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Rashid F, Chaparala R, Ahmed J, et al. Atypical right diaphragmatic hernia (hernia of Morgagni), spigelian hernia and epigastric hernia in a patient with Williams syndrome: a case report. J Med Case Rep. 2009;3:7doi: 10.1186/1752-1947-3-7.
Rashid, F., Chaparala, R., Ahmed, J., & Iftikhar, S. Y. (2009). Atypical right diaphragmatic hernia (hernia of Morgagni), spigelian hernia and epigastric hernia in a patient with Williams syndrome: a case report. Journal of medical case reports, 37. https://doi.org/10.1186/1752-1947-3-7
Rashid, Farhan, et al. "Atypical right diaphragmatic hernia (hernia of Morgagni), spigelian hernia and epigastric hernia in a patient with Williams syndrome: a case report." Journal of medical case reports vol. 3 (2009): 7. doi: https://doi.org/10.1186/1752-1947-3-7
Rashid F, Chaparala R, Ahmed J, Iftikhar SY. Atypical right diaphragmatic hernia (hernia of Morgagni), spigelian hernia and epigastric hernia in a patient with Williams syndrome: a case report. J Med Case Rep. 2009 Jan 07;3:7. doi: 10.1186/1752-1947-3-7. PMID: 19128471; PMCID: PMC2630954.
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J Med Case Rep. 2009 Jan 07;3:7. doi: 10.1186/1752-1947-3-7.

Atypical right diaphragmatic hernia (hernia of Morgagni), spigelian hernia and epigastric hernia in a patient with Williams syndrome: a case report.

Journal of medical case reports

Farhan Rashid, Ramakrishna Chaparala, Javed Ahmed, Syed Y Iftikhar

Affiliations

  1. Division of GI Surgery, Clinical Sciences Wing, The Medical School, Derby City General Hospital, Uttoxeter Road, Derby, DE22 3DT, UK. [email protected].

PMID: 19128471 PMCID: PMC2630954 DOI: 10.1186/1752-1947-3-7

Abstract

INTRODUCTION: Williams syndrome is rare genetic disorder resulting in neurodevelopmental problems. Hernias of the foramen of Morgagni are rare diaphragmatic hernias and they mostly present on the right side, in the anterior mediastinum. They are usually asymptomatic and are difficult to diagnose, especially in patients with learning disabilities.

CASE PRESENTATION: This 49-year-old woman with Williams syndrome, cognitive impairment and aortic stenosis presented to physicians with right-sided chest pain. She had previously undergone repair of her right spigelian and epigastric hernia. Her abdominal examination was unremarkable. Chest X-ray suggested right-sided diaphragmatic hernia and pleural effusion for which she received treatment. The computed tomography scan showed a diaphragmatic hernia with some collapse/consolidation of the adjacent lung. Furthermore, the patient had aortic stenosis and was high risk for anaesthesia (ASA grade 3). She underwent successful laparoscopic repair of her congenital diaphragmatic hernia leading to a quick and uneventful postoperative recovery.

CONCLUSION: These multiple hernias suggest that patients with Williams syndrome may have some connective tissue disorder which makes them prone to develop hernias especially associated with those parts of the body which may have intracavity pressure variations like the abdomen. Diaphragmatic hernia may be the cause of chest pain in these patients. A computed tomography scan helps in early diagnosis, and laparoscopic repair helps in prevention of further complications, and leads to quick recovery especially in patients with learning disabilities. In the presence of significant comorbidities, a less invasive operative procedure with quick recovery becomes advisable.

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