Facial Cleft No. 30

Keçeci, Yavuz; Gençosmanoğlu, Reha; Görken, Cenk; Cagdas, Arman

doi:10.1097/00001665-199409000-00015

Cited by 9 publications

(3 citation statements)

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“…One group of authors has proposed that the superior skin tag found in patients with this pathology is formed by a ventral outgrowth of tongue muscle [ 12 ]. Some have proposed that MCC is an inferior presentation of Tessier's facial cleft #30 which is a cleft of the mandible [ 19 – 21 ]. Karík included MCC under the category of branchiogenic disorders along with disorders of the mandible, tongue, lower lip, and thorax [ 16 ].…”

Section: Discussionmentioning

confidence: 99%

“…Those patients in whom the cord is apparent even without neck extension have difficulty extending their necks. When the fibrous cord extends to the level of the mandible, a bony spur is often seen on the anterior, inferior surface of the bone secondary to the traction placed on the mandible by this tethering cord which may be severe enough to produce an open bite deformity [ 11 , 19 , 91 , 102 , 111 ].…”

Section: Discussionmentioning

confidence: 99%

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Midline Cervical Cleft: Review of an Uncommon Entity

Puscas

2015

International Journal of Pediatrics

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Introduction. Midline cervical cleft is a rare congenital malformation which nonetheless has a classic presentation. This study presents one of the largest single series of new patients with MCC and provides an exhaustive review and catalogue of publications from the international literature. Materials and Methods. Retrospective chart review performed in two academic medical centers and literature review performed with primary verification of all quoted references. Results. Ten patients with MCC were identified (8 boys and 2 girls). All patients presented with the classic findings of this congenital anomaly, and the length of the skin defect correlated with an increase in the patient's age. Surgical excision was complete in all cases. Thorough international literature review yielded only 195 verifiable previously reported cases. Conclusions. This is one of the largest series of new patients with midline cervical cleft presented in the world literature. Although rare (with less than 200 cases published to date) this entity does have a reliable presentation that should lead to rapid and accurate diagnosis. Complete surgical excision at an early age is appropriate since the anomaly increases in length commensurate with the patient's age.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Midline Cervical Cleft: Review of an Uncommon Entity

Puscas

2015

International Journal of Pediatrics

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“…Published cases include clefting of the lower lip and mandible, lower jaw, lower lip, mandible and sternum, tongue, lower lip and mandible, and median cleft of the lower lip (Martinot-Duquennoy et al, 1991; Armstrong and Waterhouse, 1996; Aygit et al, 1999; Seyhan and Kylynr, 2002; Turki-Mehri et al, 2005; Benhammou et al, 2006). Additional cases include reports of medial lip fissure, median tongue clefts associated with clefts of the lower lip and mandible, cleft palate with intraoral hamartomas, association with orofaciodigital syndromes, hemangioma, total nasal agenesis, and mandibular clefts associated with bifid sternum, ventricular sepal defect, and bifid uvula (Chidzonga and Shija, 1988; Bartholdson et al, 1991; Kececi et al, 1994; Rosenquist, 1995; Chidzonga et al, 1996; Ozek et al, 2001; Ishii et al, 2002; Seyhan and Kylynr, 2002; Morioka et al, 2003; Morritt et al, 2007; Romero et al, 2007; Jank et al, 2008; Bhattacharya et al, 2009). These median mandibular clefts have been noted to range from minor clefting of the lower lip to absence of the manubrium, hyoid, and thyroid (Armstrong and Waterhouse, 1996).…”

Section: Discussionmentioning

confidence: 99%

A Unique Case of Tessier 30 and Bilateral Cleft Lip and Palate: A New Clinical Syndrome?

Adetayo

Martin

2013

The Cleft Palate Craniofacial Journal

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Background : Tessier 30 cleft is rare and sparsely reported in the literature. A unique case of an infant with a Tessier 30 cleft, bilateral cleft lip and palate, and other anomalies is presented. In addition to craniofacial anomalies, he had cardiac, gastrointestinal, and genitourinal defects. The constellation of these findings suggests the possibility of a new clinical syndrome. We present these findings and postoperative results following surgical treatment. Patient and Methods : A 37-week gestational male infant with multiple congenital anomalies is presented. Findings on clinical exam were notable for Tessier 30 median mandibular cleft, bilateral cleft of the lip and palate, and bifid tongue. Further workup revealed levocardia, perimembranous moderate-to-large ventricular septal defect, patent foramen ovale, double outlet right ventricle, intestinal malrotation, and bilateral undescended testicles. There were no extremity anomalies, and cytogenetic studies for 22q deletion were negative. Results : The preoperative, intraoperative, and postoperative findings and images are discussed. Conclusion : We present a unique case of a child with a Tessier 30 cleft associated with bilateral cleft lip and palate in the absence of intraoral masses or limb anomalies. Previous reports of median facial clefts have occurred either in the presence of intraoral hamartomas, suggesting the palatal defects are a result of sequence abnormalities, or in association with extremity findings consistent with the spectrum of orofaciodigital syndrome. The case we present is distinct and may represent a new clinical syndrome.

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