Alobar Holoprosencephaly (Arhinencephaly) with Median Cleft Lip and Palate: Clinical, Electroencephalographic and Nosologic Considerations (Part 2 of 2)

1963 ◽  
Vol 23 (1) ◽  
pp. 17-36 ◽  
Author(s):  
W. Demyer ◽  
W. Zeman
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Median Cleft

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.

Sphenoethmoidal meningoencephalocele associated with agenesis of corpus callosum and median cleft lip and palate

1979 ◽  
Vol 51 (3) ◽  
pp. 397-401 ◽  
Author(s):  
Katsuaki Sakoda ◽  
Susumu Ishikawa ◽  
Tohru Uozumi ◽  
Katsuhiro Hirakawa ◽  
Hideo Okazaki ◽  
...  
Keyword(s):  
Congenital Anomaly ◽  
Corpus Callosum ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Distinct Type ◽  
Agenesis Of Corpus Callosum ◽  
Content Type ◽  
Median Cleft ◽  
Fine Print

✓ A case of sphenoethmoidal meningoencephalocele associated with agenesis of the corpus callosum and median cleft lip and palate in a baby boy is reported. Two other cases have been reported previously presenting exactly the same findings. It is possible that these malformations constitute a distinct type of congenital anomaly.

scholarly journals THE APPLICATION OF STIMULATOR IN THE TREATMENT OF CLEFT LIP AND PALATE IN GOLDENHAR SYNDROME, TRISOMY 13 AND LOBAR HOLOPROSENCEPHALY WITH A MEDIAN CLEFT LIP

2018 ◽  
pp. 024
Author(s):  
Julija Radojičić
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Surgical Care ◽  
Trisomy 13 ◽  
Goldenhar Syndrome ◽  
Orthodontic Therapy ◽  
Median Cleft ◽  
Life Threatening ◽  
Oral Fixation

The clinical picture of a newborn with a syndromic cleft lip and palate is severe. The orthodontic and surgical treatment of cleft is complex and long-term. It is further complicated by various birth defects which can be life-threatening for a newborn or can make the therapy itself more difficult. The induction of a newborn into total anesthesia with a view to performing the surgery of a cleft is often made difficult or time-limited. This paper presents pre-surgical orthodontic therapy in newborns with three severe types of cleft, UCLP, BCLP and premaxillary agenesis with median cleft lip which occurred within three rare syndromes Goldenhar syndome, lobar holoprosencephaly with a median cleft lip and trisomy 13 (47XX+13). Pre-surgical orthodontic therapy was conducted by means of RBJ stimulators without extra oral fixation, whose construction was conditioned by the type of cleft. With active treatment of RBJ stimulators, the cleft area in all three types of cleft was significantly reduced, as well as the protrusion of the premaxilla in BCLP. By directing the growth of cleft segments of newborn’s upper jaw, the most approximate shape to a healthy newborn’s jaw shape is achieved. All three types of described stimulators used in the therapy of syndromic cleft lip and palate enabled primarily the feeding of newborns, and thus their survival. With their orthopedic treatment they created optimal conditions for successful performing of surgical care of syndromic cleft lip and palate.

scholarly journals Median facial cleft syndrome: A case report

2021 ◽  
Vol 7 (2) ◽  
pp. 61-63
Author(s):  
Kaushik Bhattacharya ◽  
Aditya Shikar Bhattacharya ◽  
Neela Bhattacharya
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Upper Lip ◽  
Live Births ◽  
Facial Cleft ◽  
The Face ◽  
Median Cleft ◽  
Median Facial Cleft Syndrome ◽  
Congenital Cleft ◽  
Type 3

Congenital Cleft Lip and Palate is a common birth defect with an incidence of 1 in 600 to 800 live births. This defect usually affects either the left, right or in some cases both sides of the lip and is called a Tessier Type 3 cleft. Clefting of the face in the midline is exceedingly rare and such a case of a Median Facial Cleft Syndrome is being reported which occurs in nearly 1 in 1,000,000 live births. This was first described by Bechard in 1823 and can be sporadic or part of an inherited syndrome. The child had a median cleft of the upper lip, nose, and palate of a severe, complete variety, with absent philtrum of the upper lip, premaxilla, columella, nasal septum and vomer.

Midline Defects of the Orofaciodigital Syndrome Type VI (Váradi Syndrome)

1994 ◽  
Vol 31 (5) ◽  
pp. 397-400 ◽  
Author(s):  
Philip D. Wey ◽  
Julie A. Neidich ◽  
Lloyd A. Hoffman ◽  
Gregory S. Latrenta
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Rare Disorder ◽  
Cranial Vault ◽  
Syndrome Type ◽  
Brain Malformation ◽  
Facial Growth ◽  
Midline Defects ◽  
Median Cleft ◽  
Hands And Feet

The orofaciodigital syndromes (OFDS) represent a spectrum of anomalies of the palate, cranium, hands, and feet. Váradi syndrome, designated OFDS type VI, is a rare disorder that is additionally characterized by cerebellar anomalies. The following report is of a patient with OFDS VI and characteristic multiple midline defects: median cleft lip and palate, lingual cleft with nodules, and midline brain malformation. In addition, this case is uniquely associated with the presence of midline (metopic and sagittal) craniosynostoses as well. It is unusual that deformities which result from premature fusion of cranial vault sutures would appear synchronously in a syndrome based on the concept of failure of fusion or coalescence of facial growth centers. The midline represents an independent developmental field, whereby CNS defects and midline anomalies can present concurrently.

scholarly journals Lip And Palate Reconstruction On Median Cerebrofacial Malformation Patient

1970 ◽  
Vol 2 (2) ◽  
Author(s):  
Nur Anindhawati ◽  
Prasetyanugraheni Kreshanti ◽  
Siti Handayani ◽  
Kristaninta Bangun
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Perinatal Care ◽  
Functional Limitation ◽  
Clinical Manifestations ◽  
Care Patient ◽  
Post Surgery ◽  
First Case ◽  
Patient Reported ◽  
Median Cleft

Background: Median cerebrofacial malformations are developmental anomalies of the midline brain and facial structures. The clinical manifestations ranges from holoprosencephaly with agenesis of central facial structures, to those seen in median facial dysplasia. Patients who survive are usually born with severe functional limitation and die during infancy. Nowadays, with improved perinatal care, patient with severe midline craniofacial abnormalities can be expected to live longer. The purpose of this paper is to show how reconstruction of the lip and palate on patient with median cerebrofacial malformation is done. Patient and Method: Reporting 2 cases of child with cleft median cerebrofacial malformations. First case was a 2 year-old child with complete median cleft lip and palate. Clinical examinations and CT scan revealed a holoprosencephaly. She was also diagnosed with laryngomalacia. The second case was a 4 month-old patient with median cleft lip and palate also microcephaly. We performed labioplasty in both patients and did the palatoplasty in the first patient. Result: Both cases showed no sign of complication after the operation. They both discharged one day after the operation. The first patient reported dead 1 week after the surgery at home. The cause was unknown. Discussion: Disturbed maxillary growth is postulated to be caused by the manipulation and suturing of the maxillary vomer, and scarring of the dentoalveoli post-surgery. Another contributing factor is the intrinsic tissue deficiency. Summary: With proper perinatal care and holistic teamwork that expand the survival rate we expect those with the best prognosis deserve to appear as normal as possible. Keywords: Holoprosencephaly, median facial dysplasia, median cleft lip and palate  

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