Median cleft of the upper lip: A new classification to guide treatment decisions

Boutray, Marie de; Béziat, Julien; Yachouh, Jacques; Bigorre, M.; Gleizal, A.; Captier, Guillaume

doi:10.1016/j.jcms.2016.02.012

Cited by 14 publications

(16 citation statements)

References 99 publications

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“…The differential diagnosis of a median cleft is largely dependent on the size of the abnormality and the association with other structural anomalies. 2 Primary craniofacial syndromes that are associated with a median facial cleft are typically within the holoprosencephaly spectrum and are often secondary to aneuploidy, particularly trisomy 13. Frontonasal dysplasia or median cleft face syndrome are considerations when a median cleft (either complete or incomplete) is associated with hypertelorism.…”

Section: Differential Diagnosismentioning

confidence: 99%

“…Median or midline clefts are rare congenital anomalies and account for approximately 0.38e3% of orofacial clefts. 1,2 Definition A median cleft occurs when the defect is in the median line of the face. A median cleft may be complete, extending up to and involving the nasal cavity and maxilla, or incomplete, transgressing only a portion of the vermillion.…”

Section: Introductionmentioning

confidence: 99%

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Median Facial Cleft

Benacerraf

Bromley

Jelin

2019

American Journal of Obstetrics and Gynecology

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Section: Differential Diagnosismentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Median Facial Cleft

Benacerraf

Bromley

Jelin

2019

American Journal of Obstetrics and Gynecology

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“…MCL occurs due to incomplete merging of the median nasal prominences which form the inter-maxillary segment. 2,3 Two major categories of MCL are described:  Demyer sequence: frontonasal deformity associated with hypotelorism, holoprosencephaly and facial deformity which ranges from cyclopia to midline facial cleft with pre-maxillary agenesis.  Median cleft face syndrome: charecterised by nasal deformity, hypertelorism and less chance of brain deformity (corpus callosum agenesis).…”

Section: Discussionmentioning

confidence: 99%

“… Median cleft face syndrome: charecterised by nasal deformity, hypertelorism and less chance of brain deformity (corpus callosum agenesis). [2][3][4] Radiographic imaging for the diagnosis of the associated alveolar defect and accompanying bone defects should be performed by preoperative/postoperative evaluation using 3-dimensionally reconstructed CT imaging. 3,4 Long-term follow-up of the bone defect for treatments including alveolar bone grafting is required.…”

Section: Discussionmentioning

confidence: 99%

“…When indicated, iliac bone grafting with a conventional method can manage the bone defec. [2][3][4] Various surgical techniques have been described for MCL including wedge excision with inverted-V incision, inverted-U incision and forked flap Z plasty repair. [4][5][6] The surgical technique here adopted was Inverted-V excision of the upper lip and repair of the orbicularis oris muscle.…”

Section: Discussionmentioning

confidence: 99%

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Median cleft lip: an uncommon and unique anomaly

Sarma

2019

Int Surg J

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Median or midline cleft lip [MCL] is an uncommon anomaly characterized by a midline vertical cleft through the upper lip and are either isolated or part of multiple anomalies. It can involve the pre-maxilla, the nasal septum, and the central nervous system. MCL includes Complete (42%), Incomplete (49%), and Minor forms (9%). The three main groups distinguished were: 1. Isolated MCL; 2. MCL with craniofacial malformations; and 3. MCL with extra-facial malformations. To analyze two operated cases of median cleft lip and review the relevant literature. The details of two cases of median cleft lip that were operated in 2017 were analysed. Both cases underwent wedge excision with the classical inverted V incision and muscle reconstruction with satisfactory result. Both the patients had no syndromic association or associated anomaly. All cases of MCL require evaluation for associated abnormalities. Isolated MCL can be repaired surgically with a good outcome.

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Toward clinical and molecular dissection of frontonasal dysplasia with facial skin polyps: From Pai syndrome to differential diagnosis through a series of 27 patients

Lehalle

Bruel

Vitobello

et al. 2022

American J of Med Genetics Pt A

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Unique or multiple congenital facial skin polyps are features of several rare syndromes, from the most well‐known Pai syndrome (PS), to the less recognized oculoauriculofrontonasal syndrome (OAFNS), encephalocraniocutaneous lipomatosis (ECCL), or Sakoda complex (SC). We set up a research project aiming to identify the molecular bases of PS. We reviewed 27 individuals presenting with a syndromic frontonasal polyp and initially referred for PS. Based on strict clinical classification criteria, we could confirm only nine (33%) typical and two (7%) atypical PS individuals. The remaining ones were either OAFNS (11/27—41%) or presenting with an overlapping syndrome (5/27—19%). Because of the phenotypic overlap between these entities, OAFNS, ECCL, and SC can be either considered as differential diagnosis of PS or part of the same spectrum. Exome and/or genome sequencing from blood DNA in 12 patients and from affected tissue in one patient failed to identify any replication in candidate genes. Taken together, our data suggest that conventional approaches routinely utilized for the identification of molecular etiologies responsible for Mendelian disorders are inconclusive. Future studies on affected tissues and multiomics studies will thus be required in order to address either the contribution of mosaic or noncoding variation in these diseases.

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Median cleft of the upper lip: A new classification to guide treatment decisions

Cited by 14 publications

References 99 publications

Median Facial Cleft

Median Facial Cleft

Median cleft lip: an uncommon and unique anomaly

Toward clinical and molecular dissection of frontonasal dysplasia with facial skin polyps: From Pai syndrome to differential diagnosis through a series of 27 patients

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