Cleft Palate and Beckwith-Wiedemann Syndrome

2005 ◽  
Vol 42 (2) ◽  
pp. 212-217 ◽  
Author(s):  
Carine Laroche ◽  
Sylvie Testelin ◽  
Bernard Devauchelle

Objective Patients with Beckwith-Wiedemann syndrome suffer numerous anomalies, which vary somewhat from case to case. Cleft palate in combination with this syndrome has rarely been reported in the literature. Through two cases, this report examines the staging of the surgical repairs and the role of macroglossia in cleft palate and the consequences of the scarred palate on mandibular development. Results Of four patients with Beckwith-Wiedemann syndrome, only two had a cleft palate. The timing of the repair in these two children was different. Speech development was satisfactory in the first case but mediocre in the second. This result seemed to be related to a poor social environment. Mandibular prognathism persisted in both cases. Conclusion The treatment of patients with cleft palate and Beckwith-Wiedemann syndrome remains complex. It is preferable not to operate on a cleft palate before performing a tongue reduction plasty, but rather to combine these two surgical interventions. This would reduce the risks of anesthesia and enable the palate to heal more efficiently. Surgical treatment should be performed after the age of 6 months and before problems in speech development occur. An orthognathic surgery at adolescence could be performed if prognathism persists. While the origin of the cleft palate is still being discussed, we cannot claim that macroglossia is related to the development of cleft palate, nor that the scarred palate has an impact on the mandibular development.

1986 ◽  
Vol 12 (2) ◽  
pp. 13-14
Author(s):  
Joy Moeller

Introduction: The trauma of orthognathic surgery on muscle function has frequently been overlooked by oral surgeons. The attitude of many surgeons is that the muscle fibers will adapt to the new structure in a short time. The purpose of this paper is to dispute this attitude and to address the need for the oral rnyologist to assume an important role in the healing and post-surgical treatment of the patient.


1999 ◽  
Vol 48 (2) ◽  
pp. 82-83
Author(s):  
V. V. Abramchenko

The role of ischemic-duodenal insufficiency (IDI) in spontaneous termination of pregnancy has not been fully elucidated. The development of various modifications of the surgical treatment of IDI is of great importance, which has led to a very wide use of surgical interventions on the cervix, including "prophylactic" interventions. The application of a circular suture to the cervix is appropriate only in cases where there is an organic variant of this pathology. Differential diagnosis of organic and functional IDI in pregnancy is difficult.


2018 ◽  
Author(s):  
Oksana A Jackson ◽  
Alison E Kaye ◽  
David W Low

A cleft of the palate represents one of the most common congenital anomalies of the craniofacial region. Palatal clefting can occur in combination with a cleft of the lip and alveolus or as an isolated finding and can vary significantly in severity. The intact palate is a structure that separates the oral and nasal cavities, and the function of the palate is to close off the nasal cavity during deglutition and to regulate the flow of air between the nose and mouth during speech production. An unrepaired cleft palate can thus result in nasal regurgitation of food and liquid, early feeding difficulties, and impaired speech development. The goals of surgical repair are to restore palatal integrity by closing the cleft defect and repairing the musculature to allow for normal function during speech. The secondary goal of cleft palate repair is to minimize deleterious effects on growth of the palate and face, which can be impacted by standard surgical interventions. This review describes two of the most commonly performed cleft palate repair techniques in use today, as well as highlighting special anatomic considerations, summarizing perioperative care, and reviewing postoperative complications and their management. This review contains 11 figures, 2 videos, 3 tables and 63 references Key words: cleft, cleft team, Furlow, orofacial, oronasal fistula, palatoplasty, speech, submucous cleft, velopharyngeal insufficiency


2018 ◽  
Author(s):  
Oksana A Jackson ◽  
Alison E Kaye ◽  
David W Low

A cleft of the palate represents one of the most common congenital anomalies of the craniofacial region. Palatal clefting can occur in combination with a cleft of the lip and alveolus or as an isolated finding and can vary significantly in severity. The intact palate is a structure that separates the oral and nasal cavities, and the function of the palate is to close off the nasal cavity during deglutition and to regulate the flow of air between the nose and mouth during speech production. An unrepaired cleft palate can thus result in nasal regurgitation of food and liquid, early feeding difficulties, and impaired speech development. The goals of surgical repair are to restore palatal integrity by closing the cleft defect and repairing the musculature to allow for normal function during speech. The secondary goal of cleft palate repair is to minimize deleterious effects on growth of the palate and face, which can be impacted by standard surgical interventions. This review describes two of the most commonly performed cleft palate repair techniques in use today, as well as highlighting special anatomic considerations, summarizing perioperative care, and reviewing postoperative complications and their management. This review contains 11 figures, 2 videos, 3 tables and 63 references Key words: cleft, cleft team, Furlow, orofacial, oronasal fistula, palatoplasty, speech, submucous cleft, velopharyngeal insufficiency


2020 ◽  
Vol 8 (12) ◽  
Author(s):  
Vítor Bruno Teslenco ◽  
Maylson Alves Nogueira Barros ◽  
Herbert de Abreu Cavalcanti ◽  
Guilherme Nucci dos Reis

Introdução: a macroglossia é uma condição incomum e pode ser caracterizada como uma desordem por hipertrofia muscular, podendo ter também origem tumoral, endócrina, causas congênitas e doenças adquiridas. Inúmeras são as técnicas cirúrgicas para correção desta anomalia, porém, o plano de tratamento deve ter como base a etiologia da macroglossia. A correção cirúrgica objetiva a retomada de forma e função da língua, reestabelecendo a capacidade mastigatória, respiratória, fonética e estabilidade oclusal. Objetivo: relatar a comunidade científica um caso de glossectomia parcial para correção de um quadro de macroglossia, facilitando assim, a estabilidade do tratamento ortodôntico do paciente. Relato de caso: Paciente de 50 anos, leucoderma, em tratamento por ortodontia corretiva há mais de 18 meses, sem sucesso observado. Devido ao quadro de instablidade ortodôntica, maloclusão sem resolução passiva, macroglossia diagnosticada e redução da capacidade respiratória foi optado por realizar a glossectomia parcial. O procedimento foi realizado sob anestesia geral, onde removemos o tecido muscular a partir da abordagem de buraco de fechadura (Técnica de Kole). Conclusão: constatamos no presente caso, que a técnica de buraco de fechadura empregada neste paciente se mostrou eficaz, uma vez que obtivemos uma melhora estética e funcional, diminuindo o comprimento e largura da língua. Da mesma maneira, o tratamento ortodôntico foi passível de ser finalizado.Descritores: Macroglossia; Cirurgia Bucal; Glossectomia.ReferênciasTopouzelis N, Iliopoulos C, Kolokitha OE. Macroglossia. Int Dent J. 2011;61(2):63-9.Neville BW, Allen CM, Damm DD, Chi AC. Patologia: oral e maxilofacial. 4.ed.  Rio de Janeiro: Guanabara Koogan; 2016.Gadiwalla Y, Burnham R, Warfield A, Praveen P. Surgical management ofmacroglossia secondary to amyloidosis. BMJ Case Rep. 2016:10.1136.Salmen FS, Dedivitis RA. Partial glossectomy as an auxiliary method to orthodontic treatment of dentofacial deformity. Int Arch Otorhinolaryngol. 2012;16(3):414-17.Costa SAP, Brinhole MCP, da Silva RA, Dos Santos DH, Tanabe MN. Surgical treatment of congenital true macroglossia. Case Rep Dent. 2013;2013:489194.Balaji SM. Reduction glossectomy for large tongues. Ann Maxillofac Surg. 2013;3(2):167-72.Cymrot M, Teixeira FAA, Sales FCD, Muniz NFJ. Glossectomia subtotal pela técnica de ressecção lingual em orifício de fechadura modificada como tratamento de macroglossia verdadeira. Rev Bras Cir Plást. 2012;27(1):165-69.Tanaka OM, Guariza-Filho O, Carlini JL, Oliveira DD, Pithon MM, Camargo ES.Glossectomy as an adjunct to correct an open-bite malocclusion with shortenedmaxillary central incisor roots. Am J Orthod Dentofacial Orthop. 2013;144(1):130-40.Salmen FS, Dedivitis RA. Glossectomia parcial como método auxiliar ao tratamento ortodôntico da deformidade dento-facial. Int Arch Otorhinolaryngol.  2012;16(3):414-17.Jung YW, On SW, Chung KR, Song SI. Simultaneous glossectomy with orthognathic surgery for mandibular prognathism. Maxillofac Plast Reconstr Surg. 2014;36(5):214-18.Vieira CA. Fonoterapia em glossectomia total: estudo de caso. Rev Soc Bras Fonoaudiol. 2011;16(4):479-82.


Author(s):  
Mohammed Sulaimani ◽  
Abdulaziz Rashed Alshehri ◽  
Mohammed Hmoud ◽  
Najat Waggas ◽  
Ghaleb Aboalsamh

<p>Emphysematous gastritis (EG) is a rare disease entity related to infection of the gastric wall. It is caused by gas-forming pathogens including <em>Enterobacter</em> species, <em>Clostridium perfringens</em> and others. EG patients clinically present with symptoms similar to acute abdomen and can have a mortality that can reach up to 80%. Alcoholism was identified as a risk factor for such a disease. Different diseases like diabetes mellitus, ulcerative colitis and use of nonsteroidal anti-inflammatory agents were found to be associated with EG. To date, no clear guidelines regarding EG management, however, early detection and management is crucial to prevent related deaths. In the acute setting, the role of surgery is limited. Surgical interventions might be needed if obstruction of gastric outlet or perforation ensues. Fifty six years old male, heavy smoker, alcoholic with a history of ischemic heart disease, presented to Emergency Department with a complaint of severe epigastric abdominal pain for two days. The patient was vitally stable, conscious, oriented, however, looks in severe pain. His lab investigations were within normal ranges. Computed tomography (CT) of the abdomen was consistent with emphysematous gastritis. To our knowledge, this is the first case of emphysematous gastritis reported in Saudi Arabia using only radiological modality.</p>


2019 ◽  
Vol 23 (3) ◽  
pp. 143-145
Author(s):  
S. B. Suleymanova

The review is devoted to modern ideas of etiology, pathogenesis, methods of diagnostics and surgical treatment of cystic adenomatous malformation in children. The authors have made a review of domestic and foreign literature on the evolution of molecular genetics and cytogenetic studies. The role of histochemical and ultrastructural analysis in pre- and postnatal diagnostics of congenital lung diseases in children was assessed. The authors also discuss opinion of some specialists as to the terms and tactics of surgical interventions as well as advantages of minimally invasive surgery for congenital lung developmental anomalies.


1995 ◽  
Vol 85 (4) ◽  
pp. 189-197 ◽  
Author(s):  
GR Johnson ◽  
P Han ◽  
JA Giacopelli

The authors provide a review of the principles of talectomy as a surgical treatment option for severe, rigid, and resistant clubfoot deformities. Such deformities are associated with a high recurrence rate and frequently involve a large number of surgical interventions to attain a satisfactory result. Talectomy has been successfully used as both a primary and salvage procedure to treat such deformities, often eliminating the need for subsequent surgeries. A discussion of the operative technique, indications, advantages, and common complications associated with the procedure is presented. In addition, a case report of talectomy used to treat a severe, bilateral clubfoot deformity in a 4-year-old boy is included.


2014 ◽  
Vol 75 (S 02) ◽  
Author(s):  
S. Heredero ◽  
J. Solivera ◽  
A. Romance ◽  
A. Dean ◽  
J. Lozano

Sign in / Sign up

Export Citation Format

Share Document