Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Case Reports
. 2020 Sep 11:2020:8835533.
doi: 10.1155/2020/8835533. eCollection 2020.

Urgent Airway Management and Postoperative Complications in a Patient with Trichorhinophalangeal Syndrome

Sarvie Esmaeilzadeh  1 , Ryan S D'Souza  1 , Thomas M Stewart  1 , Matthew A Sexton  1   2
Affiliations
Case Reports

Urgent Airway Management and Postoperative Complications in a Patient with Trichorhinophalangeal Syndrome

Sarvie Esmaeilzadeh et al. Case Rep Anesthesiol. .

Abstract

Trichorhinophalangeal syndrome (TRPS) is a genetic disorder that may pose anesthetic challenges. We present a case of airway management for urgent surgery in a 56-year-old female with TRPS and difficult airway (macroglossia, narrow glottic opening, and hypoplastic epiglottis). Intubation was successful with video laryngoscopy using a size 2.5 pediatric blade and size 5.0 endotracheal tube. During emergence, she experienced bronchospasm and persistent urosepsis, necessitating intensive care unit (ICU) admission. Her pulmonary reserve was hindered by a Morgagni hernia causing lung compression. Our case demonstrates challenges in TRPS including challenging airway, decreased pulmonary reserve, and joint laxity introducing potential for spinal cord injury.

PubMed Disclaimer

Conflict of interest statement

The authors have no potential conflicts of interest to disclose.

Figures

Figure 1
Figure 1
Axial (a) and coronal (b) views of CT (computed tomography) thorax, abdomen, and pelvis which revealed 6 mm left obstructing ureterolithiasis with hydronephrosis, as well as incidentally noted large 13 cm heterogeneous mass within the pelvis resulting in distortion of local structures. Additionally visualized was a large Morgagni hernia containing a large majority of the small bowel, the transverse colon, a portion of the descending colon, and a portion of the stomach with significant adjacent atelectasis of right lower lung lobe.
Figure 2
Figure 2
Chest X-ray revealing large right Morgagni hernia containing fat and several loops of small bowel. Associated right lung volume loss and atelectasis.
See this image and copyright information in PMC

References

    1. Adam M. P., Ardinger H. H., Pagon R. A., et al. Trichorhinophalangeal Syndrome. GeneReviews. Bethesda, MD, USA: National Center for Biotechnology Information; 1993.
    1. Hall B. D., Langer L. O., Giedion A., et al. Langer-Giedion syndrome. Birth Defects Original Article Series. 1974;10(12):147–164. - PubMed
    1. Michalek P., Doherty J. T., Vesela M. M. Anesthetic management of a child with langer-giedion (TRPS II) syndrome. Journal of Anesthesia. 2009;23(3):456–459. doi: 10.1007/s00540-009-0779-7. - DOI - PubMed
    1. Lu F. L., Hou J. W., Tsai W. S., Teng R. J., Yau K. I., Wang T. R. Tricho-rhino-phalangeal syndrome type II associated with epiglottic aplasia and congenital nephrotic syndrome. Journal of the Formosan Medical Association = Taiwan Yi Zhi. 1997;96(3):217–221. - PubMed
    1. Graybeal L. S., Baum V. C., Durieux M. E. Anaesthetic management of a patient with tricho-rhino-phalangeal syndrome. European Journal of Anaesthesiology. 2005;22(5):400–402. doi: 10.1017/s0265021505270679. - DOI - PubMed

Publication types

LinkOut - more resources