Communicating bronchopulmonary foregut malformations: Classification and embryogenesis

Srikanth, Maya; Ford, Edward G.; Stanley, P; Mahour, G. Hossein

doi:10.1016/s0022-3468(05)80103-4

Cited by 146 publications

(127 citation statements)

References 34 publications

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“…3,6 In our patient, there was a bifid vertebra at D9 level with scoliosis, an ectopic left kidney and dextrocardia with patent foramen ovale. Thus, the patient can be characterized as a case of VACTERL group of anomalies.…”

Section: Discussionmentioning

confidence: 69%

Esophageal lung associated with VACTERL anomaly: a case report

et al. 2017

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Esophageal lung is an extremely rare type of bronchopulmonary foregut malformation in which the main stem bronchus is anomalously connected to the esophagus instead of the trachea and is supplied by branches of pulmonary artery. Less than 25 cases have been reported so far in literature. We hereby report first case of esophageal lung associated with VACTREL anomaly. An 8-month-old female child with weight of 4.7 kg presented with recurrent lower respiratory infections. She was diagnosed to have esophageal lung on basis of clinical examination, chest X-ray f/b computed tomogram of chest and esophagobronchoscopy. There was a bifid vertebra at D9 level with scoliosis, an ectopic left kidney and dextrocardia with patent foramen ovale. Thus, this case was classified as having VACTREL association. Patient was successfully operated for excision of the hypoplastic lung via thoracotomy. She recovered well and gaining weight at 3 months f/u without any respiratory infection since surgery. High index of suspicion is necessary for the diagnosis of esophageal lung when common causes of recurrent respiratory infections are ruled out.

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Section: Discussionmentioning

confidence: 69%

Esophageal lung associated with VACTERL anomaly: a case report

et al. 2017

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“…[2][3][4][5]7] Ameliyat sonrası komplikasyonlar arasında aspirasyon, fistül nüksü, anastomoz hattı striktürü veya kaça-ğı ile trakeomalazi sayılabilir. [2,3,8] …”

Section: Trakeoözofajiyal Fistül (Töf) Ile Birlikte Olan öZofagus Atrunclassified

“…Buji ve balon dilatasyonlarına cevap vermeyen çok dirençli fibröz bantlar bulunduran vakalarda ise perforasyon riski de göz önünde tutulmalı ve rezeksiyon-anastomoz planlanmalıdır. [3,7,8] …”

Section: Konjenital öZofajiyal Web (Perde) Ve Stenozunclassified

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Congenital malformations of the esophagus

Evman¹,

Demirhan²

2013

J Kartal TR

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Primary cilia are critical for tracheoesophageal septation

Fitzsimons,

Tasouri,

Willaredt

et al. 2023

Developmental Dynamics

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IntroductionPrimary cilia play pivotal roles in the patterning and morphogenesis of a wide variety of organs during mammalian development. Here we examined murine foregut septation in the cobblestone mutant, a hypomorphic allele of the gene encoding the intraflagellar transport protein IFT88, a protein essential for normal cilia function.ResultsWe reveal a crucial role for primary cilia in foregut division, since their dramatic decrease in cilia in both the foregut endoderm and mesenchyme of mutant embryos resulted in a proximal tracheoesophageal septation defects and in the formation of distal tracheo(broncho)esophageal fistulae similar to the most common congenital tracheoesophageal malformations in humans. Interestingly, the dorsoventral patterning determining the dorsal digestive and the ventral respiratory endoderm remained intact, whereas Hedgehog signaling was aberrantly activated.ConclusionsOur results demonstrate the cobblestone mutant to represent one of the very few mouse models that display both correct endodermal dorsoventral specification but defective compartmentalization of the proximal foregut. It stands exemplary for a tracheoesophageal ciliopathy, offering the possibility to elucidate the molecular mechanisms how primary cilia orchestrate the septation process. The plethora of malformations observed in the cobblestone embryo allow for a deeper insight into a putative link between primary cilia and human VATER/VACTERL syndromes.

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Communicating bronchopulmonary foregut malformations: Classification and embryogenesis

Cited by 146 publications

References 34 publications

Esophageal lung associated with VACTERL anomaly: a case report

Esophageal lung associated with VACTERL anomaly: a case report

Congenital malformations of the esophagus

Primary cilia are critical for tracheoesophageal septation

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