Unusual Presentation of Lymphangioma Circumscriptum of the Vulva: In Association With Crohn Disease.

ABSTRACT: Lymphangioma circumscriptum (LC) is a rare benign condition, with marked dilation of surface lymphatic vessels in the deep and subcutaneous layers. Vulvar LC can become a highly disabling condition with vulvar discomfort, itching, burning and lymph seeping being the dominant symptoms. Biopsy is mandatory for the diagnosis. There is no consensus on the standard treatment for vulvar LC and recurrence is frequent. In complex cases with wide disease location, combination of different treatment options, such as abrasive methods and surgery, may lead to the best clinical and aesthetical result, with extended disease-free periods. We present a patient with a long history of Crohn disease with multiple pelvic surgeries who developed an extensive vulvar LC.

Misdiagnosis of scrotal and retroperitoneal lymphangioma in children.

BACKGROUND: Scrotal and retroperitoneal lymphangioma (SRL) in children is relatively rare and its clinical symptoms are usually difficult to distinguish from other conditions such as hydrocele and incarcerated inguinal oblique hernia. This study aimed to explore the clinical diagnosis and treatment of abdominal scrotal lymphangioma in children, and thus, to increase our understandings of this disease in clinical practice. METHOD: This study enrolled nine boys, aged 1-10, who were admitted to Shanghai Children's Hospital from January 2019 to December 2020 and who were finally confirmed with lymphangioma in the inguinal area. The clinical manifestations, diagnosis, and treatment of these children were analyzed retrospectively. The length of diagnostic process ranged from 3 weeks to 20 months. We also reviewed other cases of initially misdiagnosed cases of SRL in English publications from 2000 to 2022. RESULTS: The nine cases were misdiagnosed as hydrocele, hematoma, or inguinal hernia. Three patients received intracystic injection of bleomycin, three underwent laparoscopic mass resection, and three underwent resection of the inguinal lymphangioma under direct vision. Postoperative pathological analysis of the surgical specimens confirmed the diagnosis of benign cystic lesions and lymphangioma. Meanwhile, among the 14 cases of SRL in literature review, eight were misdiagnosed. Six were initially diagnosed as hydrocele, one as inguinal oblique hernia, and one as testicular tumor, all of which underwent ultrasonography scans. All cases were confirmed as lymphangioma after pathological examination. CONCLUSION: The non-specific clinical manifestations may contribute to the misdiagnosis of scrotal masses in children. A detailed and accurate medical history, careful physical examination, and imaging findings are important factors contributing to the preoperative differential diagnosis of scrotal lumps in children, but the final diagnosis is based on pathological examination.

Neonatal alveolar lymphangioma: A rare benign oral disease case report and literature review.

BACKGROUND: Neonatal alveolar lymphangioma (NAL) is a rare benign condition most often reported in the oral cavity of neonates of African descent. CASE DESCRIPTION: The authors present a case report of bilateral NAL, including follow-up images at 6 months and 17 months. The clinical differential diagnosis includes dental lamina cyst, hemangioma, congenital epulis of the neonate, and melanotic neuroectodermal tumor of infancy. Key differences to assist in making the distinction are described. PRACTICAL IMPLICATIONS: The distinguishing characteristics of NAL make a clinical diagnosis possible. Accurate clinical recognition of NAL is essential because biopsy is not typically indicated. Because this is a clinical diagnosis, follow-up for 1 year or until complete resolution; biopsy is suggested if clinically indicated for residual or recurrent lesion.

Skull Base Fracture or Abuse Suspicion Based on Intraorbital Lymphangioma Secondary to Periorbital Hematoma.

Intraorbital lymphangiomas are among the orbital tumors that can cause sudden eye protrusion in children. In children with periorbital hematoma (panda eye sign), a skull fracture or abuse is likely first considered as the differential diagnosis. A 7-month-old boy presented to the ophthalmologist with complaints of swelling of the right upper eyelid, subconjunctival hemorrhage on the right ear side, and periorbital subcutaneous hemorrhage, which had appeared since the morning of the day before the visit. The eyeball did not protrude. Based on the interview and clinical findings, right eyeball contusion was suspected. The patient was then followed up for observation. Later, during the physical examination, the abovementioned symptoms were noted. Hence, the patient was admitted for a close examination based on the suspicion of skull base fracture and abuse. Contrast-enhanced magnetic resonance imaging (MRI) after admission revealed a multifocal cystic structure within the right intraorbital muscular cone. Thus, he was diagnosed with right intraorbital lymphangioma. Intraorbital lymphangioma may not show ocular protrusion, and this disease should be considered in cases where abuse is suspected, considering the periorbital subcutaneous hemorrhage.

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.

Preputial lymphangioma in a stallion: First report.

This short communication describes a case of a 30-year-old stallion with a mass on the base of the penis causing paraphimosis. The patient was submitted to anti-inflammatory and diuretic therapy with no signs of improvement, so 16 days after the lesion was detected, the animal was euthanized. Necropsy was performed, and histopathological assessment of the lesion was conducted. The mass was composed primarily of channels and cavernous structures, lined by elongated cells of vascular origin, located in the preputium. The lesion was diagnosed as a preputial lymphangioma. To the authors' best knowledge, the anatomical location of this neoplasm (which is rare in veterinary medicine) has not been previously reported.

Retroperitoneal lymphangioma as the final diagnosis of a middle-aged woman with abdominal pain: a case report.

BACKGROUND: Lymphangiomas are lesions attributed to congenital malformations of the lymphatic system, or acquired chronic obstruction of the lymphatic network due to trauma, radiation, surgical manipulation, inflammation, or infection. Overall, lymaphangiomas are rare, and particularly, retroperitoneal lymphangiomas are far more uncommon per reported cases. CASE PRESENTATION: A 49-year-old Iranian woman presented with a progressive abdominal pain since approximately 1 month before admission. She was found to have a retroperitoneal lymphangioma after a precise radiological and surgical workup. CONCLUSION: Retroperitoneal lymphangiomas are rare lesions, sometimes indistinguishable from malignant lesions originating from pancreas and adjacent organs. Complete surgical removal and histologic evaluation of the lesion is the gold standard of treatment and diagnosis.

[Isolated splenic lymphangioma]. / Laparoskopicheskaya rezektsiya pri izolirovannoi limfangiome selezenki.

Splenic lymphangioma is a rare malformation of splenic lymphatic channels characterized by cysts resulting from increased number of enlarged thin-walled lymphatic vessels. In our case, there were no clinical manifestations. Lymphangioma was congenital and diagnosed by ultrasound as an accidental finding. Surgery is the only method of radical treatment of splenic lymphangioma. We describe an extremely rare case of pediatric isolated splenic lymphangioma and laparoscopic resection of spleen as the most advantageous variant of surgical treatment.

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.