scholarly journals Median cleft lip: an uncommon and unique anomaly

2019 ◽  
Vol 6 (8) ◽  
pp. 3035
Author(s):  
Vivek Parameswara Sarma
Keyword(s):  
Central Nervous System ◽  
Nasal Septum ◽  
Cleft Lip ◽  
Relevant Literature ◽  
Upper Lip ◽  
Craniofacial Malformations ◽  
Median Cleft ◽  
The Central Nervous System ◽  
Associated Abnormalities ◽  
Midline Cleft

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. 

scholarly journals Multiple Cephalic Malformations in a Calf

Animals ◽  
2020 ◽  
Vol 10 (9) ◽  
pp. 1532
Author(s):  
Di Muro G. ◽  
Cagnotti G. ◽  
Bellino C. ◽  
Capucchio M.T. ◽  
Colombino E. ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Congenital Malformations ◽  
Cleft Lip ◽  
Congenital Abnormalities ◽  
Presumptive Diagnosis ◽  
Intracranial Lesion ◽  
Median Cleft ◽  
The Central Nervous System ◽  
Similar Combination ◽  
Neurologic Findings

Congenital malformations of the central nervous system (CNS) can affect the CNS alone or the CNS and craniofacial structures. Here, we report an unusual and complex congenital cephalic malformation observed in a 3-day-old male crossbreed calf. Clinical examination disclosed a dome-shaped cranial vault, a flat face with a short snout, a median cleft lip, and increased intraorbital distance. The frontal region of the head was remarkable for a fluctuant, sac-like protrusion covered with haired skin. Neurologic findings suggested a multifocal intracranial lesion affecting the prosencephalon and the central vestibular system. While pathological and histopathological findings posited for a presumptive diagnosis of either hydranencephaly or holoprosencephaly associated with multiple congenital facial abnormalities, not all the findings could be definitely attributed to either of the two encephalic malformations alone. To our knowledge, a similar combination of severe congenital abnormalities affecting both the CNS and the craniofacial structures has not been reported in calves to date.

Median Cleft of the Lip: Its Significance and Surgical Repair

1993 ◽  
Vol 30 (1) ◽  
pp. 94-96 ◽  
Author(s):  
James Apesos ◽  
Gregg M. Anigian
Keyword(s):  
Surgical Technique ◽  
Cleft Lip ◽  
Surgical Repair ◽  
Surgical Reconstruction ◽  
Upper Lip ◽  
Facial Cleft ◽  
Median Cleft ◽  
Median Facial Cleft Syndrome ◽  
Associated Abnormalities

Median cleft lip is a midline vertical cleft through the upper lip in the absence of a prolabial remnant. This may occur as a sporadic event or be part of an inherited sequence of anomalies. A failure of formation or fusion of the medial nasal prominences derived from the frontonasal prominence is ultimately responsible for this aberration. Two categories of dysplasia are associated: (1) frontonasal deformity associated with hypotelorism and (2) median facial cleft syndrome associated with hypertelorism. A patient presents with median cleft lip, mild bifid nose, and hypertelorism. Following surgical reconstruction, a good result is achieved. The embryology, implications for associated abnormalities, and surgical technique for treating these cases are discussed.

Median cleft of the upper lip associated with lipomas of the central nervous system and cutaneous polyps

1987 ◽  
Vol 26 (4) ◽  
pp. 921-924 ◽  
Author(s):  
G. S. Pai ◽  
Abner H. Levkoff ◽  
Richard E. Leithiser ◽  
James F. Reynolds
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Upper Lip ◽  
Median Cleft ◽  
The Central Nervous System

scholarly journals The central nervous system lesion in amniotic rupture sequence

2018 ◽  
Vol 45 (S1) ◽  
pp. S4-S4
Author(s):  
P. Shannon
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Aqueductal Stenosis ◽  
Cranial Vault ◽  
Central Nervous System Lesion ◽  
Secondary Pathology ◽  
The Central Nervous System ◽  
Near Occlusion

We review the central nervous system anatomy in nine cases of amniotic rupture sequence, all of which had neuropathological examinations. Of these, four had normal brains, and in none of these was the cranial vault involved, and one had cleft lip and palate. Of the remaining five, all had portions of the scalp, calvarium and dura replaced by amnionic membrane directly overlying arachnoid. In one, the membrane covered a narrow necked large encephalocele, and the contained brain demonstrated extensive disruption and degeneration. In the remaining four, one demonstrated cranioplacental adhesion, and in three there was a broad based encephalocele covered in large part by amnion. Two of these four cases demonstrated holoprosencephaly. One case with holoprosencephaly and one without demonstrated marked aqueductal stenosis, and two of the four demonstrated aqueductal occlusion or near occlusion by neuroglial excrescences. None demonstrated ventriculomegaly. Three of these four cases demonstrate varying degrees of mechanical distortion and secondary pathology. We conclude that brains with amnionic rupture sequence demonstrate both malformation and deformation, which likely points to the embryonic stage origin of the lesion.

Lobar Holoprosencephaly with a Median Cleft: Case Report

2009 ◽  
Vol 46 (5) ◽  
pp. 549-554 ◽  
Author(s):  
Elżbieta Gawrych ◽  
Joanna Janiszewska-Olszowska ◽  
Anna Walecka ◽  
Maria Syryńska ◽  
Hanna Chojnacka
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Brain Abnormalities ◽  
Facial Deformity ◽  
Unique Case ◽  
Craniofacial Malformations ◽  
Live Births ◽  
Facial Defects ◽  
Median Cleft

Holoprosencephaly is a congenital abnormality of the prosencephalon associated with median facial defects. Its frequency is 1 in 250 pregnancies and 1 in 16,000 live births. The degree of facial deformity usually correlates with the severity of brain malformation. Early mortality is prevalent in severe forms. This report presents a child with lobar holoprosencephaly accompanied by median cleft lip and palate. The treatment and 9 months’ follow-up are presented. This unique case shows that holoprosencephaly may present different manifestations of craniofacial malformations, which are not always parallel to the severity of brain abnormalities. Patients with mild to moderate brain abnormalities may survive into childhood and beyond.

scholarly journals Median cleft lip: A new method of surgical repair

2010 ◽  
Vol 43 (01) ◽  
pp. 111-113
Author(s):  
B. V. Khandekar ◽  
S. Srinivasan ◽  
N. J. Mokal
Keyword(s):  
Cleft Lip ◽  
Surgical Repair ◽  
New Method ◽  
Muscle Repair ◽  
Median Cleft ◽  
Midline Cleft

ABSTRACTThe aim is to discuss a new method of muscle repair in midline cleft lip. Three patients with midline cleft lip were repaired with our technique of muscle repair and the results evaluated. Our new method of muscle repair in the form of ‘Z’ helps in forming the philtral dimple.

Large intraventricular epidermoid with bobble head doll syndrome in an adult

2017 ◽  
Vol 31 (2) ◽  
pp. 229-232
Author(s):  
Amit Agrawal
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Unusual Case ◽  
Relevant Literature ◽  
The Central Nervous System ◽  
Intracranial Epidermoid ◽  
Slow Growing

Abstract Intracranial epidermoid tumors are rare (0.2-1.8%), histologically benign, slow-growing, congenital neoplasms of the central nervous system arising from the retained ectodermal implants. We report an unusual case of intra third ventricular epidermoid in an adult presenting with the Bobble-head doll syndrome and review the relevant literature.

scholarly journals A Rare Case of a Combination of Tessier Cleft 0 and 3 in a 4-Year-Old Child—A Case Report

2019 ◽  
Vol 52 (02) ◽  
pp. 250-251
Author(s):  
Bibhuti Bhusan Nayak ◽  
M. Lopamudra
Keyword(s):  
Rare Case ◽  
Upper Lip ◽  
Successful Management ◽  
Transposition Flap ◽  
Satisfactory Outcome ◽  
Straight Line ◽  
Median Cleft ◽  
Median Cleft Face Syndrome ◽  
Midline Cleft ◽  
Rule Out

AbstractDescription and successful management of a patient with Tessier no. 0 and 3 facial cleft is being presented. Appropriate evaluation was done to rule out the presence of median cleft face syndrome. Lip cleft was repaired by straight line technique with staggering at the vermilion border. Nasal cleft was reconstructed by a transposition flap and the flap taken from the upper lip after correction of the midline cleft. Satisfactory outcome was achieved for this singular deformity by conforming to the basic tenets of plastic surgery.

scholarly journals Pai syndrome: a review

2020 ◽  
Vol 36 (11) ◽  
pp. 2635-2640
Author(s):  
Francesca Olivero ◽  
Thomas Foiadelli ◽  
Sabino Luzzi ◽  
Gian Luigi Marseglia ◽  
Salvatore Savasta
Keyword(s):  
Corpus Callosum ◽  
Cleft Lip ◽  
Case Reports ◽  
Case Series ◽  
Rare Condition ◽  
Frontonasal Dysplasia ◽  
Pubmed Database ◽  
Median Cleft ◽  
The Central Nervous System

Abstract Background Pai syndrome is a rare idiopathic developmental condition characterized by midline craniofacial abnormalities. It was originally described as the presence of a median cleft lip, cutaneous polyps of the nasal mucosa and face, and midline lipomas of the central nervous system, mostly at the corpus callosum. However, there is great phenotypical variability and these characteristics are rarely all present at once. Objective The aim of this review was to analyze the available evidence regarding Pai syndrome in order to better delineate this rare condition and its features. Methods We analyzed the PubMed database using the words “Pai syndrome”, “frontonasal dysplasia”, “cleft lip”, “nasal polyp”, “facial polyp”, and “corpus callosum lipoma”, including reviews, case reports and case series. Conclusion There is no consensus regarding the diagnostic criteria of Pai syndrome up to date. It is usually diagnosed at birth, and its incidence is often underestimated. At present, the etiology of Pai syndrome is unknown. Several hypotheses regarding its genetic background have been made; however, there are not enough data yet to elucidate this point. An improved awareness could help in diagnosing the condition and performing the necessary investigations. These patients should have a multidisciplinary follow-up.

Midline Facial Defects with Hypertelorism and Low-Grade Astrocytoma: A Previously Undescribed Association

2006 ◽  
Vol 43 (6) ◽  
pp. 748-751 ◽  
Author(s):  
Silvyo David Araújo Giffoni ◽  
Fernando Cendes ◽  
Marcelo Valente ◽  
Vera Lucia Gil-da-Silva-Lopes
Keyword(s):  
Magnetic Resonance Imaging ◽  
Central Nervous System ◽  
Nervous System ◽  
Magnetic Resonance ◽  
Cleft Lip ◽  
Low Grade ◽  
Resonance Imaging ◽  
Facial Defects ◽  
Median Cleft

We report on a child with midline facial defects with hypertelorism (MFDH), median cleft lip, sphenoidal ventriculocele, partial agenesis of the corpus callosum, and low-grade astrocytoma in the cervicomedullary junction. This combination of findings has not been reported previously. Although this association might be casual, it demonstrates a relationship between disorders of frontonasal process and posterior fossae. It also suggests that individuals with MFDH might require a prospective follow-up with central nervous system magnetic resonance imaging.

Sign in / Sign up

Export Citation Format

Share Document