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Case 2.12
Published in Monica Fawzy, Plastic Surgery Vivas for the FRCS(Plast), 2023
You’ve mentioned the 6th, 7th, and 8th clefts – associated with Treacher Collins. What are their phenotypes?Cleft number 6 passes between the maxilla and zygoma and may be associated with a coloboma of the lateral lower eyelid.Cleft number 7 runs between the zygoma and the temporal bone. It may extend medially across the cheek into the lateral aspect of the mouth – and may result in macrostomia.Cleft number 8 passes outwards from above the lateral canthus. It extends between the zygoma and the temporal bone into the greater wing of the sphenoid.
Simpson–Golabi–Behmel Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Distinctive facies include (i) macrocephaly (70% of cases); (ii) ocular hypertelorism, epicanthal folds, downslanting palpebral fissures, strabismus or esotropia, cataracts, coloboma of the optic disc, ocular nerve palsies; (iii) redundant, furrowed skin over the glabella; (iv) wide nasal bridge and anteverted nares in infants, broad nose in older individuals; (v) macrostomia (abnormally large mouth); (vi) macroglossia (abnormally large tongue); (vii) dental malocclusion; (viii) midline groove in the lower lip and/or deep furrow in the middle of the tongue; (ix) cleft lip and/or submucous cleft palate (with a bifid uvula, 13%), high and narrow palate; (x) micrognathia (small mandible) in neonates, macrognathia in older individuals; (xi) preauricular tags, fistulas, ear lobule creases, helical dimples [2].
Anatomy and Embryology of the Mouth and Dentition
Published in John C Watkinson, Raymond W Clarke, Terry M Jones, Vinidh Paleri, Nicholas White, Tim Woolford, Head & Neck Surgery Plastic Surgery, 2018
In the 6-week-old embryo, the two mandibular processes fuse in the midline to form the tissues of the lower jaw. Rarely, persistence of a midline groove in this region produces a mandibular cleft. The mandibular and maxillary processes meet at the angle of the mouth (labial commissure), thus defining its outline. Disturbances in this development may give rise to macrostomia (enlarged oral orifice) or microstomia (small oral orifice), or rarely to an astomia (lack of an oral orifice). From the corners of the mouth, the maxillary processes grow inwards beneath the lateral nasal processes and towards the medial nasal processes of the upper lip (Figure 41.20). An appreciation of this arrangement helps explain the occurrence of a unilateral or bilateral cleft lip (when the medial nasal and maxillary processes fail to merge successfully) and a median cleft (when the two median nasal processes fail to merge). The severity of a cleft may vary from a barely imperceptible groove to a complete cleft. Between the merging maxillary and the lateral nasal process lies the naso-optic furrow. From each furrow a solid ectodermal rod of cells sinks below the surface and canalizes to form the nasolacrimal duct. Persistence of the naso-optic furrow may produce an oblique facial cleft.
A case of ablepharon macrostomia syndrome requiring multidisciplinary care
Published in Clinical and Experimental Optometry, 2021
Denise Alexopoulos, Tracy L Matchinski
This patient was new to the clinic and during the first paediatric primary care examination efforts concentrated on ocular health. Due to the complexity of the case and poor cooperation of the child, limited time was available to measure visual abilities (Table 1). A congenital horizontal nystagmus was observed in both eyes. Gross observation of the anterior segment revealed bilateral partial tarsorrhaphies and corneal opacification. Tactile palpation was soft and equal in both eyes. Macrostomia and dental abnormalities were present. Using a multidisciplinary approach to management, the primary author referred the patient to two additional areas of expertise – a paediatric low vision rehabilitation (LVR) specialist and a paediatric ophthalmologist.
Surgical repair for transverse facial cleft: two flaps with a superiorly rotated single Z-plasty lateral to the commissure
Published in Journal of Plastic Surgery and Hand Surgery, 2019
Pan Zhou, Lin Qiu, Yan Liu, Tianwu Li, Xionghui Ding
We collected data from 27 patients who were treated in the Burn and Plastic Surgery Department of Children’s Hospital of Chongqing Medical University between 2008 and 2017. The youngest patients’ age at surgery was 84 days, and the oldest was 4.5 years (mean, 6.7 months). The patient cohort consisted of 10 girls and 17 boys, including three with a minor commissural cleft for unilateral clefts ending medial to the anterior border of the masseter muscle and six with major commissural clefts ending lateral to the masseter muscle [2] and 17 cases manifesting between them. One patient had a bilateral macrostomia. Fourteen patients manifested a left-side anomaly, while 12 patients had a right-side anomaly. Twenty patients in the cohort had an ear malformation such as accessory ear, and 21 had a hemifacial microsomia.