Abstract
Oblique facial clefts are extremely rare congenital deformities with a reported incidence of 0.24% of all facial clefts. This report presents a patient with a right-sided oblique cleft extending through the upper lip, the alar groove and the lower palpebra accompanied by a left-sided complete cleft lip and palate. Hypertylorism and bilateral microphthalmia as well as flexion wrist contractures were also present. Primary straight-line closure of the oblique cleft was undertaken followed by primary closure of the contralateral cleft lip. The treatment modality and 2 year follow-up results are presented.
Oblique facial clefts are extremely rare congenital deformities with a reported incidence of 0.24% of all facial clefts but their precise incidence in the population is unknown. T essier described an anatomical classification system, in which a number is assigned to each craniofacial cleft on the basis of its position relative to the sagittal midline and the orbit. The Tessier type 3 cleft extends from the philtrum of the upper lip, through the wing of the nostril and reaches the medial canthus of the eye.
The etiology of clefts is explained as faded fusion of the mesoderm during embryonic processes. In clefts located in areas that do not correspond to any known embryonic fusion line, amniotic bands are implicated in the etiology. It is thought that these clefts occur subsequent to the fusion of the facial processes. They may be complete or incomplete, can involve the palate and sometimes extend into the temporal region. All known cases of oblique facial clefts are sporadic with no syndromic association or sex predilection .
The treatment modalities in orofacial clefts comprise the use of tissue expanders , rotation and advancement of a flap of the cheek , bony defect obliteration with autogenous iliac bone graft chips and soft tissue reconstruction with multiple Z-plasty flaps .
Case report
The patient was a female infant, the first child of healthy non-consanguineous parents (mother 20 years old, father 23 years old) delivered at 40 weeks’ gestation by Caesarian section, weighing 3820 g and 52 cm long. Her 1- and 5-Apgar scores were 10. There was no history of any relevant abnormalities in the family and no teratogenic factors identifiable. Prenatal ultrasonography in the sixth and ninth gestational month did not show any congenital malformation.
The infant was found to have a right-sided oblique cleft, corresponding to Tessier type 3, which extended through the upper lip, the alar groove and the lower palpebra as well as a left-sided complete cleft lip ( Fig. 1 ). In the oral cavity, there was a complete cleft palate associated with bilateral alveolar clefts. Hypertylorism, bilateral microphthalmia and flexion wrist contractures were observed.
Sonography of the heart revealed an atrial septal defect (ASD II) of no hemodynamic significance. Postnatal sonography of the brain revealed a slight asymmetry of the lateral ventricles (the right of 0.2 cm; the left of 0.46 cm). A cyst of the septum pellucidum (0.4 cm) was found. A three-dimensional computed tomography (CT) scan of the craniofacial skeleton revealed the extent of the clefting and hypertelorism ( Fig. 2 ). CT of the head confirmed the presence of the septum pellucidum cyst. Chromosomal examination revealed a normal female karyotype.
Since the second week of life, the child wore plaster corrective splints on the forearms and wrists. Active rehabilitation was introduced when she was 6 months old. At 6 months, primary straight-line closure of the oblique cleft was undertaken. The flaps of the oral mucosa and skin were separated from the cleft ridges, mobilized and sutured together. Three days after surgery a nasopalatal fistula (average dimension 7 mm) was found close to the nasolabial fold.
Primary closure of the contralateral complete cleft lip was undertaken, according to M urawski , in the eleventh month of life with no complications ( Fig. 3 ). The secondary palate was closed later than normal, after the second year of life, due to frequent infections of the upper airway, which necessitated postponing surgery several times. The appearance of the face 2 years after surgery is shown in Fig. 4 and the follow-up CT scans are shown in Fig. 5 . It can be seen that cleft closure caused a significant narrowing of the bony defect.
