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A Case Report of Adult Omphalocele with Herniated Liver
*Corresponding author: Srinivas Pai, Department of Surgery, Dr. Chandra Dayanand Sagar Institute of Medical Education and Research, Dayanand Sagar University, Kanakapura, Harohalli, Karnataka, India. srinpai@gmail.com
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Received: ,
Accepted: ,
How to cite this article: Pai S. A Case Report of Adult Omphalocele with Herniated Liver. Karnataka J Surg. 2026;3:1. doi: 10.25259/KJS_21_2025
Abstract
Omphalocele is a congenital defect of the abdominal wall at the umbilicus, causing herniation of the intra-abdominal contents. The small intestine is the usual content of this sac, though rarely the colon, liver, spleen, and kidney may also be seen. It is unusual that an omphalocele presents in adulthood, even though few cases have been reported. Presentation of case A 20-year-old male presented with an omphalocele with the left lobe of the liver as content. He underwent surgery, with caecopexy and appendectomy. The liver was mobilised, and the defect was repaired using retro-muscular placement of a polypropylene mesh. He recovered well, and at follow-up after 18 months, he is doing well. This case report highlights the unusual presentation of neglected omphalocele in adulthood and the possible treatment options.
Keywords
Adult omphalocele
Liver herniation
Neglected omphalocele
Omphalocele
Retrorectus hernioplasty
INTRODUCTION
Omphalocele is a congenital defect at the umbilicus with herniation of intra-abdominal organs. The incidence of Omphalocele is 1 : 4000. There is no gender preponderance.[1] It is unusual that an omphalocele presents in adulthood, even though few cases have been reported.[2,3] Here we present a 20-year-old male with an untreated omphalocele containing liver.
CASE REPORT
A 20-year-old male presented with a neglected congenital anterior abdominal wall swelling. He had it from birth and his parents were told that the child would need surgery, but neglected this advice due to fear and financial constraints. The parents continued to neglect this into his early adulthood, but when he entered college, he consulted us for cosmetic reasons, fearing issues in his future life. On examination, a 10 cm × 12 cm ventral hernia was found with a firm, non-tender content covered by lax skin [Figure 1]. Umbilicus was not seen separately. The swelling was irreducible but became prominent on coughing. No other congenital abnormalities were detected on clinical examination. A contrast-enhanced CT scan of the abdomen revealed a ventral hernia with a defect of 8 cm × 9 cm, with the left lobe of the liver herniating into it [Figure 2] and with transposition of the inferior vena cava. No other abnormality was detected. Laparotomy was performed, and the caecum, ascending colon, and part of the transverse colon were found not to have any peritoneal or omental attachments and hence were freely mobile, though the splenic flexure and the duodenojejunal flexure were intact and anatomically placed. Appendectomy with caeco-colopexy was performed. The liver was mobilised by incising the triangular ligaments and placed inside the abdominal cavity. The retro-rectus/retro-muscular plane was created using blunt and sharp dissection so as to allow closure without undue increase in intra-abdominal pressure. A 15 cm × 15 cm polypropylene mesh was placed in the retro-muscular plane and fixed [Figure 3]. The anterior rectus fascia was then closed as usual. A drain was inserted in the retro-muscular plan to prevent seroma formation. Excess lax skin was excised and then closed. The postoperative period was uneventful. The drain was removed on postoperative day 7 and patient was subsequently discharged. Regular follow-up was carried out till 18 months post-operatively with no evidence of recurrence or any other complications. The patient has returned back to his lifestyle and is thriving well.
- Anterior abdominal hernia with absent umbilicus.
- CT scan of abdomen showing left lobe of liver herniating into the omphalocele. CT: Computed tomography.
- A 15 cm × 15 cm polypropylene mesh placed in the retromuscular plane and fixed.
DISCUSSION
During the intrauterine life, the midgut herniates into the extraembryonic coelom at the site of the umbilical cord, only to return back into the abdominal cavity by the 11th week. Abnormalities occurring during this period can result in the development of an omphalocele.[1] A minor omphalocele is a defect of <5 cm, whereas a giant omphalocele is a large defect (>5 cm) usually occurring because of defective fusion of lateral abdominal plates. Omphalocele is commonly associated with other congenital anomalies, namely cardiac (16%–47%), gastrointestinal (gastro-oesophageal reflux) (40%), genitourinary (40%), central nervous defects, pulmonary hypoplasia, and musculoskeletal anomalies (10%–30%). It is also associated with chromosomal abnormalities and some congenital syndromes.[4] Omphalocele repair may be performed immediately after diagnosis, staged (usually 2 or 3 surgeries are required), or delayed (within weeks).[5] All omphaloceles need repair, and when neglected, the absence of protective fascia and muscles makes the intra-abdominal structures are more vulnerable to trauma; though such cases are not common, they have been reported.[6] Giant omphalocele with hepatic herniation in an adult is treated on the same grounds as any other ventral hernia. Based on the limited literature available in this regard, we can conclude that both open and laparoscopic methods are valid options, sometimes with partial resection of herniated organs. A retro muscular placement of a polypropylene mesh is both effective and economical. It has been proven multiple times to be a safe option for ventral hernias and is the standard of care in many institutions.[7] Laparoscopic repair has recently replaced retro-muscular mesh repair as the preferred treatment option, but retro-muscular repair still remains an effective method of repairing large ventral abdominal wall defects. Larger defects with domain loss are better treated with the component separation technique, either open or laparoscopically.
CONCLUSION
Adult presentation of omphalocele is a rare condition and comes with difficulty in surgical treatment because of late presentation and domain loss domain loss. Timely surgical intervention with appropriate planning and reconstruction—such as retro-muscular mesh placement—can yield excellent outcomes even in delayed cases. This case reinforces the importance of early diagnosis and repair, but also demonstrates that with meticulous surgical technique, favourable results are achievable in adulthood.
Author contributions
SP: Primary care surgeon and prepared the documents for publishing. Literature search and assisted the surgery
Ethical approval
Institutional Review Board approval is not required.
Declaration of patient consent
Patient’s consent not required as patients identity is not disclosed or compromised.
Financial support and sponsorship
Nil.
Conflicts of interest
Dr. Srinivas Pai is on the Editorial Board of the Journal.
Use of artificial intelligence (AI)-assisted technology for manuscript preparation
The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.
REFERENCES
- An Epidemiologic Study of Congenital Malformations of the Anterior Abdominal Wall in More Than Half a Million Consecutive Births. Am J Hum Genet. 1981;33:470-8.
- [Google Scholar]
- Hepatic Omphalocele in an Adult. Scand J Plast Reconstr Surg Hand Surg. 2004;38:236-9.
- [CrossRef] [PubMed] [Google Scholar]
- Liver Developed Entirely Outside the Abdominal Cavity in Adult Omphalocele. Indian J Gastroenterol. 2014;33:581.
- [CrossRef] [PubMed] [Google Scholar]
- Differential Diagnosis of Abdominal Wall Defects - Omphalocele versus Gastroschisis. Chirurgia (Bucur). 2014;109:7-14.
- [Google Scholar]
- Past and Current Surgical Treatment of Giant Omphalocele: Outcome of a Questionnaire Sent to Authors. J Pediatr Surg. 2011;46:482-8.
- [CrossRef] [PubMed] [Google Scholar]
- Perforation of Small Intestine Inside an Internal Omphalocele After Blunt Trauma: Case Report and Review of the Literature. J Trauma. 2000;50:343-7.
- [CrossRef] [PubMed] [Google Scholar]
- Analysing the Benefits of Laparoscopic Hernia Repair Compared to Open Repair: A Meta-Analysis of Observational Studies. J Minim Access Surg. 2012;8:111-7.
- [CrossRef] [PubMed] [PubMed Central] [Google Scholar]