Clin J Gastroenterol (2015) 8:130–133 DOI 10.1007/s12328-015-0571-1

CASE REPORT

Laparoscopic excision of an adult retroperitoneal cystic lymphangioma coexisting with an esophageal hiatus hernia Daisuke Izumi1 • Eiichiro Toyama1 • Hironobu Shigaki1 • Shiro Iwagami1 Yoshifumi Baba1 • Naoko Hayashi1 • Masayuki Watanabe1 • Hideo Baba1



Received: 3 March 2015 / Accepted: 5 May 2015 / Published online: 22 May 2015 Ó Springer Japan 2015

Abstract Lymphangiomas are rare cystic tumors that may be present in the retroperitoneum. Retroperitoneal lymphangiomas account for approximately 1 % of all lymphangiomas. We experienced an adult patient with a retroperitoneal cystic lymphangioma coexisting with an esophageal hiatus hernia. Abdominal ultrasonography showed a cyst in the abdomen of a 76-year-old woman. She was admitted to our clinic because of an enlargement of the cyst and epigastric pain after meals. She had a long history of heartburn after meals and had not undergone any treatment. She had no record of previous illnesses. Computed tomography revealed a single bunch cystic tumor with septations, located from the mediastinum to the retroperitoneal space. The tumor was not enhanced, and there was no solid part. She was diagnosed with a retroperitoneal cyst. Laparoscopic total excision was performed because the cyst was increasing in size and the patient had symptoms. The cyst was successfully removed. Although laparoscopic excision of retroperitoneal cystic lymphangiomas is the treatment of choice, surgical methods should be carefully chosen in selected patients. Keywords Adult retroperitoneal lymphangioma  Esophageal hiatus hernia  Laparoscopic excision

& Hideo Baba [email protected] 1

Department of Gastroenterological Surgery, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Chuo, Kumamoto 860-8556, Japan

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Introduction Cystic lymphangioma is a typical benign tumor in children, thought to be caused by anomalies in the development of the lymphatic system. Most cystic lymphangiomas are located in the neck area, but they have also been reported in the axilla, thoracic cavity, and abdomen [1]. Retroperitoneal cystic lymphangiomas are rare lesions that are often found incidentally during diagnostic procedures or at the time of surgery for unrelated indications [2]. Cystic lymphangiomas located in the retroperitoneum and mediastinum have never been reported. We experienced an adult patient with a retroperitoneal cystic lymphangioma that invaded the mediastinum through an esophageal hiatus hernia.

Case report A cyst was discovered in the abdomen of a 76-year-old woman during abdominal ultrasonography. She had a longterm history of heartburn after meals that had never been treated. Five years earlier, she had been diagnosed with a cyst in her abdomen and had been followed up since at the referring clinic. She was referred to our clinic because the cyst had enlarged and she experienced epigastric pain after meals. She had no record of other previous illnesses. Her height and weight were 135 cm and 34 kg, respectively, and she had a tortoise back. Whole blood count and other blood tests were all within normal ranges except for a slight elevation of C-reactive protein (3.24 mg/dL). Tumor markers were within the normal range (CEA, 19 U/mL; CA19-9, 9.2 U/mL). Computed tomography revealed a single bunch cystic tumor with septations present from the mediastinum to the retroperitoneal space. The tumor was

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not enhanced, and there was no solid part (Fig. 1a). Magnetic resonance imaging showed that the cyst was low intensity on T1W1 and high intensity on T2W1 (Fig. 1b). Upper gastrointestinal endoscopy showed a hernia of an esophageal hiatus with severe reflux esophagitis and Barrett syndrome in the lower esophagus. Biopsy results showed no malignancy, although an iodine spray test revealed a broad achromic lesion caused by esophagitis (Fig. 1c). Endoscopic ultrasonography showed a cystic mass outside the wall on the right side of the esophagus and the lesser curvature of the stomach from the posterior mediastinum to the retroperitoneum. The interior of the cyst was relatively anechoic, except for the septa, and there was no solid lesion (Fig. 1d). The absence of an obvious laminar structure indicated that the mass was not an intestinal duplication. Cytodiagnosis following ultrasonography-guided aspiration showed that the cyst was class I and contained numerous lymphocytes. The patient was diagnosed with a retroperitoneal cyst. Laparoscopic excision was performed because the cyst was increasing in size and the patient had symptoms of

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abdominal distension and heartburn. The patient was placed in a supine position, and four trocars were inserted. Examination of the abdominal cavity showed the cyst, excluding the lesser omentum, on the dorsal side. The border between the cyst and lesser omentum was clear, suggesting that the cyst originated in the retroperitoneum. The cyst was punctured and its contents aspirated with a SAND ballooncatheterÒ (Hakko, Tokyo, Japan), followed by ablation while pulling the cyst (Fig. 2a). A visual field was secured by lifting the lateral side of the liver. Ablation of the head side found the cyst entering the mediastinum through an esophageal hiatus. The cyst was pulled back into the abdominal cavity and extracted without breaking the wall (Fig. 2b). The abdominal part of the esophagus was ablated and taped, and the esophageal hiatus sutured with two stitches. This was followed by cardioplasty using the Nissen procedure. In addition, because inspection of the abdominal cavity revealed an inapparent right femoral hernia, a transabdominal preperitoneal repair procedure was performed. The cyst was collected, and the operation finished. The amount of bleeding was minimal, and the operation took 3 h.

Fig. 1 Diagnostic imaging of our patient. a Computed tomography (coronal view), showing a large cystic mass lying between the chest and abdomen. b Magnetic resonance imaging (axial view) showing a large cystic mass passing through an esophageal hiatus. c Gastrointestinal fiber showing a gastroesophageal hernia and reflux esophagitis. d Endoscopic ultrasonography showing a unilocular cyst adjacent to the esophagus and stomach

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Fig. 2 Operative method. a The cyst was punctured and its contents aspirated with a SAND balloon-catheterÒ (Hakko), followed by ablation while pulling the cyst. b Ablation of the head side showed

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the cyst entering the mediastinum through an esophageal hiatus. The cyst was pulled back into the abdominal cavity, and extracted without breaking the wall

The patient’s postoperative course was uneventful, and she was discharged from hospital on postoperative day 4. She has been well and asymptomatic since.

Discussion

Fig. 3 Diagnostic evaluation. a Confirmation of the passage part of the esophageal hiatus in the extracted specimen. b Histologic examination, showing abundant lymphangiectasis, formation of lymph follicles, lymphatic tissue, and an increase in smooth muscle, resulting in a diagnosis of cystic lymphangioma

The passage part of the esophageal hiatus was confirmed in the extracted specimen (Fig. 3a). Histologic examination showed abundant lymphangiectasis, formation of lymph follicles, lymphatic tissue, and increase in smooth muscle, resulting in a diagnosis of cystic lymphangioma (Fig. 3b).

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Cystic lymphangioma is a congenital hamartoma of the lymphatic system. About 75 % of these lymphangiomas occur in the neck, and are called cystic hygromas. Another 20 % occur in the axillary region, with the rest occurring in the mediastinum, mesentery, internal viscera, and bones. Retroperitoneal cystic lymphangiomas are rare, with an incidence of about 1 % [3]. Most occur in childhood, with 68 % of them occurring in children under the age of 10 years [4]. Cystic lymphangiomas are rare in adults. Their exact etiology is uncertain, although inflammatory and fibrotic processes or a genetic predisposition is likely. Over the past 10 years, there have been case reports on 26 adults with retroperitoneal lymphangioma, six in male individuals and 20 in female individuals [1–3, 5–16]. The mean age was 44.8 years, although, as in our patient, these lesions can occur in older individuals. Tumor size is usually 5–10 cm, but some are as large as 20 cm. The cooccurrence of esophageal hiatus hernia in our patient introduced complications, in that it allowed the cystic lymphangioma that originated in the retroperitoneum to expand into the mediastinum. The major axis of this tumor was 18 cm long. To our knowledge, there have been no previous reports of a lymphangioma expanding from the retroperitoneum to the mediastinum. Although most lymphangiomas are detected incidentally, as most have no symptoms, assessment of the 26 previously described patients with retroperitoneal lesions

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showed that 17 (65 %) had abdominal pain, 3 (12 %) had abdominal distension, and 2 (8 %) had palpable tumors. In addition, one patient experienced acute abdominal pain caused by the rupture of a cyst. Though our patient experienced epigastric pain after meals, none of her symptoms were caused by the cyst, because she had esophageal hiatus hernia and reflux esophagitis. Because lymphangiomas have a tendency to invade surrounding tissue, surgical excision is the treatment of choice [17]. Recurrence is rare following total excision. Of the 26 previously described patients, 18 (69 %) underwent laparotomies [1–3, 8–12, 14–16], 5 (19 %) underwent laparoscopic operations [2, 15, 16], and 1 (4 %) was observed [13], with treatment of the other 2 (8 %) being unclear [6, 7]. When the boundary and surroundings can be clearly distinguished, allowing excision without wound extension or aspiration of the cyst content, laparoscopic surgery is stress free and beneficial for the patient. Preoperative diagnostic imaging of our patient showed the boundary clearly, and total excision was possible without rupture of the wall of the cyst using paracentesis and aspiration. Another advantage of laparoscopic surgery was the ability to repair the esophageal hiatus hernia and a right femoral hernia at the same time. In summary, this report describes an adult with a retroperitoneal cystic lymphangioma coexisting with an esophageal hiatus hernia. The cystic lymphangioma was totally excised by laparoscopic surgery. Although the prognosis of patients with lymphangioma is very good, reports of recurrence caused by incomplete excision and death caused by complications require that the surgical method be selected very carefully, based on preoperative diagnostic imaging. Disclosures Conflict of Interest: Daisuke Izumi, Eiichiro Toyama, Hironobu Shigaki, Shiro Iwagami, Yoshifumi Baba, Naoko Hayashi, Masayuki Watanabe, and Hideo Baba declare that they have no conflict of interest. Human/Animal Rights: All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2008(5). Informed Consent: Informed consent was obtained from all patients for being included in the study.

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Laparoscopic excision of an adult retroperitoneal cystic lymphangioma coexisting with an esophageal hiatus hernia.

Lymphangiomas are rare cystic tumors that may be present in the retroperitoneum. Retroperitoneal lymphangiomas account for approximately 1% of all lym...
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