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Laryngotracheoesophageal cleft

Julie E Strychowsky, Reza Rahbar
Laryngotracheoesophageal clefts are rare congenital anomalies of the aerodigestive tract. Patients may present with airway and/or swallowing impairments. An approach to evaluation and management is presented. Important pearls for conservative and surgical management are discussed. Open versus endoscopic surgical techniques are reviewed.
June 2016: Seminars in Pediatric Surgery
Koji Fukumoto, Go Miyano, Masaya Yamoto, Hiroshi Nouso, Hiromu Miyake, Masakatsu Kaneshiro, Hideaki Nakajima, Mariko Koyama, Kyoko Mochizuki, Masato Shinkai, Naoto Urushihara
BACKGROUND/PURPOSE: In Japan, surgical repair of a laryngotracheoesophageal cleft (LTEC) typically consists of the anterior approach, with the lateral approach as an alternative. Endoscopic surgery to repair the tracheoesophageal septum has been reported, and this study reviewed our experience treating several cases of LTEC endoscopically. METHODS: Endoscopic repair of LTEC was performed in 7 patients (3 boys, 4 girls; age range 4 months to 2 years 10 months; mean age 11 months; mean weight at surgery 7...
October 2015: Journal of Pediatric Surgery
Evan J Propst
UNLABELLED: An infant with a type IV laryngotracheoesophageal cleft underwent transcervical repair using a long tapered cartilage graft. A laryngofissure was carried down to the innominate artery to expose the cleft that extended 5 mm inferior to the vessel. The edges of the cleft were excised, the trachealis was separated from the esophagus that was reapproximated, and clavicular periosteum was placed. A long posterior costal cartilage graft that tapered inferiorly in anteroposterior dimension and engaged behind the cricoid plate was inserted...
April 2016: Laryngoscope
Daniel P Ryan, Daniel P Doody
Congenital obstructions and anomalies of the pediatric airway are rare problems that may be associated with mild symptoms or critical stenoses that may be life threatening in the first few days of life. This review provides an overview of the embryologic development of the airway, different congenital anomalies associated with airway development, and surgical correction that may be associated with good long-term outcome.
October 2014: Seminars in Pediatric Surgery
Kyoko Mochizuki, Masato Shinkai, Hiroshi Take, Norihiko Kitagawa, Hidehito Usui, Fumio Asano, Hisayuki Miyagi, Kouji Fukumoto
Type IV laryngotracheoesophageal cleft (LTEC) is a rare congenital anomaly that is associated with high morbidity and mortality despite various forms of surgical repair. This article presents our strategy for surgical management of type IV LTECs using a combination of lateral thoraco-cervical and laryngoscopic approaches.
September 2014: Pediatric Surgery International
Yvonne K Y Cheng, Jin Huang, Kwok Ming Law, Yiu Man Chan, Tak Yeung Leung, Kwong Wai Choy
BACKGROUND: Prenatal sonographic diagnosis of Optiz G/BBB syndrome is difficult because the common clinical features, such as hypertelorism, hypospadias and abnormalities of midline structures, including laryngotracheoesophageal defects, are subtle. METHOD: Chromosomal microarray (CMA) analysis using a target enriched Fetal DNA Chip design was performed on the DNA of a fetus with congenital cardiac abnormalities. RESULTS: Fetal DNA chip revealed a 48Kb single copy number loss within chromosome region Xp22...
September 25, 2014: Clinica Chimica Acta; International Journal of Clinical Chemistry
Douglas R Johnston, Karen Watters, Lynne R Ferrari, Reza Rahbar
OBJECTIVES: Review the latest diagnostic and treatment modalities for laryngeal and laryngotracheoesophageal clefts as they can be a major cause of respiratory and feeding morbidity in the infant and pediatric population. METHODS: Literature review of published reports. RESULTS: The presentation of laryngeal cleft usually involves respiratory symptoms, such as stridor, chronic cough, aspiration, and recurrent respiratory infections. Clefts of the larynx and trachea/esophagus can occur in isolation, as part of a syndrome (Opitz-Frias, VATER/VACTERL, Pallister Hall, CHARGE), or with other associated malformations (gastrointestinal, genitourinary, cardiac, craniofacial)...
June 2014: International Journal of Pediatric Otorhinolaryngology
Crispin Leishman, Philippe Monnier, Yves Jaquet
OBJECTIVE: To review the presentation and evaluation of laryngotracheoesophageal clefts as well as their treatment modalities, especially endoscopic closure. STUDY DESIGN: retrospective case series. METHODS: All patients treated for laryngotracheoesophageal clefts in our clinic during the last 15 years were included. Analysis of preoperative data, surgical success and functional outcome was performed. RESULTS: A total of 18 patients were included in our study...
February 2014: International Journal of Pediatric Otorhinolaryngology
Gali Pariente, Danielle Landau, Micha Aviram, Reli Hershkovitz
We report 2 cases of a rare sonographic appearance of duodenal atresia. The first was associated with severe hydramnios and a small, centrally located stomach. After birth due to recurrent pneumonia, bronchoscopy revealed a type 4 laryngotracheoesophageal cleft. The second case had an enlarged stomach without dilatation of the duodenum or hydramnios. After birth, radiography showed an enlarged stomach and duodenum: the classic "double bubble." The appearance of duodenal atresia on sonography is diverse. It may show a double bubble but may also show a single bubble: the stomach...
November 2012: Journal of Ultrasound in Medicine: Official Journal of the American Institute of Ultrasound in Medicine
Shilpa Sharma, Amita Sen, Arti Maria, Jaswinder Kaur, Ashok Kumar, Rakesh Garg, Ajay Goila
No abstract text is available yet for this article.
June 2012: European Journal of Pediatric Surgery
James A Owusu, James D Sidman, Glen F Anderson
Laryngotracheoesophageal cleft (LTEC) is a rare congenital anomaly that results from failed posterior fusion of the cricoid cartilage and incomplete development of the tracheoesophageal septum. LTEC presents with increased secretions, respiratory distress, aspiration and recurrent pulmonary infections. The severity of presenting symptoms is dependent on the type of cleft. LTEC is most commonly classified into four types (I, II, III and IV) based on the inferior extent of the cleft. Types III and IV LTEC are associated with high morbidity and mortality and require timely diagnosis and repair for survival...
September 2011: International Journal of Pediatric Otorhinolaryngology
Christopher Dravis, Mark Henkemeyer
We report that the disruption of bidirectional signaling between ephrin-B2 and EphB receptors impairs morphogenetic cell-cell septation and closure events during development of the embryonic midline. A novel role for reverse signaling is identified in tracheoesophageal foregut septation, as animals lacking the cytoplasmic domain of ephrin-B2 present with laryngotracheoesophageal cleft (LTEC), while both EphB2/EphB3 forward signaling and ephrin-B2 reverse signaling are shown to be required for midline fusion of the palate...
July 1, 2011: Developmental Biology
Kenneth Geller, Young Kim, Jeffrey Koempel, Kathryn D Anderson
OBJECTIVE: The objective of this paper is to present our surgical experience with a cohort of four infants with laryngotracheoesophageal cleft (LTEC) in order to highlight our early failures and complications and to propose a comprehensive, three-layered approach in treating stages III and IV LTEC. METHOD: An IRB approved, retrospective chart review was carried out of a cohort of four cases occurring within a 2-year period of time. RESULTS: All patients had other significant anomalies, and the mortality rate was high: 75%...
June 2010: International Journal of Pediatric Otorhinolaryngology
Tatsuo Nakaoka, Sadashige Uemura, Tsunehiro Yano, Terutaka Tanimoto, Hiromu Miyake, Shingo Kasahara, Shunji Sano
A full-term newborn male infant presented with dyspnea and cleft lip and palate. He was thought to have esophageal atresia with tracheoesophageal fistula. He underwent bronchoscopy before operation that showed a laryngotracheoesophageal cleft (LTEC) type III. The left main bronchus originated from the lower esophagus. His diagnosis was communicating bronchopulmonary foregut malformation (CBPFM) type IA associated with LTEC type III. Enhanced chest computed tomographic scan showed the left pulmonary artery originated from the descending aorta...
May 2009: Journal of Pediatric Surgery
I Koerte, T Nicolai, I Joppich, D von Schweinitz, H-G Dietz
No abstract text is available yet for this article.
April 2009: European Journal of Pediatric Surgery
G da Silva Dalben, A Richieri-Costa, L A de Assis Taveira
OBJECTIVE: The G/BBB syndrome is an X-linked recessive disorder characterized by eye anomalies, laryngotracheoesophageal cleft, congenital heart disease, genitourinary anomalies and gastrointestinal disorders. Patients may also present cleft lip and palate, high-arched palate and thin upper lip. This study aimed to investigate the occurrence of tooth abnormalities and soft tissue changes in patients with G/BBB syndrome. DESIGN: Cross-sectional. SUBJECTS AND METHODS: Twenty-one patients with G/BBB syndrome were analyzed as to the presence of tooth abnormalities and soft tissue alterations...
November 2008: Oral Diseases
Joyce So, Ines Müller, Melanie Kunath, Susanne Herrmann, Reinhard Ullmann, Susann Schweiger
Opitz G/BBB syndrome (OS) is a congenital midline malformation syndrome characterized by hypertelorism, hypospadias, cleft lip/palate, laryngotracheoesophageal abnormalities, imperforate anus, developmental delay and cardiac defects. The X-linked form is caused by mutations in the MID1 gene, while no gene has yet been identified for the autosomal dominant form. Here, we report on a 15-year-old boy who was referred for MID1 mutation analysis with findings typical of OS, including apparent hypertelorism, hypospadias, a history of feeding difficulties, dysphagia secondary to esophageal arteria lusoria, growth retardation and developmental delay...
January 1, 2008: American Journal of Medical Genetics. Part A
J Penchyna-Grub, H Alvarez-Neri, G Blanco-Rodríguez, J D Porras Hernández
Laryngotracheoesophageal clefts (LTEC) are rare and potentially lethal malformations. Their therapeutic approach is still controversial. We report on a one month old infant with a type III LTEC who presented with aspiration and was fed through an orogastric tube for three weeks. Rigid endoscopy assessed the defect precisely. A percutaneous transendoscopic gastrostomy was done one week before the one stage anterior translaryngeal approach of the defect with a two-layer repair. The patient was orotracheally intubated, sedated and relaxed for four days after the repair...
April 2007: Cirugía Pediátrica: Organo Oficial de la Sociedad Española de Cirugía Pediátrica
Brice Antao, Giampiero Soccorso, Neil Bateman, Rang Shawis
H-type tracheoesophageal fistula and laryngotracheoesophageal cleft are both rare anomalies. Laryngotracheoesophageal clefts are identified as a part of Opitz-Frias syndrome. We report a neonate with this combination of rare congenital anomalies. These associated malformations can have major implications in terms of resuscitation, diagnosis and surgical management, which are discussed.
November 2007: European Archives of Oto-rhino-laryngology
Neeraj N Mathur, Giles J Peek, C Martin Bailey, Martin J Elliott
OBJECTIVE: To review our strategy for the treatment of patients with Type IV laryngotracheoesophageal cleft-a very rare congenital malformation. METHODS: Retrospective review of nine cases of Type IV laryngotracheoesophageal cleft managed between October 1994 and January 2004 at Great Ormond Street Hospital for Children, London. RESULTS: Three children were not operated upon (Group A) because of serious co-morbidity and parental decision not to opt for repair; they died at the ages of 2, 7 and 14 days, respectively...
November 2006: International Journal of Pediatric Otorhinolaryngology
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