z-plasty Was designed from the
mucosa to increase its Lenght. Orbicularis oris muscle was dissected From the
abnormal attacthment sites on the Cleft margins, with horizontal incisions made
İn the muscle, so the contractions in the Muscle were opened and range of
motion was İncreased. Orbicularis oris muscle was Repaired in the midline by
suturing with 4-0 Vicryl. One z-plasty designed in the mucosa Were sutured
appropriately with 5-0 vicyrl to Form the lip tubercle and the mucosa was Repaired.
The prolabium flap that was Elavated at begining of the operation was Sutured
to the base of the columella to allow For the elongation of the columella.
Lateral Skin flaps were primarly sutured in the midline With 6-0 biosyn.
Rifampicin was applied to the Suture lines and suture lines was closed with Sterile
dressing. Then, the patient was followed up İnpatient for 4 days, suture line
was cleaned With saline daily and the dressing was Renewed by applying
rifampicin. The dressing On the suture line was left open at discharge And the
patient’s skin sutures were removed On the 8th postoperative day. No Complications
were observed in the Outpatient follow-ups. In the postoperative evaluation,
the Results of the patient were satisfactory. Sufficient lenght was provided in
the Columella and the flattened appearance of the Nose tip was eliminated. Vermillocutaneous
line was created with Minimal scarring. The vertical scar line was Equidistant
from the bilateral filtral columns. Cupid bow was formed as desired. İn the Evaluation
the height of the left apex of the Cupid bow was minimally superior to the Contralateral
apex
Discussion:Median
clefts of the lip were defined By demyer as two separate groups, including Orbital
hypotelorism and hypertelorism. 7 ın 1968, millard defined median lip
clefts as Clefts that cross the midline of the upper lip, Regardless of extent
and size. Then he dividedİt into 2 classes; the first group includes Agenesis
of the frontonasal process and the Second group is defined as the cleft of
theMedian segments. Therefore, the second Group is associated with
hypertelorism and Cranial malformations. In our case, corpus callosum agenesis Was
detected in cranial imaging and Hypertelorism was present in examination. In That
case, it is tought that our patient belong To the second group defined by
millard. The Patient was screened for additional midline Defects by us in the
preoperative periods. Due to the scarcity of median cleft lip Cases and
resources on this subject, there is No definitive procedure for surgical Managment
of upper lip clefts. Millard Recommended excision of inverted cleft lips in 1968.
Then, in his work in 1977, he proposedAn inverted v-shaped excision from the
2mm Superior white roll on either side of the cleft. Thus, the skin in the
midline of the cupid bow Was elongated. 8 nakamura, tomonari and Goto described
wedge excision at the Wemillon border, but did not provide Sufficient
information on excision or Manipulation of the orbicularis oris muscle. Many
types of excision, such as elliptical Excision, have been published by many Authors.
In our patient, excision was not Considered due to the short columella and Nasal
tip flattening. It was sutured to the base Of the columella by elevating the
prolabium as A fasciocutaneous flap, thus providing many Benefits such as
elongation of the columella, Elimination of asymmetry in the nostrils, and Correction
of nasal tip. With the dissection And horizontal incision made to the
orbicularis(photography 3, postoperative 1st day)(photography 4, postoperative
4th month) Oris muscle, tension-free repair of the muscle İn the midline was
possible. By suturing the Skin in the midline without tension, and Performing
z-plasty on the mucosa, both the Length of the mucosa was increased and the Lip
tubercle was formed. In the patient, whoDid not have complications in the Postoperative
period, mouth opening was Evaluated as compatible with her peers. In
conclusion, median cleft lip is a very rare Congenital anomaly. Due to the low
number ofCases and scarcity of resources in the Litarature, a common decision
could not be Reached in the treatment process.therefore, a Lot of work is
needed on this subject. We Would like to contribute to the literature by Publishing
our own procedure.
Sources1.
Millard dr and williams s. Median Lip clefts of the upper lip. Plast reconstr.
Surg.
1968;42:4 2. Tessier p. Anatomical classification Of
facial, craniofacial, and latero-facial clefts. J
Maxillofac
surg 1976;14:69 3. Tessier p.
Anatomical classification Of facial, craniofacial, and latero-facial clefts. In:
tessier p, ed. Symposium onplastic surgery İn the orbital region. St. Louis: cv
mosby,
1976 4. Apesos j and anigian g. Median Cleft of
the lip: ıts significance and surgical Repair. Cleft palate j 1993;30:94–96 5. Kawamoto hk and patel pk. Atypical Facial
clefts. In: bentz m, ed. Pediatric plastic Surgery. Stamford: appleton and
lange, 1998 6. Johnston mc, sulik kk.
Some Abnormal patterns of development in the Craniofacial region. Birth defects
orig artic ser 1979;15:23‑42
7. Demyer w. The median cleft face Syndrome: differential diagnosis of
cranium Bifidum occultum, hypertelorism, and medianCleft nose, lip, and palate.
Neurology.1967;17:961–971. 8. Millard
dr jr. Median cleft lip with Hypertelorism. In: millard dr, jr, ed. Cleft Craft:
the evolution of ıts surgery. Vol. 2. Boston: little, brown; 1977:727–768. 9. Nakamura j, tomonari h, goto sTrue median
cleft of the upper lip associated With three pedunculated club-shaped skin Masses.
Plast reconstr surg 1985;75:727–731
Dokuz eylül
univercity hospital plasticReconstructive and resthetic surgery Departmant
izmir/turkey
Prof.dr.
Adnan menderes Dr. Fatih berk ateşşahin Dr. Hasan basri çağlı Dr. Safa eren
atalmış Dr. Merve terzi Dr. Tahir babahan