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Hirschsprung’s Disease (HD) - Congenital Megacolon Child Imaging with Low Dose Technology

Dr. Tesfaye Kebede, MD, SCR, SSBI 



|

Wudassie Diagnostic Center, Addis Ababa, Ethiopia

|2020-01-02
Siemens-healthineers-top-axial-plane-study

Top, Axial plane study image, Axial arterial and delayed CT images. Pediatric 70 kV with 110 mAs imaging protocol.

Epidemiology

Hirschsprung’s Disease (HD), also known as congenital aganglionic megacolon, is an anomaly characterized by an absence of ganglion cells in the myenteric and submucosal plexuses in a variable length of bowel segment, especially in boys. Hirschsprung disease affects approximately 1:5000-8000 live births. In short segment disease, there is a significant predilection for males which reduces with increasing length of involvement. Interestingly, it is almost never seen in premature infants.

Siemens-Healthineers-coronal-images

Coronal images consecutively plain, arterial and delayed phases along with vrt hollo preset. Pediatric 70 kV with 110 mAs imaging protocol.

History

A 6-year-old male patient, presented with acute abdominal pain, swelling of the abdomen, vomiting and constipation. He was referred to the radiology department with a clinical suspicion of bowel obstruction. Whole abdomen low dose contrast CT was performed with dedicated child abdomen protocol.

Siemens-healthineers-sagittal-images

Sagittal images consecutively plain, arterial and delayed phases along with vrt hollo vascular preset. Pediatric 70 kV with 110 mAs imaging protocol.

Diagnosis

Whole abdomen low dose multi-phase CT study (total DLP 83 and effective dose 0.5 milli gray) performed on the Siemens Healthineers SOMATOM go.Top 128-slice

CT scanner, showed a large bowel obstruction R/O Hirschsprung disease. Liver is normal in size and no focal lesions are seen. The large bowel loops are dilated markedly up to the rectosigmoid junction and filled with feces. There is no evidence of mass lesion. Both the kidneys, pancreas and spleen are normal. Urinary bladder is distended and normal in wall thickness.


Conclusion

Low-dose MDCT has a good role in localizing the site of TZ of HD in infants. Low dose technologies like tin filter and low kV imaging facilitate the achievement of the ALARA (as low as reasonably achievable) principle especially in pediatric CT imaging. Iterative reconstruction algorithm SAFIRE helps to reduce noise from low dose imaging and helps to visualize superior image quality in such low dose imaging. Such low dose protocols are important especially in developing countries like Ethiopia where a significant proportion of the population is in the pediatric age group (around 13 million people are under the age of 5 which constitute about 16% of the total population)

Examination Protocol

Estimated by applying a conversion factor of 0.020

        Contact

        <p>Dr. Tesfaye Kebede, MD, SCR, SSBI</p>

        Dr. Tesfaye Kebede, MD, SCR, SSBI

        Department of Radiology

        Wudassie Diagnostic Center, Ethiopia

        References

        Peña A, Hong AR. Hirschsprung Disease. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:345.

        M.L. Proctor, J. Traubici, J.C. Langer, et al.Correlation between radiographic transition zone and level of aganglionosis in Hirschsprung’s disease: implications for surgical approach

        T.L. SlovisConference on the ALARA (as low as reasonably achievable) concept in pediatric CT: intelligent dose reduction

        Pediatr Radiol, 32 (4) (2002), pp. 217-313

        Roy CC, Silverman A, Alagille D. Congenital aganglionic megacolon. Pediatric Clinical Gastroenterology. 4th ed. St. Louis, MO: 7 Mosby; 1995. p. 503-15.

        H.J. Kim, A.Y. Kim, C.W. Lee, et al.Hirschsprung disease and hypoganglionosis in adults: radiologic findings and differentiation

        Ralls MW, Coran AG, Teitelbaum DH. Reoperative surgery for Hirschsprung disease. Semin Pediatr Surg. 2012;21:354-363. https://www.ncbi.nlm.nih.gov/pubmed/22985841

        University of California San Francisco Pediatric Surgery. Hirschsprung’s Disease. Available at: http://pedsurg.ucsf.edu/conditions–procedures/hirschsprungs-disease.aspx Accessed September 22, 2016.

        Miyamoto M, Egami K, Maeda S, Ohkawa K, Tanaka N, Uchida E et al. Hirschsprung’s disease in adults: report of a case and review of the literature. J Nippon Med Sch 2005;72:113-20.

        Cincinnati Children’s Hospital Medical Center. Hirschsprung Disease. June 2015. Available at: https://www.cincinnatichildrens.org/health/h/hirschsprungAccessed September 22, 2016.

        Heanue TA, Pachnis V. Enteric nervous system development and Hirschsprung’s disease: advances in genetic and stem cell studies. Nat Rev Neurosci. 2007;8:466–79. [PubMed] [Google Scholar]

        Whitehouse FrKJ. Myenteric plexus in congenital megacolon: Study of eleven cases. Archives of Internal Medicine. 1948;82:75–111. [PubMed] [Google Scholar]

        Hirschsprung Disease”. NORD (National Organization for Rare Disorders). 2017. Retrieved 14 December 2017.

        Heanue TA, Pachnis V. Enteric nervous system development and Hirschsprung’s disease: advances in genetic and stem cell studies. Nat Rev Neurosci. 2007 Jun. 8(6):466-79. [Medline].

        Whitehouse FR, Kernohan JW. Myenteric plexus in congenital megacolon; study of 11 cases. Arch Intern Med (Chic). 1948 Jul. 82(1):75-111. [Medline].