Paramedian nasal cleft

Paramedian nasal cleft is a rare developmental defect during embryogenesis characterized by a unilateral or bilateral coloboma of the nose, ranging in severity from a small notch, resulting in minor deviation of the nasal septum, to variable-sized clefts of the nasal ala which may be associated with small cysts or sinuses in the nasal midline. Defect may be isolated or may occur in association with cleft lip and/or other craniofacial anomalies (e.g. hypertelorism, broadening of nasal root, midline cleft). Dorsum and apex of nose are usually well preserved.

Microcephaly

Head circumference below 2 standard deviations below the mean for age and gender.

Total: 2

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PMID (PMCID)
24677696	MALE	Infant, Newborn
	Rare nasal cleft in a patient with holoprosencephaly due to a mutation in the ZIC2 gene.
	Savastano CP, Bernardi P, Seuanez HN, Moreira MA, Orioli IM. Birth Defects Res A Clin Mol Teratol. 2014;100(4):300-6.
	The male newborn presented with alobar HPE, microcephaly, ocular hypertelorism, upslanting palpebral fissures, a bulky nose with a left paramedian alar cleft.
12494439	MALE	Infant, Newborn
	Documentation of anomalies not previously described in Fryns syndrome.
	Arnold SR, Debich-Spicer D D, Opitz JM, Gilbert-Barness E. Am J Med Genet A. 2003;116A(2):179-82; discussion 183.
	We report on a case of Fryns syndrome with microcephaly, multiple facial anomalies, hypoplasia of distal phalanges, diaphragmatic defect with a thin, translucent diaphragm, microphthalmia (right), anophthalmia (left), and multiple midline developmental defects including gastroschisis, central nervous system defects including left arrhinencephaly and cerebellar hypoplasia, midline cleft of the upper lip, alveolar ridge and maxillary bone, and cleft nose with bilateral choanal atresia.