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.

Objective Assessment of the Nasal Airway in Unilateral Cleft Lip and Palate - a Long-term Study

2009 ◽  
pp. 091202121239062
Author(s):  
Maria Mani ◽  
Staffan Morén ◽  
Ornolfur Thorvardsson ◽  
Olafur Jakobsson ◽  
Valdemar Skoog ◽  
...  
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Objective Assessment ◽  
Nasal Airway ◽  
Term Study ◽  
Long Term Study

Treatment of Unilateral Cleft Lip and Palate Patient With Intraoral Tooth Tissue Borne Distractor and Facemask Therapy: A Case Report

2021 ◽  
pp. 105566562199336
Author(s):  
Akansha Bansal Agrawal ◽  
Harshavardhan Kidiyoor ◽  
Anand K. Patil Morth
Keyword(s):  
Case Report ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Face Mask ◽  
Rapid Maxillary Expansion ◽  
Growth Potential ◽  
Maxillary Expansion ◽  
Long Term Stability ◽  
Tooth Tissue

This case report demonstrates the successful use of intraoral distractor/hygenic rapid expander (HYRAX) for rapid maxillary expansion in anteroposterior direction with an adjunctive use of face mask therapy for anterior orthopedic traction of maxillary complex in a cleft patient with concave profile. The patient was a 13-year-old girl who reported with a chief complaint of backwardly positioned upper jaw and a severely forward positioned lower jaw. Therefore, a treatment was chosen in which acrylic bonded rapid maxillary expansion was done with tooth tissue borne intraoral distractor/HYRAX having a different activation schedule along with Dr Henri Petit facemask to treat maxillary retrognathism. As a result, crossbite got corrected and attained a positive jet with no bone loss in cleft area over a period of 5 months which was followed by fixed mechanotherapy achieving a well settled occlusion in 1 year. After completion of expansion and fixed mechanotherapy, ANB became +1 post-treatment which was −4 pretreatment. The prognathic profile was markedly improved by expansion and taking advantage of the remaining growth potential, thus minimizing the chances of surgery later in life. This provided a viable alternative to orthognathic surgery with good long-term stability.

Repair of Protruding Bilateral Cleft Lip and Palate With Staged Premaxilla Setback Osteotomy, Cheiloplasty, and Palatoplasty in Trisomy 17p Patient: A Review of Syndromic Clinical Characteristic

2021 ◽  
pp. 105566562110698
Author(s):  
Kristaninta Bangun ◽  
Jessica Halim ◽  
Vika Tania
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Surgical Techniques ◽  
Chromosome 17 ◽  
Long Term Effects ◽  
Surgical Strategies ◽  
Increased Risk ◽  
The Cost

Chromosome 17 duplication is correlated with an increased risk of developmental delay, birth defects, and intellectual disability. Here, we reported a female patient with trisomy 17 on the whole short arm with bilateral complete cleft lip and palate (BCLP). This study will review the surgical strategies to reconstruct the protruding premaxillary segment, cleft lip, and palate in trisomy 17p patient. The patient had heterozygous pathogenic duplication of chromosomal region chr17:526-18777088 on almost the entire short arm of chromosome 17. Beside the commonly found features of trisomy 17p, the patient also presented with BCLP with a prominent premaxillary portion. Premaxillary setback surgery was first performed concomitantly with cheiloplasty. The ostectomy was performed posterior to the vomero-premaxillary suture (VPS). The premaxilla was firmly adhered to the lateral segment and the viability of philtral flap was not compromised. Two-flap palatoplasty with modified intravelar veloplasty (IVV) was performed 4 months after. Successful positioning of the premaxilla segment, satisfactory lip aesthetics, and vital palatal flap was obtained from premaxillary setback, primary cheiloplasty, and subsequent palatoplasty in our trisomy 17p patient presenting with BLCP. Postoperative premaxillary stability and patency of the philtral and palatal flap were achieved. Longer follow-up is needed to evaluate the long-term effects of our surgical techniques on inhibition of midfacial growth. However, the benefits that the patient received from the surgery in improving feeding capacity and facial appearance early in life outweigh the cost of possible maxillary retrusion.

scholarly journals Steno-Fallots tetralogi og Bartholin-Pataus syndrom. En hjertemisdannelse og et misdannelsessyndrom første gang beskrevet af danske anatomer i 1600-tallet

2015 ◽  
Vol 54 ◽  
pp. 197
Author(s):  
Jesper Brandt Andersen ◽  
Niels W. Bruun
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Trisomy 13 ◽  
Latin Text ◽  
Female Fetus ◽  
Left Hand ◽  
Anatomy And Physiology ◽  
Patau Syndrome ◽  
Heart Malformation ◽  
The Right

Jesper Brandt Andersen & Niels W. Bruun: Tetralogy of Steno-Fallot and Bartholin-Patau syndrome. A heart malformation and a malformation syndrome first described by Danish anatomists in the seventeenth century. The heart malformation tetralogy of Steno-Fallot was first described by the Danish anatomist Niels Stensen (Nicolaus Steno) (1638–1686) in Thomas Bartholin’s Acta Medica & Philosophica Ann. 1671 & 1672 in 1673, but this was not discovered until 1942. Stensen’s description was built upon a dissection of a female fetus, which he made during his stay in Paris 1664–1665. We bring the first full Danish translation of Stensen’s Latin text and an analysis of his description in relation to his contemporaries and the present. Stensen describes three of the four elements of the tetralogy described in three adult patients by Fallot in 1888, namely ventricular septal defect, pulmonic stenosis and dexteriority of the aorta. The fact that Stensen does not mention the hypertrophy of the right ventricle may have two good reasons. Firstly, the difference between the wall thickness of the right and left ventricles is generally less pronounced in a fetus than after the birth and this would be expected even more in a heart malformation with overload on the right ventricle.Secondly, Stensen may have considered the right sided hypertrophy as merely a result of the three other elements of the tetralogy than as a malformation in itself.Stensen’s description reveals an impressive knowledge about the circulation of the blood in the heart of a fetus, and we speculate that he may have been the first in history to deliver such a precise description, not only of the anatomy and physiology of the tetralogy of Steno-Fallot, but also of the anatomy and physiology of the blood circulation in the fetal heart. Stensen’s fetus had several other malformations, i.e. cleft lip and palate, schisis of the abdomen and thorax and syndactyly of the second to fifth fingers on the left hand. We suggest that the fetus may have had acrofacial dysostosis 1 (Nager syndrome), which is caused by a mutation on chromosome 1q21.2.Likewise, Stensen’s mentor, the Danish anatomist Thomas Bartholin (1616–1680), was the first to describe a case report of the Bartholin-Patau syndrome in his Historiarum anatomicarum rariorum Centuria III & IV in 1657, but this was not discovered until 1960, the same year as Patau and collaborators showed that this syndrome is caused by trisomy 13. We bring the first full Danish translation of Bartholin’s Latin text with an analysis in relation to his age and the present.

Alveolar Bone Formation in Patients with Unilateral and Bilateral Cleft Lip and Palate after Early Secondary Gingivoalveoloplasty: Long-Term Results

2007 ◽  
Vol 119 (5) ◽  
pp. 1527-1537 ◽  
Author(s):  
Maria Costanza Meazzini ◽  
Chiara Tortora ◽  
Alberto Morabito ◽  
Giovanna Garattini ◽  
Roberto Brusati
Keyword(s):  
Bone Formation ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Alveolar Bone ◽  
Long Term Results ◽  
Bilateral Cleft Lip

Long-Term Stability of Alveolar Bone Graft in Cleft Lip and Palate Patients

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Arezoo Jahanbin ◽  
Elaheh Kamyabnezhad ◽  
Mohammad Ali Raisolsadat ◽  
Fahimeh Farzanegan ◽  
Erfan Bardideh
Keyword(s):  
Bone Graft ◽  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Alveolar Bone ◽  
Term Stability ◽  
Long Term Stability
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