Peri-Hilar Cystic Lymphangioma Mimicking a Biliary Cystic Lesion Causing Biliary Obstruction: A Case Report and Literature Review.

BACKGROUND Lymphangiomas are rare and benign malformations of the lymphatic system. The presentation of intra-abdominal lymphangiomas, especially from within the hepatoduodenal ligament, is rare in the adult population. In this report, we examine a lymphangioma within the hepatoduodenal ligament resulting in biliary obstruction. CASE REPORT A 62-year-old man with surgical history of cholecystectomy presented to the hepatobiliary clinic for a peri-hilar cystic lesion identified on surveillance magnetic resonance imaging (MRI). The patient's MRI revealed a 5.5-cm cystic lesion at the peri-hilar region, likely arising from the biliary tree, which had been increasing in size and causing biliary dilatation. The patient underwent an endoscopic ultrasound, showing a 4.3×2.2 cm cystic structure likley arising from the cystic duct stump with internal septation. An endoscopic retrograde cholangiopancreatography (ERCP) was performed and demonstrated no communication between the biliary tree and the cystic lesion. Given the uncertain etiology of the lesion and its obstructive nature, the patient was moved to the operating room for a complete excision. A well-encapsulated cystic lesion was identified between the cystic duct and the common hepatic duct, which did not communicate with the biliary tree. Pathology confirmed the diagnosis of lymphangioma with features of vascular channel proliferation in the background of fibrotic stroma and lymphoid aggregates. The vascular channel proliferation demonstrated positive immunohistochemical staining for D2-40. At 3-year follow-up, there was no evidence of post-resection recurrence. CONCLUSIONS This case represents an acquired lymphangioma occurring as a sequela of cholecystectomy, likely caused by interruption of the lymphatic drainage system secondary to surgical manipulation.

Lymphangiomatosis presented with melena and chylous ascites: A case report.

INTRODUCTION: Lymphangioma, a rare benign tumor of the lymphatic system, is called lymphangiomatosis when it involves >1 organ, which is more rarely complicated with thoracic obstruction, the relationship between them remains unclear. With the development of enteroscopy, clinicians know more about small intestinal lymphangioma and attempt to treat it through enteroscopic injection sclerotherapy(EIS). PATIENT CONCERNS: A 59-year-old male firstly manifested with gastrointestinal bleeding after a gastric perforation, who was diagnosed with lymphangiomatosis by balloon-assisted enteroscopy and abdomen CT showing >1 organ with multiple cysts besides the small intestine. The patient received an EIS, then the melena disappeared. Surprisingly he came back because of refractory ascites confirmed to be chylous by chemical tests 7 months later. DIAGNOSIS: Lymphangiography could not determine the location of lymphatic leakage, Ultrasonography showed stenosis of the left cervical part of the thoracic duct. INTERVENTION: On the condition that medical treatment is ineffective, thoracic duct exploration and lysis of fibrous adhesion were performed. OUTCOMES: Ascites significantly reduced at last. LESSONS: Lymphangiomatosis is the malformation of the lymphatic system involving multiple organs, it has a possibility to be associated with thoracic obstruction. Capsule endoscopy and enteroscopy are effective methods to diagnose small intestinal lymphangioma, and EIS is an effective therapy.

Lymphangioma of small bowel in adults: A rare cause of abdominal symptoms.

BACKGROUND: Lymphangioma is unusual malformation of the lymphatic system and rarely occurred in adults below diaphragm. Lymphangioma in small intestine sometimes caused unspecific symptoms and even bleeding, however, this area was most difficult for endoscopic examination and worth investigation. METHODS: From 1999 to 2019, we retrospectively collected eighteen adult patients with the pathological diagnosis of lymphangioma from duodenum to terminal ileum. The clinical symptoms, origin site, predisposing factor, pre-operative image surveillance, surgical intervention, histopathological findings, follow-up period, and outcome were reviewed and analyzed. RESULTS: The mean age of diagnosis was 50.11 years old with female predominance. The most common symptoms were abdominal pain (77.78%), palpable mass (27.78%) and intestinal bleeding (16.67%). With the different gross and microscopic findings, the adult intestinal lymphangioma could be classified as "simple", "polyposis", and "cystic cavernous". CONCLUSION: Intestinal lymphangioma in adults is a rare cause of abdominal discomfort but should be listed in differential diagnosis during daily practice. The morphology and classification may be different from pediatric group, and more large-scale studies are need for thorough investigation.

Rare finding of duodenal lymphangioma in a patient with Helicobacter pylori associated gastritis: A case report.

RATIONALE: Lymphangiomas are lymphatic malformations most commonly found in the head, neck and axilla. Their incidence in gastrointestinal tract, especially duodenum is very rare. They are mostly congenital and are usually seen in children. Owing to their asymptomatic nature, these tumors are often diagnosed incidentally. Surgical resection is the definite treatment. PATIENT CONCERNS: We represent a case of a 30-year-old male with duodenal lymphangioma with presentation of intermittent dyspepsia for 1 year, partially relieved by a proton pump inhibitor. DIAGNOSES: Upon physical examination, there was mild tenderness observed in the epigastrium. The rest of the physical examination was unremarkable. His complete blood count report was unremarkable. Upon a negative stool for Helicobacter pylori antigen test, the patient underwent an esophagogastroduodenoscopy which revealed H pylori gastritis and a duodenal lymphangioma. INTERVENTIONS: Patient was put on triple therapy (clarithromycin, amoxicillin and omeprazole) for 14 days and his symptoms improved. The lymphangioma was not resected owing to small size. OUTCOMES: Patient was followed till 1 year and his symptoms had improved. LESSONS: The case describes a correlation between H pylori gastritis and a duodenal lymphangioma. There is likely to be an association between the two and therefore, further studies are required to find out any relationship that may exist between the 2 conditions.

A large pericardial cystic lymphangioma presenting as acute-onset respiratory distress in a child: a case report.

BACKGROUND: Lymphangiomas are rare benign malformations of the lymphatics that occur due to blockage of the lymphatic system during fetal development. They commonly occur in the neck and axilla, while involvement of the pericardium is rare. We report herein the case of a 16-month-old Sri Lankan child with a large pericardial cystic lymphangioma presenting with sudden-onset shortness of breath. CASE PRESENTATION: A 16-month-old Sri Lankan boy presented with sudden-onset dyspnea for 1-day duration following a febrile illness that lasted 2 days. On examination, he was afebrile and had subcostal, intercostal, and suprasternal recessions, with a respiratory rate of 50 breaths per minute. He had a loud expiratory grunt. The chest expansion was reduced on the right side, which was dull to percussion. Auscultation revealed a marked reduction of air entry over the right lower and mid zones. Chest X-ray showed a well-demarcated opacity involving the lower and mid zones of the right hemithorax associated with a tracheal shift to the opposite side. Ultrasound scan of the chest revealed fluid-filled right hemithorax suggesting a septate pleural effusion. A contrast-enhanced computed tomography scan of the thorax showed a large multiloculated extrapulmonary cystic lesion involving the right hemithorax with a mediastinal shift towards the left side associated with displacement of the right-side mediastinal structures. He underwent mini-thoracotomy and surgical excision of the cyst. A large cyst originating from the pericardium was observed and excised during surgery. Histological examination revealed a lesion composed of cysts devoid of a lining epithelium but separated by connective tissue, mature adipose tissue, and lymphoid aggregates. The child showed complete recovery postoperatively with full expansion of the ipsilateral lung. CONCLUSION: We report the case of a patient with cystic lymphangioma who was perfectly well and asymptomatic until 16 months of age. This case report presents the very rare occurrence of a large cystic lymphangioma originating from the pericardium. It highlights the importance of considering rare possibilities and performing prompt imaging in situations of diagnostic uncertainty to arrive at an accurate diagnosis that can be lifesaving.

Recurrent haemoptysis: a rare diagnosis of kaposiform lymphangiomatosis and review of literature.

Kaposiform lymphangiomatosis (KLA) is a rare clinicopathological entity among lymphatic anomalies. The main involved sites are the mediastinum and the lungs but the disease can also affect multiple extrathoracic organs. Little is known about the pathophysiology, the natural history, the treatment response and the long-term outcome of this disorder. KLA is typically diagnosed in childhood. We present here the case of an adult man with 13 years recurrent episodes of haemoptysis who was finally found to suffer from KLA. Following this, we present a comprehensive review of the literature.

Right Ventricular Epicardium-Originated Cardiac Lymphangioma: A Rare Tumor and Rare Origin.

Tumors of the heart are uncommon, and lymphangioma is one of the rarest forms of this pathologic process. We describe an asymptomatic patient whose tumor was detected incidentally during coronary artery bypass operation. We detected a spongiform thin-layered mass covering about one-third of the right ventricular epicardium. Excisional biopsy was performed, and the main operation continued. Cardiac lymphangiomas are rare tumors of the heart and mostly benign. In general, surgical excision is performed when it is diagnosed. We detected the lesion incidentally; for this reason, we took only an excisional biopsy specimen. After exact pathologic diagnosis, the patient was observed with routine medical follow-up.

Disseminated lymphangiomas as a cutaneous manifestation of Noonan syndrome.

Lymphangioma is a known, but rare manifestation of Noonan syndrome. We present the case of disseminated and circumscribed cutaneous lymphangiomas in the context of Noonan syndrome. Oral rapamycin is a promising treatment in these extensive and morbidity-causing cases.

A Long-term Follow-up of Mandibular Deviation Caused by Congenital Cervical Lymphangioma Treated with an Orthodontic Approach.

AIM: To report a treatment case of mandibular deviation caused by congenital cervical lymphangioma with traditional orthodontic techniques, following-up by 10-year retention. BACKGROUND: Lymphangiomas, developmental anomalies, can induce various disturbances of swallowing, mastication, speech, breathing, and skeletal deformities as well as psychological stress and anxiety for the patient and their family. Lymphangiomas are benign with virtually no possibility of turning into a malignant lesion, so clinical management aims to treat the patient functionally. CASE DESCRIPTION: A girl, aged 6 years and 4 months, complained about facial asymmetry and anterior crossbite caused by congenital cervical lymphangioma. Her facial profile was the straight type with an adequate lip position. Anterior and right-side posterior crossbites were observed. On the frontal cephalogram, the menton shifted 3.0 mm to the right. A functional appliance with an expander was placed to correct her dental midline deviation and posterior crossbite. After 2-year treatment, the anterior and right-side posterior crossbites were improved. Multibracket treatment began after the growth spurt. After 44-month active treatment, a functional occlusion, including a Class I molar relationship with a proper interincisal relationship, was achieved. A functional occlusion was maintained during a 10-year retention period, while a mandibular downward growth was observed through the retention period. CONCLUSION: Conventional orthodontic techniques enable functional and stable occlusion even in patients with mandibular deviation caused by congenital cervical lymphangioma, although only using early orthodontic management by itself may have some limitations. CLINICAL SIGNIFICANCE: The hybrid technique combining functional appliance and intermaxillary elastics proves to be an effective therapy for correcting occlusal cant and mandibular deviation caused by cervical lymphangioma.

Laryngeal lymphangioma as a cause of respiratory distress in an adult with Down's syndrome: an extremely rare presentation.

A 32-year-old man with Down's syndrome was referred to the ear, nose and throat (ENT) department in view of failed attempts at extubation, and subsequently, at decannulation of tracheotomy tube. He had previously required ventilatory support and had history of intubation for 1 week. A flexible fibre-optic laryngoscopy showed a smooth mass covering the laryngeal inlet which moved with respiration. Direct laryngoscopy under general anaesthesia revealed a smooth mucosa covered fleshy mass arising from the left aryepiglottic fold and arytenoid, obstructing the laryngeal inlet. The mass was removed using controlled plasma ablation, and histopathological examination of the same was consistent with lymphangioma. Endoscopic examinations during the regular follow-up visits revealed well-healed supraglottic area with adequate glottic chink and the patient could be successfully decannulated.

[Lymphangiomatosis: Rare Cause of Pleural Effusion]. / Lymphangiomatose − seltene Ursache eines Pleuraergusses.

A 33-year-old man presented to us with slowly progressive dyspnea. At the physical examination we found pleural effusion on the right side, lymph edema of the left lower limb and foot and blotchy map-like skin changes. The pleural puncture revealed a chylous effusion. In whole body MRI we saw the pleural effusion on the right, an increase in lymph vessels in the pelvis and paraaortic, and prominent chylous vessels. There was no bone involvement. The abdominal thoracic duct was enlarged by up to 3 mm. We diagnosed a general lymphatic anomaly (GLA) - lymphangiomatosis. GLA is a rare disease of unknown origin with dysplasia of the lymphatic system. The thoracic manifestation is often the development of chylous pleural effusions. However, many other organs can also be involved. Therapy includes in most cases symptomatic relief and the attempt to slow the progression of the disease.

Quilotórax bilateral y ascitis quilosa como consecuencia de la rotura espontánea de un linfangioma retroperitoneal / Bilateral chylothorax and chylous ascites resulting from the spontaneous rupture of a retroperitoneal lymphangioma

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