Objective: To investigate the clinicopathological features, clinical manifestations and different diagnosis of patients with complicated lymphatic anomaly. Methods: The clinical and pathologic data of four patients with complicated lymphatic anomaly diagnosed and treated in Peking Union Medical College Hospital from January 2000 to December 2021 were collected and analyzed. Results: One Gorham-Stout disease case and three generalized lymphatic anomaly cases were included in this cohort. Patients' ages ranged from 7 to 32 years. There were three males and one female. The positions of biopsy included three bone biopsy and one bronchus biopsy. Microscopically, all cases showed diffuse enlarged lymphatic channels. At the same time, osteogenesis was obvious in Gorham-Stout disease case. Radiologically, cortical loss was seen in Gorham-Stout disease, and lytic bone confined to the medullary cavity presented in generalized lymphatic anomaly. The three generalized lymphatic anomaly cases also had coagulopathy, and two had effusion. Conclusions: The histologic feature of complicated lymphatic anomaly was diffuse lymphatic malformation, and the diagnosis depends on clinical and pathologic information. The treatment and prognosis of these diseases are different, and therefore it is necessary to understand their clinical and pathologic features and make the correct diagnosis.
Male ; Humans ; Female ; Child ; Adolescent ; Young Adult ; Adult ; Osteolysis, Essential/pathology* ; Lymphatic Abnormalities/surgery* ; Bone and Bones/pathology* ; Diagnosis, Differential ; Prognosis

The cisterna chyli, a dilated lymphatic sac in the retrocrural space, is usually located to the right of the aorta. We report a case of a left-sided cisterna chyli, which was incidentally detected on the radiologic examinations of a preoperative workup for cholangiocarcinoma. Computed tomography (CT) and magnetic resonance (MR) images revealed a cisterna chyli measuring 2.5 cm in length in the left retrocrural space. The dilated lumbar lymphatics joined with the cisterna chyli, which was continuous with the left-sided thoracic duct. To the best of our knowledge, this is the second antemortem case of a left-sided cisterna chyli in literature. The cisterna chyli can mimic retrocrural lymphadenopathy, solid tumor with cystic degeneration, abscess or hematoma. The left-sided cisterna chyli should be referred to as a structure so as to be cautious in surgical approach.
Abscess ; Aorta ; Cholangiocarcinoma ; Hematoma ; Lymphatic Abnormalities ; Lymphatic Diseases ; Thoracic Duct

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, post-double switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ≤0.9 g/dL) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.
Adhesives ; Arteries ; Cardiac Surgical Procedures ; Child ; Child, Preschool ; Chyle ; Chylous Ascites ; Embolization, Therapeutic ; Endoscopy, Gastrointestinal ; Female ; Heart Defects, Congenital ; Heart Septal Defects, Ventricular ; Humans ; Ligation ; Lymphatic Abnormalities ; Lymphography ; Pleurodesis ; Postoperative Complications ; Protein-Losing Enteropathies ; Pulmonary Atresia ; Rehabilitation ; Serum Albumin ; Thoracic Duct ; Tomography, Emission-Computed ; Weight Gain

Congenital cystic lymphatic malformations on the extremities are very rare. The patient described in this case study presented with a cutaneous and pedicled macrocystic lymphatic malformation that was eliminated by electrocauterization. A 4-day-old female infant with a congenital cutaneous mass on the dorsal area of her left first metacarpophalangeal joint presented to an outpatient clinic. An electrocautery device was used to cut the pedicle gently with minimal bleeding to avoid mass rupture and to minimize morbidity. A simple wet dressing was applied for 1 week, and the wound subsequently healed completely. Cutaneous macrocystic lymphatic malformations are very rare, especially on the extremities, and no consensus exists on their treatment, which has not been previously described. This report presents this rare case, along with a review of the literature.
Ambulatory Care Facilities ; Bandages ; Consensus ; Electrocoagulation ; Extremities ; Female ; Hemorrhage ; Humans ; Infant ; Lymphatic Abnormalities ; Lymphocele ; Metacarpophalangeal Joint ; Rupture ; Skin Neoplasms ; Upper Extremity ; Wounds and Injuries

BACKGROUND: Lymphatic malformation (LM) is a form of congenital vascular malformation with a low incidence. Although LM has been studied, no consensus has emerged regarding its cause or treatment. METHODS: In this study, we retrospectively evaluated 40 patients who visited our vascular anomalies center for the treatment of cervicofacial LM, which is a common manifestation of LM. The medical records of patients over a period of 12 years were reviewed and analyzed for commonalities regarding the diagnosis and the results of treatment. RESULTS: Suspected cervicofacial LM was confirmed through imaging studies. No difference in incidence was observed according to sex, and 73% of patients first presented with symptoms before the age of two years. The left side and the V2-V3 area were most commonly affected. No significant differences in incidence were observed among the macrocystic, microcystic, and combined types of LM. A total of 28 out of 36 patients received sclerotherapy as the first choice of treatment, regardless of the type of lesion. Complete resolution was achieved in only 25% of patients. CONCLUSIONS: LM is important to confirm the diagnosis early and to choose an appropriate treatment strategy according to the stage of the disease and each individual patient's symptoms. When treatment is delayed or an incorrect treatment is administered, patient discomfort increases as the lesion gradually spreads. Therefore, more so than is the case for most other diseases, a team approach on a case-by-case basis is important for the accurate and appropriate treatment of LM.
Consensus ; Diagnosis ; Humans ; Incidence ; Lymphangioma ; Lymphatic Abnormalities ; Medical Records ; Retrospective Studies* ; Sclerotherapy ; Vascular Malformations

Lymphangioma is a congenital abnormality of the lymphatic system detected primarily in early childhood. There are rare reports of mediastinal lymphangioma in older adults. We hereby report on a 66-year-old female patient who underwent kidney transplantation 20 years previously and who developed pathologically confirmed solitary mediastinal lymphangioma 1 year ago. Chest radiography showed a mediastinal nodule, which was not observed 2 year previously, therefore she was referred to the pulmonary division. She had no symptoms, and chest computed tomography demonstrated a 25-mm, well-defined, low-density nodule located at the anterior mediastinum. The size of the nodule had increased from 25 mm to 34 mm 1 year later, and it was completely resected via video-assisted thoracic surgery. The histological diagnosis was cystic lymphangioma. Therefore, we recommend that clinicians consider cystic lymphangioma as a possible diagnosis even in older patients with a mediastinal cystic mass that shows progressive enlargement.
Adult ; Aged* ; Congenital Abnormalities ; Diagnosis ; Female ; Humans ; Kidney Transplantation* ; Kidney* ; Lymphangioma* ; Lymphangioma, Cystic ; Lymphatic System ; Mediastinal Cyst* ; Mediastinum ; Radiography ; Thoracic Surgery, Video-Assisted ; Thorax

Microcystic lymphatic malformation (lymphangioma circumscriptum) is the most common cutaneous lymphatic malformation, consisting of abnormal, dilated, and tortuous lymphatic vessels in the dermis and subcutaneous tissue. It is due to irregular vessel contraction with subsequent dilatation and fluid build-up that manifests clinically as multiple, grouped, small macroscopic superficial vesicles filled with clear or serosanguineous fluid. The lesions are frequently located over proximal limbs, axillae and chest but may occur on any part of the body. We report a case of lymphangioma circumscriptumin a four-year-old child, partially treated with fractional carbon dioxide laser ablation under local anesthesia. Most of the lesions resolved but the treated areas healed with hypertrophic scarring after one month, which was cosmetically acceptable. Compared to surgical excision, carbon dioxide laser ablation may provide less chances of contractures especially in areas overlying a joint and may be considered as an alternative to more invasive procedures.

Human ; Female ; Child Preschool ; Anesthesia, Local ; Axilla ; Cicatrix ; Contracture ; Dermis ; Dilatation ; Lasers, Gas ; Lymphangioma ; Lymphatic Abnormalities ; Lymphatic Vessels ; Subcutaneous Tissue

Lymphatic malformations including macrocystic and microcystic malformation are congenital benign vascular disease. The incidence in children is much higher than adults and they most often occur in head and neck. There are a variety of diagnosis and treatment methods in lymphatic malformations. This paper reviewed the recent literatures with respect to the pathology, imaging and treatment of the lymphatic malformations in children. And we detailed the advantage and disadvantage of surgical excision, sclerotherapy, laser ablation and drug treatment of lymphatic malformations particularly in children. Surgical excision continues to be integral to treat in many cases when the lesions involve the eye socket or are large enough to affect breathing, but it should not be overused without consideration of the histologic types and extent. In addition, cervicofacial macrocystic lesions tend to sclerotherapy while superficial mucosal microsystic lesions are amenable to laser therapy. In order to obtain the best treatment outcomes, the treatment protocol should be individualized and comprehensive.
Child ; Head ; pathology ; Humans ; Laser Therapy ; Lymphatic Abnormalities ; diagnosis ; therapy ; Neck ; pathology ; Sclerotherapy ; Treatment Outcome

OBJECTIVETo analyze the clinical characteristics and diagnosis of 2 cases with chylothorax due to primary lymphatic dysplasia and to elevate pediatrician's recognition level for this disease.

METHODClinical manifestations of the children were retrospectively analyzed. Primary lymphatic dysplasia was diagnosed by lymphoscintigraphy.

RESULTThe first patient was a male aged 2-year-7-month who presented with a history of tachypnea for 43 days, fever and sore throat for 5 days at the early stage of the illness. He had a history of external injury before his illness. Physical examination showed his left chest bulging and left side diminished breath sound. His pleural effusion showed dark red (It was divided into two layers after standing, the upper layer turned into milky white, and the lower turned into hemorrhagic liquid) . White blood cell (WBC) count was 9 000×10(6)/L, mononuclear cell was 0.9, polykaryocytes was 0.1, triglyceride was 12.37 mmol/L in the pleural effusion. Contrast-enhanced lung CT (revascularization) showed pericardial effusion and a massive left sided pleural effusion. The second patient was a male aged 9 years and 6 months, who presented with a history of cough for 24 days, intermittent fever, vomiting, abdominal pain for 19 days, and edema of lower limbs for 4 days. Physical examination showed edema in both eyelids, lower extremities and scrotum. The level of albumin was 14 g/L and the titer of Mycoplasma pneumoniae IgM was 1: 320 in the serum. His hydrothorax pleural effusion showed milk white. White blood cell (WBC) count was 74×10(6)/L, mononuclear cell was 0.78, polykaryocytes was 0.22, triglyceride was 1.01 mmol/L in the pleural effusion. Chyle test showed positive in his pleural effusion and seroperitoneum. High-resolution CT of the lung revealed bilateral interstitial and parenchymal infiltration and both sided pleural effusion. Abdominal ultrasound showed giant hypertrophy of the gastric mucosa and massive ascites. Gastroscopy showed giant hypertrophy of the gastric mucosa. Lymphoscintigraphy revealed primary lymphatic dysplasia in both children.

CONCLUSIONPrimary lymphatic dysplasia might occur in children and result in dropsy of serous cavity (chylothorax, chylopericardium, chylous ascites). Dropsy of serous cavity showed bloody or milk white. WBC count might elevate with lymphocyte increasing mostly, triglyceride was often higher than 1.0 mmol/L in dropsy of serous cavity. Primary lymphatic dysplasia can be diagnosed by lymphoscintigraphy.

Child ; Child, Preschool ; Chylothorax ; diagnosis ; etiology ; pathology ; Humans ; Leukocyte Count ; Lymphatic Abnormalities ; complications ; diagnosis ; pathology ; Lymphoscintigraphy ; Male ; Pericardial Effusion ; diagnosis ; etiology ; Pleural Effusion ; diagnosis ; etiology ; pathology ; Tomography, X-Ray Computed

The presence of aberrant thyroid tissue in the lateral neck is very rare. In addition, nodular hyperplasia in ectopic thyroid has rarely been reported. Due to the unusual location, the presence of lateral aberrant thyroid tissue could be misdiagnosed as a lymphadenopathy, neurogenic tumor, etc. We report on a case of nodular hyperplasia arising from the right lateral aberrant thyroid tissue.
Congenital Abnormalities ; Hyperplasia ; Lymphatic Diseases ; Neck ; Thyroid Dysgenesis ; Thyroid Gland