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Laryngo-Tracheo-Esophageal Cleft

LC


Presentation

  • The authors present a female patient of type-IV laryngo-tracheo-esophageal cleft (LTEC) who survived the definitive operation and was discharged from the hospital.[ncbi.nlm.nih.gov]
  • A review of the literature did not reveal any reports of a similar presentation. We share our experience of an unusual and unexpected presentation of a case of LTEC.[ijaweb.org]
  • As evident from contrast esophagogram and endoscopy, the Type II LTEC was present in the reported case.[pediatriconcall.com]
  • Clinical description Depending on the severity of the malformation, patients may present with stridor, hoarse cry, swallowing difficulties, aspirations, cough, dyspnea and cyanosis through to early respiratory distress.[orpha.net]
  • Gregory J Stock}, journal {Zeitschrift fur Geburtshilfe und Perinatologie}, year {1988}, volume {192 4}, pages { 181-3 } } F Heitmann , Sevcan Erdem , 2 authors Gregory J Stock Published in Zeitschrift fur Geburtshilfe und Perinatologie 1988 A report is presented[semanticscholar.org]
Hypersomnia
  • The editors and contributing authors are internationally recognized authorities who cover everything from sleep and ADHD, headaches, restless leg syndrome, parasomnias, hypersomnias, autism, brain tumors, epilepsy, traumatic brain injury, and other primary[books.google.de]
Coarse Facial Features
  • Examination of the child at the time of admission revealed sick, febrile, anemic child with coarse facial features, dry and coarse skin and marked respiratory distress. Child was malnourished (weight 2.9 kg).[pediatriconcall.com]
Heart Disease
  • We believe that our procedure is a feasible choice for long-term survival in patients with a severe LTEC with congenital heart disease.[ci.nii.ac.jp]
  • Congenital heart disease, hypoparathyroidism, laryngo-tracheo-esophageal anomalies and autoimmune disorder (late onset autoimmune hypothyroidism) have been described in DiGeorge syndrome.[pediatriconcall.com]
  • Congenital heart disease has not been mentioned in previous reports of Feingold syndrome. Paramembranous VSD is a relatively common cardiac malformation and has as yet not required surgical closure in the patient reported herein.[nature.com]
  • The presence of comorbidities, such as congenital heart disease generally prompts close medical scrutiny of these neonates.[theajo.com]
Systolic Murmur
  • First heart sound was loud with grade III systolic murmur. Other systems were normal. Laboratory investigations revealed dimorphic anemia and toxic granulation in the peripheral blood film. Blood culture grew staphylococcus aureus.[pediatriconcall.com]
Drooling
  • It is therefore recommended the LTEC should be kept in mind in a newborn presenting with: (a) excessive frothiness and drooling of secretions (b) recurrent aspiration and choking during feed (c) repeated attacks of cyanosis and (d) recurrent pneumonias[pediatriconcall.com]
  • Two weeks after the surgery, patient developed copious drooling of secretions.[joacp.org]
  • In the neonate, excessive drooling is often the first symptom. If feedings are attempted, the baby often chokes, regurgitates, and becomes cyanotic.[emedicine.medscape.com]
Restless Legs Syndrome
  • The editors and contributing authors are internationally recognized authorities who cover everything from sleep and ADHD, headaches, restless leg syndrome, parasomnias, hypersomnias, autism, brain tumors, epilepsy, traumatic brain injury, and other primary[books.google.de]
Headache
  • The editors and contributing authors are internationally recognized authorities who cover everything from sleep and ADHD, headaches, restless leg syndrome, parasomnias, hypersomnias, autism, brain tumors, epilepsy, traumatic brain injury, and other primary[books.google.de]

Workup

  • History Physical examination FFL FEES Imagin airway films CXR MBS WORKUP—AIRWAY FILMS WORKUP—CXR WORKUP—MBS cumulative radiation provides little in the way of detailed laryngeal anatomy only evaluates a few cycles potential false negative if patient[laryngeal-cleft.com]
Staphylococcus Aureus
  • Blood culture grew staphylococcus aureus. Serum T3 level was 2.0 pg/dl (normal 3.1-8.6 pg/dl and Serum T4 was 0.76 μg/dl (normal 12-33 μg/dl) and TSH level of 18.94 μIU/ml (normal level 1.7-9.1 μIU/ml) were suggestive of hypothyroidism.[pediatriconcall.com]

Treatment

  • Management and treatment Once the cleft is diagnosed, it is essential to determine its length to orient the management and treatment approach.[orpha.net]
  • Multidisciplinary contributions on medical treatment, radiation oncology, and surgery and anesthesia are included. Highlights include new material on minimally invasive procedures and thoroughly updated diagnostic and treatment information.[books.google.de]
  • Treatment requires endoscopic or external surgery to close the cleft.[ncbi.nlm.nih.gov]
  • Sleep in Childhood Neurological Disorders features Dedicated focus on neurologically-based sleep disorders in children Diagnostic tables, key points, clinical pearls, and treatment algorithms throughout to enhance clinical utility Directions for "Future[books.google.de]
  • Focus on evidence-based treatments and outcomes to apply today’s best practices.[books.google.de]

Prognosis

  • Prognosis The prognosis is variable depending on the severity of the LC and associated malformations. Early diagnosis and appropriate treatment and management help to reduce mortality and morbidity.[orpha.net]
  • The prognosis is variable depending on the severity of the LC and associated malformations. Early diagnosis and appropriate treatment and management help to reduce mortality and morbidity.[ncbi.nlm.nih.gov]
  • Prognosis is always guarded, as review of 85 cases by Roth et al in 1983 revealed overall mortality of 46% (43% type I and II, 42% for type III and 93% for type IV).[pediatriconcall.com]
  • Advances in both surgical and anaesthetic techniques should continue to improve prognosis and reduce morbidity and mortality rates, which remain significant. Declaration of interest None declared.[academic.oup.com]
  • The long-term prognosis is good as these children outgrow their problem.[virtualpediatrichospital.org]

Etiology

  • Etiology Laryngeal clefts result from failure of fusion of the posterior cricoid lamina and abnormal development of the tracheo-esophageal septum.[orpha.net]
  • Etiology has been attributed to genetic factors, infections, and teratogens, but in most instances, no cause is identifiable.[emedicine.medscape.com]
  • Therefore, there is a need for waiving other possible etiologies of airway compromise such as associated cardiac or vascular malformations, gastro esophageal reflux, and esophageal stricture ( 6 , 17 – 19 ).[frontiersin.org]

Epidemiology

  • Summary Epidemiology The estimated annual incidence of LC is 1/10,000 to 1/20,000 live births, accounting for 0.2% to 1.5% of congenital malformations of the larynx.[orpha.net]
  • The epidemiology of tracheo-oesophageal fistula and oesophageal atresia in Europe. EUROCAT Working Group. Arch Dis Child 1993; 68:743. GeneReviews [Internet]. Pagon RA, Bird TD, Dolan CR, et al., editors.[pedclerk.uchicago.edu]
  • Richter 1955 first successful repair by Pettersson 1979 first endoscopic repair by Yamashita 1987 Tucker described submucouscleft 1989 Benjamin and Inglisdeveloped classification system most widely used today 2006 Sandumodified classification system EPIDEMIOLOGY[laryngeal-cleft.com]
Sex distribution
Age distribution

Pathophysiology

  • The association of complete laryngotracheoesophageal cleft with left lung agenesis: Pathophysiological clues provided by an experiment of nature. J Pediatr Surg 1998;33:1546-9. [Figure 1][ijaweb.org]
  • Pathophysiology TE fistulas occur due to abnormal septation of the caudal foregut during the fourth and fifth weeks of embryonic development.[pedclerk.uchicago.edu]

Prevention

  • We found that a large bore nasogastric tube (10 F) passed into the esophagus was useful in decompressing the stomach and keeping the ETT near the carina prevented its displacement.[joacp.org]
  • After providing sufficient explanation to the parents, laryngotracheal separation was performed at 1 year 7 months of age to prevent aspiration completely.[ci.nii.ac.jp]
  • Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets[books.google.de]
  • Management involves maintenance of satisfactory ventilation, prevention of secondary pulmonary complications as a result of repeated aspirations, and adequate feeding. Endotracheal intubation may be required for respiratory distress in severe cases.[ncbi.nlm.nih.gov]

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