Congenital Pulmonary Lymphangiectasia Masked by Postoperative Pulmonary Venous Obstruction in an Infant with Total Anomalous Pulmonary Venous Connection.

Congenital pulmonary lymphangiectasia (CPL) is associated with fetal pulmonary venous obstructive physiology. The precise morbidity of CPL is unknown as CPL is generally fatal in neonates. Here, we report an infant with secondary CPL in total anomalous pulmonary venous connection (TAPVC). He developed severe pulmonary hypertension (PH) after corrective surgery for TAPVC. However, cardiac catheterization showed mild left pulmonary venous obstruction (PVO), which was deemed unnecessary for re-intervention. He died at 11 months-old due to an exacerbation of PH. Autopsy revealed medial hypertrophy of the pulmonary arteries, mild left PVO, and marked dilatation and proliferation of the pulmonary lymphatics which might have been involved in the PH, although CPL was not conclusively identified based on the previous biopsy findings. We should be aware of the possibility of CPL in addition to postoperative PVO when encountering patients with fetal pulmonary venous obstructive physiology. Furthermore, a cautious approach to the interpretation of lung biopsy results is warranted.

Long-term efficacy of lymphovenous anastomosis for the treatment of retroperitoneal lymphangiectasia: A case report and a review of literature.

Lymphovenous anastomosis (LVA) represents an alternative treatment for retroperitoneal lymphangiectasia. In contrast to sclerotherapy or excision, which may risk lymphatic obstruction and subsequent lymphedema, LVA preserves existing lymphatic architecture and transit. This report shows long-term efficacy of LVA for functional decompression of a symptomatic pathologically dilatated retroperitoneal lymphatics. A 47-year-old female with retroperitoneal lymphangiectasia refractory to multiple percutaneous drainages and treatments with sclerosing agents underwent LVA with anastomosis of a dominant segment of retroperitoneal lymphangiectasia to the deep inferior epigastric vein. Postoperative serial magnetic resonance imaging with 3-dimensional volume calculation over the 27 months follow-up showed evidence of decompression of the lesion with patent bypass. There were no known immediate complications nor requirement of further interventions. The patient's subjective pain also decreased substantially. This report confirms long-term efficacy of LVA for retroperitoneal lymphangiectasia as an alternative to sclerotherapy and surgical excision in the setting of previously failed treatments.

Congenital interstitial lung diseases: What the anesthesiologist needs to know.

Congenital interstitial lung diseases can affect both adults and children. Pediatric congenital interstitial lung diseases generally carry high risk for morbidly and mortality and include congenital alveolar capillary dysplasia with misalignment of pulmonary veins, congenital alveolar dysplasia, acinar dysplasia, congenital pulmonary lymphangiectasis, diffuse pulmonary lymphangiomatosis, neuroendocrine cell hyperplasia of infancy, pulmonary hemosiderosis, pulmonary alveolar proteinosis, and pulmonary interstitial glycogenosis. Given their usual non-specific clinical presentation, they are frequently misdiagnosed and recognized late, particularly in children who have been apparently healthy for several years (eg, diffuse pulmonary lymphangiomatosis). Some diseases have a very poor prognosis, whereas others have a benign course with appropriate treatment. The current manuscript reviews congenital interstitial lung diseases that typically affect neonates and young children and may be encountered by the pediatric anesthesiologist.

A rare case of pulmonary lymphangiectasia associated with CHD.

CHD may, at times, occur in the framework of other rare pathologies. These, having similar clinical manifestations, present a diagnostic dilemma for the clinician.The authors present the case of an infant with non-syndromic complete atrioventricular septal defect, whose post-operative period was surprisingly complicated by progressive pulmonary hypertension. Despite intensive care, the infant ultimately died. The diagnosis of unilateral primary pulmonary lymphangiectasia was only possible post mortem.

Individual approach for treatment of primary intestinal lymphangiectasia in children: single-center experience and review of the literature.

BACKGROUND: Intestinal lymphangiectasia is a rare disease. Thus, prospective studies are impossible, and therapy is still controversial. Several medicines are suggested for treatment but there are no existing indications for drug choice and treatment guidelines. We aimed to introduce the action mechanism of each drug and treatment overview in a single-center experience and a review of the literature on second-line therapy for primary intestinal lymphangiectasia. METHOD: Children under 18 years old diagnosed with intestinal lymphangiectasia from June 2000 to June 2020 were included and retrospectively reviewed in the study. Capsule endoscopy, MR lymphangiography, or whole-body MRI for investigating the extent of abnormal lymphatic vessels in addition to endoscopy and biopsy were conducted. The individual treatment approaches depended upon the lymphangiectasis locations involved. RESULTS: Only one patient showed a response to dietary therapy. One patient was successfully cured after two therapeutic lymphatic embolization. Octreotide was tried for two patients who had extensive lymphangiectasis. Lymphangiectasis recurred when octreotide was used for 3 months in one patient, and there was no effect in the other patient. Sirolimus was tried for four patients. Two of them had abnormal lymphatic lesions only in the intestine, and the others had extensive lymphangiectasis. The former group showed clinical improvement after 3-4 months of sirolimus treatment, whereas the latter group showed clinical improvement only after 1 month of sirolimus treatment. CONCLUSION: Surgery or embolization is a potential therapeutic option for patients with focal abnormal lymphatic lesions. Octreotide is not an optimal choice for patients with extensive lymphangiectasis. Sirolimus is an effective and safe drug and can be the first drug of choice for patients with extensive lymphangiectasis.

Effective Initial Treatment of Diffuse Pulmonary Lymphangiomatosis with Sirolimus and Propranolol: A Case Report.

Diffuse pulmonary lymphangiomatosis (DPL), an exceptionally rare disease, mainly occurs in children and young adults of both sexes. Even though DPL is considered to be a benign disease, its prognosis is relatively poor. Because of its rarity, little guidance on diagnosis and treatment is available, which makes working with patients with DPL challenging for clinicians. We present here a case of a young man with DPL in whom treatment with sirolimus and propranolol rapidly achieved positive radiological and clinical effects.

Abnormal pulmonary lymphatic flow in patients with paediatric pulmonary lymphatic disorders: Diagnosis and treatment.

Pulmonary lymphatic disorders are characterized by the presence of the abnormal lymphatic tissues in the thoracic cavity, presenting clinically as chylothorax, chylopericardium, chyloptysis, interstitial lung disease and plastic bronchitis. These conditions include: neonatal chylothorax, cardiac and non-cardiac plastic bronchitis, non-traumatic chylothorax, post congenital cardiac surgery chylothorax and complex lymphatic malformations. Recently developed lymphatic imaging techniques, such as intranodal lymphangiography and dynamic contrast enhanced magnetic resonance lymphangiography demonstrated abnormal pulmonary lymphatic flow from thoracic duct into pulmonary parenchyma as a pathophysiological mechanism of these diseases. Novel minimally invasive lymphatic interventions, such as thoracic duct embolization, interstitial lymphatic embolization and surgical lympho-venous anastomosis, provide an effective treatment of these conditions.

Oral lymphangiectasia and gastrointestinal Crohn disease.

Lip edema with non-caseating granulomas or lymphangiectasia pose a clinical and pathological challenge. These findings can be attributed to cheilitis granulomatosa (CG), Melkersson-Rosenthal syndrome (MRS), or Crohn disease (CD) depending on the appropriate clinical context. Lymphangiectasis, in particular, is a common pathological finding in CD due to lymphatic obstruction by granulomas and intralymphatic granulomas. Because oral symptoms can precede gastrointestinal symptoms of CD or be seen in patients with asymptomatic gastrointestinal disease, the identification of lymphangiectasia should raise the possibility of underlying CD. We present a case of a young woman with several years of lip swelling, with notable lymphangiectasia and subtle granulomas on pathological evaluation. The patient was diagnosed with MRS at an outside institution and treated with systemic steroids, without further systemic evaluation. We believe that early recognition of lymphangiectasia and consideration of CD early in the work-up are critical for early diagnosis and appropriate management. Neither clinical nor histopathological findings should be used in isolation to diagnose GC, MRS, or CD as there is significant debate as to the etiology and overlapping findings of these conditions. We highlight the importance of lymphangiectasia in diagnosing underlying CD in the appropriate clinical context.

Current and future role of fetal cardiovascular MRI in the setting of fetal cardiac interventions.

Over recent years, technical developments resulting in the feasibility of fetal cardiovascular magnetic resonance (CMR) have provided a new diagnostic tool for studying the human fetal heart and circulation. During the same period, we have witnessed the arrival of several minimally invasive fetal cardiac interventions (FCI) as a possible form of treatment in selected congenital heart diseases (CHDs). The role of fetal CMR in the planning and monitoring of FCI is not yet clear. Indeed, high-quality fetal CMR is not available or routinely offered at most centers caring for patients with prenatally detected CHD. However, in theory, fetal CMR could have much to offer in the setting of FCI by providing complementary anatomic and physiologic information relating to the specific intervention under consideration. Similarly, fetal CMR may be useful as an alternative imaging modality when ultrasound is hampered by technical limitations, for example, in the setting of oligohydramnios and in late gestation. In this review, we summarize current experience of the use of fetal CMR in the diagnosis and monitoring of fetuses with cardiopathies in the setting of a range of invasive in utero cardiac and vascular interventions and medical treatments and speculate about future directions for this versatile imaging medium.

Localized acquired lymphangiectasias after breast surgery: Enhanced non-invasive diagnosis using dermoscopy and reflectance confocal microscopy.

BACKGROUND: Acquired lymphangiectasias represent cystic dilatations of the cutaneous lymphatic vessels resulting from damage and/or obstruction of previously normal lymphatics, usually secondary to surgery, radiotherapy, and/or infections. MATERIALS AND METHODS: The clinical, dermoscopic, and confocal microscopy features of three cases of acquired lymphangiectasias occurring after breast surgery are described along with histopathological correlations. RESULTS: Polarized dermoscopy revealed in all lesions the presence of well-circumscribed, white-yellowish lacunae surrounded by pale septa. In addition, some lesions showed scattered reddish areas and red lacunae. Confocal microscopy showed in the upper dermis numerous, roundish dark cavities separated by thin septa. These features corresponded histopathologically to saccular dilations and ectatic lymphatic vessels lined by a single layer of endothelial cells in the papillary/reticular dermis. CONCLUSION: The use of non-invasive diagnostic techniques may be addressed to an enhanced non-invasive diagnosis of acquired lymphangiectasias by showing peculiar features, thus avoiding the need for skin biopsy.

Imaging of fetal lymphangiectasias: prenatal and postnatal imaging findings.

Lymphangiectasias are lymphatic malformations characterized by the abnormal dilation and morphology of the lymphatic channels. The classification and treatment of these disorders can be challenging given the limited amount of literature available in children. Various imaging modalities are used to confirm suspected diagnosis, plan the most appropriate treatment, and estimate a prognosis. Prenatal evaluation is performed using both prenatal US imaging and fetal MRI. These modalities are paramount for appropriate parental counseling and planning of perinatal care. During the neonatal period, chest US imaging is a useful modality to evaluate pulmonary lymphangiectasia because other modalities such as conventional radiography and CT display nonspecific findings. Finally, the recent breakthroughs in lymphatic imaging with MRI have allowed us to better classify lymphatic disorders. Dynamic contrast-enhanced lymphangiography, conventional lymphangiography and percutaneous lymphatic procedures offer static and dynamic evaluation of the central conducting lymphatics in children, with excellent spatial resolution and the possibility to provide treatment. The purpose of this review is to discuss the normal and abnormal development of the fetal lymphatic system and how to best depict it by imaging during the prenatal and postnatal life.

Vulvar lymphangiectasia secondary to gastrointestinal tuberculosis infection in a teenager.

We report a rare presentation of vulvar lymphangiectasia that developed secondary to gastrointestinal tuberculosis in a teenager, and its spontaneous resolution after anti-tuberculous treatment was completed.

Treating and preventing recurrence of recurrent genital acquired lymphangiectasia using lymphaticovenous anastomosis at genital area: A case report.

After the simple resection of genital acquired lymphangiectasia, a high recurrence rate is reported, as lymphatic fluid from the legs to the genital region continues. Here we report a case in which we treated and prevented recurrence of recurrent genital acquired lymphangiectasia with genital lymphaticovenous anastomosis (LVA). A 48-year-old woman underwent hysterectomy and pelvic lymph node dissection for uterine cervical cancer at 30 years of age. The patient consulted our department for lymphedema in bilateral legs and acquired lymphangiectasia in the major labia. Despite nine attempted genital acquired lymphangiectasia resections and LVA in the lower limbs, which was aimed to prevent the recurrence, it recurred within 1-2 months postoperatively. Then we injected indocyanine green (ICG) to the bilateral edge of the anus before 10th LVA. ICG flowed from the anus to the genital area and we performed acquired lymphangiectasia resection and genital LVA on this lymphatic vessel (one anastomosis). After 6 months postoperatively, no recurrence of acquired lymphangiectasia, lymphatic leakage, or cellulitis had occurred. We found that a lymphatic flow from the anus into genital acquired lymphangiectasia occurred in one of the patients and the genital LVA, anastomosis of that responsible lymphatic vessel to the vein, might prevent genital acquired lymphangiectasia recurrence after resectional treatment.

Primary pulmonary lymphangiectasia in Noonan syndrome: apropos of an extremely rare manifestation and a brief literature review.

Noonan syndrome (NS) is a genetic multisystem disorder characterised by distinctive facial features, developmental delay, learning difficulties, short stature, congenital heart disease, renal anomalies, bleeding difficulties and lymphatic malformations. Although lymphatic dysplasias are present in 20% of patients with NS, however pulmonary lymphangiectasia has rarely been described. In this present paper, we report a 24-year-old male who was diagnosed with Noonan syndrome and primary pulmonary lymphangiectasia by using chest imaging modalities. A brief overview of the current literature is also provided laying emphasis on the clinical, pathogenetic and diagnostic aspects of this uncommon Noonan syndrome complication.

[Clinical characteristics of Kaposiform lymphangiomatosis: a report of 8 cases].

Objective: To investigate the clinical symptoms, treatment intervention and prognosis of Kaposiform lymphangiomatosis(KLA). Methods: Medical information and clinical characteristics data of 8 KLA patients who were admitted to Department of Pediatric Surgery of West China Hospital of Sichuan University from January 2016 to February 2019 were retrospectively reviewed and analyzed. There were 5 males and 3 females with age of 5.8 years old (from 8 months to 29 years old). Results: The lesions in all patients were diffusely distributed. In all 8 patients, the lung and mediastinum were involved with different degrees. Three cases had lesions involving pelvic and abdominal organs. Three cases had lesions involving bones. One case simultaneously involved pelvic and abdominal organs, and 1 case was involved laryngeal and neck. The clinical characteristics were mainly respiratory symptoms. In the laboratory tests, 6 patients had different degrees of thrombocytopenia (minimum 3 × 10(9)/L), and 4 patients had severe fibrinogen reduction (minimum 0.42 g/L). Three patients had prolonged activated partial thromboplastin time (up to 64.2 seconds) and 3 patients had prolonged prothrombin time (up to 18.6 seconds). After surgery (including thoracotomy, chest tube, pericardiocentesis, splenectomy) and empiric medicine therapy (vincristine, sirolimus and corticosteroid), the symptoms improved in 1 case, 2 cases died of complications, 2 cases were stable and 3 cases progressed up to February 2019. Conclusions: KLA is a rare disease that should be differentiated from other types of vascular diseases. Currently, there is no consensus treatment guidelines exist. Accurate diagnosis in KLA can be a challenge. The situation in patients with KLA is prone to rapid deterioration and progress. Future research efforts should seek to develop target-specific drugs for KLA.

Proliferative Cells From Kaposiform Lymphangiomatosis Lesions Resemble Mesenchyme Stem Cell-like Pericytes Defective in Vessel Formation.

Kaposiform lymphangiomatosis (KLA) is a vascular anomaly featuring lymphatic expansion. It has no known cause, no effective treatment, and is associated with high morbidity. Proliferative cells from 3 KLA patient lesions were characterized relative to adiopose-derived mesenchyme stem cells (ADSCs) and cells derived from a patient with the related disease kaposiform hemangioendothelioma (KHE). KLA cells variably expressed markers of mesenchyme stem cells (CD73, CD90, CD105, CD146) and lacked endothelial cell markers (CD31, CD34) as determined by flow cytometry. They expressed markers of vascular pericytes (neural/glial antigen 2, alpha-smooth muscle actin, platelet-derived growth factor-beta receptor, and CXCL12) as determined by quantitative reverse transcription polymerase chain reaction. Lesion cells transcribed vascular markers VEGFC and VEGFD, as well as VCAM-1, the latter of which was confirmed by flow cytometry, consistent with angiogenic MSC-like pericytes. Furthermore, conditioned medium from each was shown to promote the proliferation of growth factor-starved lymphatic endothelial cells. Unlike kaposiform hemangioendothelioma-derived MSC-like pericytes and ADSCs, KLA isolates were defective in support of vascular network formation in co-cultures with either vascular or lymphatic endothelial cells. Genetic analysis by whole exome sequencing revealed novel variant alleles in 2 populations of KLA cells (BAD, TSC1) that may bear on aberrant pericyte growth and function.

Lymphangiomatosis: a rare entity presenting with involvement of the sacral plexus.

Lymphangiomatosis is an uncommon disease process characterized by multisystem lymphatic malformations that can involve numerous body systems, including organs, muscles, soft tissues, and bones. Involvement of the nervous system is rare and has even been previously described as a site of sparing. We present a case of a 24-year-old female with known lymphangiomatosis, presenting with acute onset of lower extremity paresthesias, weakness, and new urinary retention. MRI of the pelvis revealed lymphangiomatosis of the sacral plexus, which has not been previously reported. We will review the clinical and imaging manifestations of lymphangiomatosis and provide a differential diagnosis for masses of the lumbosacral plexus. Although lower extremity pain and weakness encountered in the emergency department or outpatient setting is most frequently caused by lumbar spine pathology, occasionally, abnormalities of the lumbosacral plexus may prove to be the cause. While peripheral nerve sheath tumors lead the differential diagnosis of tumor or tumor-like entities involving the lumbosacral plexus, lymphangiomatosis is a rare differential consideration.

Acquired Lymphangiectasia of the Scrotum.

A 56-year-old man presented with multiple, skin-colored, raised eruptions of the scrotum that had been present for 2 years. Their onset had been gradual, and they had been increasing in size, resulting in cosmetic disfigurement. A year previously, he had been operated on for a bilateral vaginal hydrocele with partial excision and eversion of the sac (Jabouley method).1 There had been no extramarital or unprotected sexual contact, other hospitalizations, or major surgery, swelling of the legs, or long periods of incumbency. Cutaneous examination revealed multiple, discrete and/or coalescing verrucous papules distributed on the upper portion of the scrotum and associated with edema of the penis (Figure 1). The inguinal lymph nodes were not enlarged. Complete blood counts and ultrasonography of the abdomen were normal. Tissue sections stained with hematoxylin and eosin showed hyperkeratosis and multiple ectatic vessels, primarily confined to the papillary dermis, abutting the overlying epidermis, and demarcated by a single endothelial lining. The dilated vessels contained homogenous eosinophillic material (Figure 2).