Abstract:Esophageal duplication cysts (EDCs) are rare developmental anomalies. They may occur anywhere along the esophagus with the predominant location in the thoracic segment. Presently, most are diagnosed prenatally or in early childhood. The prevalence of EDCs is estimated at 1 in 8200 live births. Usually, cysts are asymptomatic in the neonatal period, but they may cause respiratory distress or feeding difficulties depending on the size and location of the lesion.This report presents a female neonate with a cyst l… Show more
“…Such non-communicating, isolated EDCs have a wall with gastrointestinal epithelium and a well-developed coating of smooth muscle, similar to that seen in regular EDC, but without an anatomical association with the alimentary tract. This type of tumor has been reported in various locations, including the tongue [ 7 ], pleural space [ 8 ], liver [ 9 ], pancreas [ 10 ], biliary tree [ 11 ] and retroperitoneum (as is described in the presented case). IEDC in adults is very rare and the consensus on the diagnosis, including the characteristic feature of malignancy, has not been established.…”
Background
Adult cases of retroperitoneal isolated enteric duplication cyst (IEDC) are rare, with only 17 case reports in the relevant literature. We herein present a case, which was characterized by changes in intra-cystic density on computed tomography (CT), which was safely resected by laparoscopic surgery.
Case presentation
The patient was a 60-year-old male who received abdominal CT to investigate the cause of increased serum CA19-9 levels. CT revealed a unilocular cystic mass located in the lower right retroperitoneum. The size increased from 5 to 10 cm in three and a half years and the CT value decreased from 101 Hounsfield Units (HU) to 20 HU. We performed laparoscopic surgical resection, because the possibility that the enlargement of the lesion represented malignant transformation could not be denied. The large cystic mass firmly adhered to the appendix and its mesentery via the retroperitoneum, the appendix was resected en bloc with the cystic lesion. Microscopically, it had no communication with the appendix, and had an intestinal wall structure of muscularis mucosae and muscularis propria. The final pathological diagnosis was IEDC in the retroperitoneal space. There was no histological evidence of malignancy.
Conclusion
When we encounter a retroperitoneal cystic lesion, we should consider the possibility of malignancy to determine the treatment strategy and perform a careful operation without breaking the cyst wall, irrespective of the preoperative diagnosis.
“…Such non-communicating, isolated EDCs have a wall with gastrointestinal epithelium and a well-developed coating of smooth muscle, similar to that seen in regular EDC, but without an anatomical association with the alimentary tract. This type of tumor has been reported in various locations, including the tongue [ 7 ], pleural space [ 8 ], liver [ 9 ], pancreas [ 10 ], biliary tree [ 11 ] and retroperitoneum (as is described in the presented case). IEDC in adults is very rare and the consensus on the diagnosis, including the characteristic feature of malignancy, has not been established.…”
Background
Adult cases of retroperitoneal isolated enteric duplication cyst (IEDC) are rare, with only 17 case reports in the relevant literature. We herein present a case, which was characterized by changes in intra-cystic density on computed tomography (CT), which was safely resected by laparoscopic surgery.
Case presentation
The patient was a 60-year-old male who received abdominal CT to investigate the cause of increased serum CA19-9 levels. CT revealed a unilocular cystic mass located in the lower right retroperitoneum. The size increased from 5 to 10 cm in three and a half years and the CT value decreased from 101 Hounsfield Units (HU) to 20 HU. We performed laparoscopic surgical resection, because the possibility that the enlargement of the lesion represented malignant transformation could not be denied. The large cystic mass firmly adhered to the appendix and its mesentery via the retroperitoneum, the appendix was resected en bloc with the cystic lesion. Microscopically, it had no communication with the appendix, and had an intestinal wall structure of muscularis mucosae and muscularis propria. The final pathological diagnosis was IEDC in the retroperitoneal space. There was no histological evidence of malignancy.
Conclusion
When we encounter a retroperitoneal cystic lesion, we should consider the possibility of malignancy to determine the treatment strategy and perform a careful operation without breaking the cyst wall, irrespective of the preoperative diagnosis.
“…However, this test still has a notable rate of false positive and false negative results. In some cases, prenatal ultrasound can be used to assist diagnosis[ 5 , 6 ]. Clinically, it is difficult to distinguish intestinal duplication from mesenteric cyst and Meckel’s diverticulum when the symptoms are mainly acute intestinal obstruction or bleeding.…”
BACKGROUND
Alimentary tract duplication is a rare congenital disease that may occur in any part of the alimentary tract, whereas thoracoabdominal duplications only account for approximately 2% of all alimentary tract duplication cases. Many symptoms, including abdominal pain, abdominal distension, vomiting, gastrointestinal bleeding, chest discomfort, chest pain, and shortness of breath, may be present in patients with abdominal or thoracic duplication.
CASE SUMMARY
A 10-mo-old infant, with a free previous medical history, was admitted to our hospital with melena three times in 6 d. Enhanced magnetic resonance imaging of the thoracic vertebrae revealed multiple cervicothoracic vertebral deformities, spina bifida, meningomyelocele towards the posterior mediastinum, and possible concurrent infection. Upper gastroenterography indicated intestinal malrotation. A laparoscopic abdominal examination was performed, and the operation was intraoperatively converted to laparotomy. This case was diagnosed intraoperatively as thoracoabdominal intestinal duplication. The intestinal duplications in the abdomen and large part of the thorax were excised. The results of postoperative pathological examination confirmed that this case was alimentary tract duplication and that part of the duplication contained gastric mucosa. The infant recovered well and was discharged 1 wk after the surgery. A follow-up computed tomography scan 3 mo after operation showed myelomeningocele while the posterior mediastinal cyst was significantly reduced.
CONCLUSION
Thoracoabdominal duplication should be considered if a child has suspected abdominal intestinal duplication with hematochezia as an onset symptom.
Enteric duplication cysts (EDCs) are rare congenital malformations formed during the embryonic development of the digestive tract. They are usually detected prenatally or in the first years of life. The size, location, type, mucosal pattern and presence of complications produce a varied clinical presentation and different imaging findings. Ultrasonography (US) is the most used imaging method for diagnosis. Magnetic resonance (MR) and computed tomography (CT) are less frequently used, but can be helpful in cases of difficult surgical approach. Conservative surgery is the treatment of choice. Pathology confirms the intestinal origin of the cyst, showing a layer of smooth muscle in the wall and an epithelial lining inside, resembling some part of the gastrointestinal tract (GT). We review the different forms of presentation of the EDCs, showing both the typical and atypical imaging findings with the different imaging techniques. We correlate the imaging findings with the surgical results and the final pathological features.Teaching Points• EDCs are rare congenital anomalies from the digestive tract with uncertain pathogenesis.• More frequently, diagnosis is antenatal, with most EDCs occurring in the distal ileum.• Ultrasonography is the method of choice for diagnosis of EDCs.• Complicated EDCs can show atypical imaging findings.• Surgery is necessary to avoid complications.
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