Fetal CHAOS

Classification according to ICD-10
Q31.8	Other congenital malformations of the larynx - atresia
ICD-10 online (WHO version 2019)

Fetal CHAOS , acronym for English C ongenital H igh A irway ( upper airways ) O bstruktion S yndrom , is a very rare congenital , often lethal extending disorder of the lung development with accompanying cardiac insufficiency during pregnancy , triggered by a (possibly incomplete) closure of the upper respiratory tract .

Classification

There are three forms:

complete laryngeal atresia without tracheo-oesophageal fistula
complete laryngeal atresia with tracheo-oesophageal fistula
almost complete upper airway obstruction.

Clinical manifestations

During pregnancy, fluid always forms in the lungs of the unborn child. The occlusion leads to congestion, so that the lung volume and the pleural pressure become unnaturally larger and, as a result, the growth and further development of the heart is prevented and the cardiac output is reduced.

Also ascites is frequently observed in fetal CHAOS.

There is an association with Fraser syndrome and other malformations .

Diagnosis

The main method in prenatal diagnosis is sonography , which can detect the following changes from the first trimester:

Expansion of the trachea distal to the obstruction
enlarged and echogenic lungs
Flattening to inversion of the diaphragm
fetal ascites
Displacement and compression of the heart

possibly fetal anasarca and polyhydramnios

The same abnormalities can also be detected immediately after the birth.

Another imaging method is available with fetal MRI .

therapy

By means of fetal surgery may be trying to open the upper airways even during pregnancy again to relieve heart and lungs. With a specialized delivery procedure (EXIT) involving anesthetists , neonatologists , ear, nose and throat doctors and obstetricians, it is possible to initiate ventilation therapy. If left untreated, this disease is almost invariably fatal.

literature

D. Paoletti, M. Robertson: Congenital high airways obstruction syndrome - first and second trimester diagnosis. In: Australasian journal of ultrasound in medicine. Volume 17, number 2, May 2014, pp. 82-84, doi : 10.1002 / j.2205-0140.2014.tb00111.x , PMID 28191213 , PMC 5024938 (free full text).
T. Kohl, P. Van de Vondel, R. Stressig, HC Wartenberg, A. Heep, S. Keiner, A. Müller, A. Franz, S. Fröhlich, W. Willinek, U. Gembruch: Percutaneous fetoscopic laser decompression of congenital high airway obstruction syndrome (CHAOS) from laryngeal atresia via a single trocar – current technical constraints and potential solutions for future interventions. In: Fetal Diagnosis and Therapy . Volume 25, number 1, 2009, pp. 67-71, doi : 10.1159 / 000200017 , PMID 19202341 .

MH Hedrick, MM Ferro, RA Filly, AW Flake, MR Harrison, NS Adzick: Congenital high airway obstruction syndrome (CHAOS): a potential for perinatal intervention. In: Journal of pediatric surgery. Volume 29, Number 2, February 1994, pp. 271-274, PMID 8176604 .

Individual evidence

↑ ^a ^b ^c Radiopaedia
↑ ^a ^b emedicine
↑ CJ Hartnick, M. Rutter, F. Lang, JP Willging, RT Cotton: Congenital high airway obstruction syndrome and airway reconstruction: an evolving paradigm. In: Archives of Otolaryngology - Head & Neck Surgery. Volume 128, Number 5, May 2002, pp. 567-570, PMID 12003589 .
^ ME Adin: Congenital high airway obstruction syndrome (CHAOS) associated with cervical myelomeningocele. In: Journal of clinical ultrasound: JCU. Volume 45, Number 8, October 2017, pp. 507-510, doi : 10.1002 / jcu.22430 , PMID 27874207 .
^ V. Lago Leal, LM Cortés, C. Seco Del Cacho: Prenatal diagnosis of congenital high airway obstruction syndrome. In: The Indian journal of radiology & imaging. Volume 28, number 3, 2018 Jul-Sep, pp. 366-368, doi : 10.4103 / ijri.IJRI_396_16 , PMID 30319217 , PMC 6176680 (free full text).
^ R. Sharma, AK Dey, S. Alam, K. Mittal, H. Thakkar: A Series of Congenital High Airway Obstruction Syndrome - Classic Imaging Findings. In: Journal of clinical and diagnostic research: JCDR. Volume 10, Number 3, March 2016, pp. TD07 – TD09, doi : 10.7860 / JCDR / 2016 / 15243.7463 , PMID 27134966 , PMC 4843351 (free full text).
↑ LD Padmanabhan, S. Nampoothiri: Prenatal detection of congenital high airway obstruction syndrome with encephalocele. In: The Indian journal of radiology & imaging. Volume 26, number 1, 2016 Jan-Mar, pp. 70-72, doi : 10.4103 / 0971-3026.178336 , PMID 27081227 , PMC 4813078 (free full text).
↑ SH Fang, R. Ocejo, M. Sin, NN Finer, BP Wood: Radiological cases of the month. Congenital laryngeal atresia. In: American journal of diseases of children. Volume 143, Number 5, May 1989, pp. 625-627, PMID 2719001 .
↑ Thomas Kohl (DZFT) et al .: Fetoscopic and ultrasound ‐ guided decompression of the fetal trachea in a human fetus with Fraser syndrome and congenital high airway obstruction syndrome (CHAOS) from laryngeal atresia. Retrieved August 9, 2018 .

[radio-1] Radiopaedia

[emed-2] ↑ ^a ^b emedicine

[Hartnick-3] CJ Hartnick, M. Rutter, F. Lang, JP Willging, RT Cotton: Congenital high airway obstruction syndrome and airway reconstruction: an evolving paradigm. In: Archives of Otolaryngology - Head & Neck Surgery. Volume 128, Number 5, May 2002, pp. 567-570, PMID 12003589 .

[4] ME Adin: Congenital high airway obstruction syndrome (CHAOS) associated with cervical myelomeningocele. In: Journal of clinical ultrasound: JCU. Volume 45, Number 8, October 2017, pp. 507-510, doi : 10.1002 / jcu.22430 , PMID 27874207 .

[5] V. Lago Leal, LM Cortés, C. Seco Del Cacho: Prenatal diagnosis of congenital high airway obstruction syndrome. In: The Indian journal of radiology & imaging. Volume 28, number 3, 2018 Jul-Sep, pp. 366-368, doi : 10.4103 / ijri.IJRI_396_16 , PMID 30319217 , PMC 6176680 (free full text).

[6] R. Sharma, AK Dey, S. Alam, K. Mittal, H. Thakkar: A Series of Congenital High Airway Obstruction Syndrome - Classic Imaging Findings. In: Journal of clinical and diagnostic research: JCDR. Volume 10, Number 3, March 2016, pp. TD07 – TD09, doi : 10.7860 / JCDR / 2016 / 15243.7463 , PMID 27134966 , PMC 4843351 (free full text).

[7] LD Padmanabhan, S. Nampoothiri: Prenatal detection of congenital high airway obstruction syndrome with encephalocele. In: The Indian journal of radiology & imaging. Volume 26, number 1, 2016 Jan-Mar, pp. 70-72, doi : 10.4103 / 0971-3026.178336 , PMID 27081227 , PMC 4813078 (free full text).

[8] SH Fang, R. Ocejo, M. Sin, NN Finer, BP Wood: Radiological cases of the month. Congenital laryngeal atresia. In: American journal of diseases of children. Volume 143, Number 5, May 1989, pp. 625-627, PMID 2719001 .

[9] Thomas Kohl (DZFT) et al .: Fetoscopic and ultrasound ‐ guided decompression of the fetal trachea in a human fetus with Fraser syndrome and congenital high airway obstruction syndrome (CHAOS) from laryngeal atresia. Retrieved August 9, 2018 .