Clinical Features and Management of a Median Cleft Lip

BACKGROUND: Median cleft lip is a rare anomaly consisting of a midline vertical cleft through the upper lip. It can also involve the premaxillary bone, the nasal septum, and the central nervous system. In our current report, we present the clinical features of 6 patients with a median cleft lip and their surgical management according to the accompanying anomalies. METHODS: From December 2010 to January 2014, 6 patients with a median cleft lip were reviewed. Five of these cases underwent surgical correction; alveolar bone grafting was performed in a patient with a median alveolar cleft. The surgical technique included inverted-U excision of the upper lip and repair of the orbicularis oris muscle. The mean follow-up period was 20.4 months (range, 7.4-44.0 months). RESULTS: The study patients presented various anomalous features. Five patients received surgical correction, 4 with repair of the median cleft lip, and one with iliac bone grafting for median alveolar cleft. A patient with basal sphenoethmoidal meningocele was managed with transoral endoscopic surgery for repair of the meningocele. Successful surgical repair was achieved in all cases with no postoperative complications. CONCLUSIONS: Relatively mild forms of median cleft lip can be corrected with inverted-U excision with good aesthetic outcomes. In addition, there is a broad spectrum of clinical features and various anomalies, such as nasal deformity, alveolar cleft, and short upper frenulum, which require close evaluation. The timing of the operation should be decided considering the presence of other anomalies that can threaten patient survival.

Medical Mystery: Ectrodactyly A Case Report.

Congenital absence of all or part of one ormore fingers or toes is defined as Ectrodactyly. It is the word derived from Greek ektromameaning “abortion” and dactylymeaning digits. Also called split hand or footmalformation (SHFM), Ectrodactyly is a rare limb malformation with median cleft of hand and foot and aplasia or hypoplasia of the phalanges, metacarpals andmetatarsals. Irregular pattern of inheritance exists for ectrodactyly;may be autosomal dominant, autosomal recessive or X linked. In this condition, themedian cleft of the hand gives the hand, the appearance of lobster claws- so also called ‘Lobster Claw Hand”. Ectrodactylymay be very commonly associated with other anomalies of face, eyes and skeletal system. We report a rare case of unilateral ectrodactyly of right hand .The present case is not associated with any other anomaly as described in various textbooks and reference articles. The details of etiology, diagnosis, genetic causes, association with other anomalies and treatment will be discussed.

Surgical Correction of a Median Cleft of the Upper Lip Associated with Enlarged Frenulum and Palatal Masses

PURPOSE: Median cleft of upper lip is defined as any congenital vertical cleft through the midline of the upper lip. It is uncommon, its embryological pathogenesis remains unexplained to date. The authors hereby report a rare case of median cleft of the upper lip associated with enlarged frenulum and palatal mass. This case offers some understanding of the possible embryologic development of this anomaly. METHODS: A 10-month-old boy born by normal vaginal delivery at full-term had a notch in the midline of the upper lip with widened philtrum along with enlarged median frenulum, alveolar cleft, and mass of the hard palate. We performed en bloc resection of the enlarged frenulum and palatal mass and cheiloplasty under general anesthesia. RESULTS: Histological examination revealed that the frenulum and palatal mass was consisted of fibrous tissue with normal mucous membrane. The postoperative course was satisfactory. CONCLUSION: A rare case of median cleft of the upper lip with associated enlarged frenulum and palatal mass was presented with proper surgical management. The surgical technique includes marginal excision of the clefted epithelium and reconstruction of orbicularis oris muscle, in addition to en bloc resection of the palatal mass and frenulotomy.

A Case Report of Holoprosencephaly

PURPOSE: Holoprosencephaly(HPE) is a rare developmental defect due to incomplete cleavages of the prosencephalon during the third week of fetal development. Chromosomal anomalies, genetic syndrome, teratogen, or genetic disorder of non-syndromic HPE are usually accepted as etiology. The consequences of prechordal mesoderm defect are varying degrees of deficit of midline facial development, especially the median nasal process(premaxilla), and incomplete morphogenesis of the forebrain. We experienced a case of lobar HPE with complete cleft lip and palate. METHODS: A female newborn infant was born at 38+6 weeks' gestational age via NSVD. The infant's birth weight was 3.6kg, height 52cm, and head circumference 32.5cm, showing microcephaly, flat nose, median complete cleft lip & palate, and hypotelorism, along with defects of midfacial development including losses of premaxilla, philtrum, nasal septum, and columella. RESULTS: There were no specific findings noted from the head and neck X-ray and tests for endocrine and metabolic disorders, but clinical characteristics of midface and dysgenesis corpus callosum on brain MRI were seen, so that this case was diagnosed with HPE. CONCLUSION: HPE is divided into three categories of alobar, semilobar, and lobar prosencephaly according to the degree of cerebral hemisphere separation. Assesment of patient's brain abnormality and malformation is essential in determining the extent and benefit of surgical intervention. This case was included in the lobar type HPE which shows relatively good prognosis compared with other types and reconstruction of median complete cleft lip & palate and midfacial defects will be performed.

Two Cases of the Incomplete Upper Median Cleft Lip: New Classification

A median cleft lip has been broadly defined as any congenital vertical cleft through the center of the upper lip. In the Tessier 0 cleft, the location of the median cleft of the upper lip has a spectrum of dysmorphic gradation. This varies from a simple central vermillion notch to a wide cleft accompanied by a bifid nose and hypertelorism. Despite its extreme variability, the median cleft is quite rare and has a reported incidence amongst cleft patients alone of 0.43 to 0.73%. We experienced two cases of incomplete median cleft lip without other craniofacial anomalies. One of them who had reverse V-shaped defect of upper vermilion and fibrous band in her oral mucosa, was corrected with orbicularis oris muscle union and Z-plasty. The other patient had triangular shaped upper lip and deficiency of vermilion. We reviewed the classifications of median cleft lip and devised new classification, so that it helps understanding of median cleft lip.

Median Cleft of the Lower Lip and Mandible: A Case Report

A Case of Median Cleft Face Syndrome

Median cleft face syndrome is diagnosed by two or more of the following anomalies : ocular hypertelorism, cranium bifidum occultum, median cleft nose, median cleft lip and median cleft premaxilla. The most consistent and prominent ocular finding associated with this syndrome is hypertelorism. We experienced a case of median cleft face syndrome which had orbital hypertelorism, cranium bifidum occultum, bifid cleft nose, V-shaped frontal hairline, and polysyndactly. We report this case with a brief review of the associated literature.