Original article

A report on three unusual cleft lips

Median cleft is rare among facial clefts, including bilateral and unilateral clefts. Median upper cleft lip and median alveolar cleft correspond to Type 14 and Type 0, respectively, in Tessier's classification system. Some authors have reported surgical procedures for median cleft. In the case of median alveolar cleft, bone grafting to the cleft side and orthodontics are generally applied, similarly to bilateral or unilateral cleft. Median alveolar cleft is usually accompanied by median upper cleft lip, the degree of which differs in each case. The symptoms include, but are not limited to, median lip defect, wide philtrum, and vermilion notch. However, an isolated alveolar cleft is extremely rare. We encountered a patient with an isolated alveolar cleft who did not have a light median upper cleft lip, such as a wide philtrum or vermilion notch. We herein report this case and describe its treatment.

Median cleft of the upper lip (MCL) is a specific and rare entity on the spectrum of facial clefts. MCL have different clinical expressions and can be either isolated or part of multiple malformations. Confusion still exists regarding the explanation and classification of MCL; some cases have been reported in the literature, but no studies carried out a complete review of the literature.

This study reviewed cases of MCL in 2 French units and conducted a systematic review of the literature, in order to derive a new classification.

Fourteen patients with MCL in the 2 units and 195 cases in the literature were reviewed. They involved complete (42%), incomplete (49%), and minor forms (9%). Epidemiological and clinical data were collected, from which a classification was derived, based on the type of cleft and its belonging to other syndrome(s). Three main groups were distinguished, namely, isolated MCL, MCL within craniofacial malformations, and MCL with extrafacial malformations. Each group and subgroup was associated with a prognosis and led to specific management.

This study reviewed all of the various forms of MCL and their associated anomalies, in order to have a global view of MCL and to derive a useful classification scheme to guide management of care.

Median cleft lip is a rare congenital anomaly. The wide diastema with mesial tipping observed in these patients has been largely overlooked. A midline submucosal alveolar cleft prevents adequate treatment. The purpose of this article is to describe an alveolar bone grafting (ABG) technique used in the combined surgical-orthodontic approach to diastema treatment in patients presenting with incomplete median cleft lip. Patients treated for incomplete median cleft lip and diastema were identified in the clinic registry from 1981 to 2007. Six patients were identified; 4 underwent ABG before permanent maxillary incisor eruption, the other 2 were seen later when they were 11 years old. All 6 ABGs were successful. The incisors erupted through the graft or were successfully moved into it with lasting results. Follow-up ranged from 8 to 21 years. The existence of a midline submucosal alveolar cleft and subsequent diastema should be recognized and addressed in all patients who present with incomplete median cleft lip repair. This includes taking maxillary occlusal view X-rays before the age of 5 years to detect the cleft, and proceed to ABG if necessary, generally before permanent maxillary incisor eruption.

Congenital midline cervical cleft is a very uncommon malformation. Through a series of five cases treated in the pediatric otolaryngology head and neck surgery department of an academic care center, authors discuss embryopathogeny and therapeutic management. It appears that complete surgical removal leads to cure in classic cases. No recurrence was noted and cosmetic damages were minor.

Congenital Mid-line Cervical Cleft (CMCC) is a rare but interesting congenital anomaly of the neck that is frequently misdiagnosed. Much controversy exist regarding its aetiology. Embryologically, the occurrence of CMCC is thought to be due to many hypotheses that include impaired mid-line fusion of the first or second branchial arches, exteriorisation of a thyroglossal duct remnant, and or increased pressure in the cervical area from the pericardial roof in the developing embryo. Histologically, the cleft usually consists of a stratified keratinized squamous epithelium with hyperkeratosis, dermal fibrosis and little or no skin appendages.Associated clinical features could include thyroglossal duct cysts,cleft lip/mandible/sternum,cervical contractures, mandibular spurs, microgenia and or bronchogenic cysts. In this article we present a patient with CMCC. An extensive review of the literature is also included.