CT Lymphangiography (CTL) in Primary Intestinal Lymphangiectasia (PIL): A Comparative Study with Intraoperative Enteroscopy (IOE).

RATIONALE AND OBJECTIVES: To investigate the clinical feasibility of CT lymphangiography (CTL) in primary intestinal lymphangiectasia (PIL) by comparison with intraoperative enteroscopy (IOE) during exploratory laparotomy. MATERIALS AND METHODS: Eleven PIL patients (F/M, two/nine, age range 10-37 years) were recruited in this study, and they were performed IOE during exploratory laparotomy for suspected serious lymphatic-intestinal leakages. All the patients were performed CTL before surgery, and the imaging data were reviewed by two radiologists separately. CTL assessments included intestinal lesions, edematous lesions, intestinal and mesenteric lymphangiectasia, lymphaticabdominal leakages, lymph fluid reflux, lymphangioma and abnormal lymphatics in other area. The intestinal lymphangiectasia and lymphaticintestinal leakages were confirmed by histology and IOE. RESULTS: For CTL, (1) nine intestinal wall thickening; (2) eight ascites, complicated with four pleural effusions, (3) eight intestinal and mesenteric lymphangiectasia, (4) six lymph fluid reflux (5) one lymphatic-abdominal leakage, (6) two lymphangioma. While for IOE, intestinal lymphangiectasia has been confirmed in all patients, including five segemental and six diffusive lesions in intestinal mucosa. Besides, one lymphatic-intestinal fistula, one lymphatic-abdominal leakage was confirmed. Compared to IOE and histology, the accuracy of CTL was 72.7% in detecting intestinal lymphangiectasia. CONCLUSION: Compared to IOE, CTL demonstrates feasibility in detection of intestinal lymphangiectasia and other abnormalities in whole lymphatic circulation for PIL. Combination of CTL with IOE accommodates guidance for preoperative evaluation and therapeutic management for PIL.

Milky Mesentery: Acute Abdomen with Chylous Ascites.

BACKGROUND: Clinical presentations of intestinal lymphangiectasia include pitting edema, chylous ascites, pleural effusion, diarrhea, malabsorption and intestinal obstruction. CASE CHARACTERISTICS: An 8-year-old male child presented to the emergency department with clinical features of peritonitis, raising suspicion of appendicular or small bowel perforation. INTERVENTION/OUTCOME: Diagnosis of chylous ascites with primary intestinal lymphangiectasia made on laparotomy. MESSAGE: Acute peritonitis may be a presentation of primary intestinal lymphangiectasia and chylous ascites.

[Surgical treatment of rare combination of intestinal malrotation with secondary lymphangiectasia].

The rare combination of intestinal lymphangiectasia with malrotation of the duodenum in a child of three months of life is described. Basing on the literature review only 3 similar cases were described in the world practice. The boy with protein-losing enteropathy was examined at Moscow Scientific Centre of Children's Health. The child had vomiting, diarrhea, loss in body weight, hypoproteinemia, lymphopenia. The infectious nature of the disease was excluded. It had been suggested the Waldman desease (primary intestinal lymphangiectasia). The prognosis for such disease is unfavorable. An examination of the child was continued against the backdrop of ongoing symptomatic therapy. Complete physical examination included monitoring laboratory blood tests, X-ray examination with contrast, CT-scan, gastroduodenoscopy with biopsy of the mucosa of the small intestine. Malrotation duodenum with the recurrent mid-gut volvulus with the development of secondary intestinal lymphangiectasia was diagnosed. Modern methods of examination and multidisciplinary approach made it possible to diagnose the case. Operation to eliminate fixation duodenum resulted in the recovery of the patient. At the present time the child grows and develops according to age and does not require treatment. The prognosis for this disease is regarded as favorable.

Indocyanine green lymphography and lymphaticovenous anastomosis for generalized lymphatic dysplasia with pleural effusion and ascites in neonates.

BACKGROUND: The fatality rate of generalized lymphatic dysplasia (GLD) with chylous pleural effusion and ascites is particularly high when it persists over a prolonged period. The purpose of this report was to determine the utility of indocyanine green (ICG) lymphography and lymphaticovenous anastomosis (LVA) in GLD with chylous pleural effusion and ascites in neonates. METHODS: We tested the lymphatic function in the 4 extremities for 8 GLD neonate patients using ICG lymphography, and on the basis of the results, we performed LVA for 5 of them. LVA was performed at the extremities under general anesthesia using incisions <1 cm in length. The outcome of LVA was evaluated with the amount of ascites discharged from thoracostomy tube or abdominal tube, except for 1 patient who had no drainage tube. RESULTS: In all cases, ICG lymphography showed varying degrees of dermal backflow in the limbs with lymphostasis. After LVA surgery, effusion stopped in 2 cases and decreased in 1 case. In the cases where effusion stopped, backflow as observed with ICG lymphography was minimal, and in the case where effusion decreased but did not stop, backflow was moderate. CONCLUSIONS: The application of ICG and LVA could possibly be used to diagnose and treat lymphatic pleural effusion or ascites.

[Surgical therapy of segmental jejunal, primary intestinal lymphangiectasia]. / Die operative Therapie einer segmentalen, primären intestinalen Lymphangiektasie des Jejunums mit ausgeprägtem Chylaskos.

Primary intestinal lymphangiectasia (PIL) is a protein-losing, exsudative gastroenteropathy causing lymphatic obstruction. Diagnosis depends on clinical examination and histological findings. Conservative treatment modalities include a low-fat diet and enteral nutritional therapy in order to reduce enteric protein loss and to improve fat metabolism. Other treatment options consist of administration of antiplasmin or octreotide to lower lymph flow and secretion. We report on a 58-year-old patient who underwent exploratory laparotomy due to a worsening physical status, recurrent chylaskos and leg oedema under conservative dietary therapy. Intraoperative findings showed a typical PIL of the jejunum about 20 cm distal to the Treitz's ligament. Histological examinations confirmed this diagnosis. One year after segmental small bowel resection (105 cm) with end-to-end anastomosis the patient is healthy, free of symptoms, has gained weight and his serum protein level has increased. Intraabdominal ascites and leg oedema have not reoccurred since.

Malrotation of the intestine and chronic volvulus as a cause of protein-losing enteropathy in infancy.

Protein-losing enteropathy in children is caused by intestinal metabolic, inflammatory, or infectious processes, or by lymphatic obstruction (intestinal lymphangiectasia). In this report, a 17-month-old child is presented with protein-losing enteropathy due to intestinal malrotation and chronic midgut volvulus causing lymphatic obstruction and spillage of lymph in the intestine and the peritoneum. This report should alert the pediatrician that intestinal malrotation should be added to the wide list of possible causes of protein-losing enteropathy in children.

Malformación linfangiomatosa intestinal con tejido pancreático ectópico: a propósito de un caso y revisión de la literatura / Intestinal lymphangiomatosis with ectopic pancreatic tissue: a case report and literature review

Intestinal lymphangiomatosis is a rare malformation typically affecting children. Its etiology is unknown, although several hypotheses have been proposed. We present a case of intestinal lymphangiomatosis with ectopic pancreatic tissue of the mesentery, an association not previously described in literature. We emphasize the fact that this entity has sometimes been associated with other intra-abdominal malformations, even conditions undescribed in scientific literature. It must be considered the first differential diagnosis for pediatric patients with radiological evidence of mesenteric cystic lesions, subsequently confirmed by pathological examination.

La malformación linfangiomatosa intestinal es una entidad poco frecuente, típica de la edad pediátrica, cuya etiopatogenia es desconocida, aunque se barajan varias hipótesis. Presentamos un caso de linfangiomatosis intestinal con tejido pancreático ectópico mesentérico, una asociación no descrita anteriormente en la literatura. Destacamos la importancia de conocer que esta entidad, en ocasiones, se asocia a otro tipo de malformaciones intrabdominales, incluso no descritas previamente en la literatura. Se debe plantear como el primer diagnóstico diferencial durante la edad pediátrica, ante el hallazgo radiológico de lesiones quísticas mesentéricas, confirmando posteriormente mediante un estudio anatomopatológico de las lesiones.

Primary intestinal lymphangiectasia successfully treated by segmental resections of small bowel.

Primary intestinal lymphangiectasia is a rare cause of protein-losing enteropathy and usually presents with intermittent diarrhea or malnutrition. Diagnosis depends largely on its pathologic condition demonstrating greatly dilated lymphatics mainly in the lamina propria of the mucosa. We report a case of primary intestinal lymphangiectasia, of the diffuse type, presenting with abdominal pain and voluminous diarrhea in a previously healthy 8-year-old boy. He had periumbilical pain for 3 months before presentation. He was managed by segmental bowel resections and end-to-end anastomoses. The histopathologic condition of the resected small intestine showed lymphatic dilation limited mainly to the subserosa and mesentery but was not prominent in the mucosa. Abdominal pain and diarrhea subsided postoperatively. The present case is the fourth report describing a response to operative resection.

Congenital chylous ascites: the roles of fibrin glue and CD31.

UNLABELLED: A 6-month-old male infant who presented with abdominal distension and congenital chylous ascites was diagnosed. He was initially refractory to conservative therapy, and then was completely cured with ligation of megalymphatics and fibrin glue application. Immunoperoxidase staining for CD31 on the biopsied peritoneal tissues highlighted the lining cells of lymphatic spaces, which indicated lymphangiectasia. CONCLUSION: This case emphasizes the effectiveness of lymphatic ligation of the retroperitoneal megalymphatics in conjunction with fibrin glue application to cure congenital lymphangiectasia.

Lymphangiectasia of small intestine presenting as intussusception.

Intussusception is defined as telescoping of a segment of gastrointestinal tract into an adjacent one. In small children, it is the commonest cause of intestinal obstruction. More than 90% of childhood intussusceptions are idiopathic. We report a rare case of localized small intestinal lymphangiectasia, presenting as intussusception in a 6-month-old male child. The child presented with features of acute intestinal obstruction for which he was later operated. The gross examination of excised ileocecal mass revealed intussusception. Histopathologic examination revealed lymphangiectasia of small intestine, which acted as a lead point for ileocecal intussusception. Postoperative period was uneventful.

Segmental dilatation of the ileum accompanying hypoproteinemia.

PURPOSE: Segmental intestinal dilatation (SID) is a rare pathologic finding, which causes intestinal obstruction in newborn period and gastrointestinal bleeding, anemia, abdominal pain, malabsorption, and growth failure in older children. We present a case of SID causing hypoproteinemia. PATIENT: A 10-year-old girl presented with recurrent abdominal distension since she was 8.5 months old. She was diagnosed to have protein-losing intestinal lymphangiectasia. Result of physical examination was normal except moderate growth retardation. Her blood workup showed depletion in total protein, albumin, and globulin levels. Gastrointestinal series revealed a dilated segment of small intestine, and Tc(99m)-pertechnetate scintigraphy detected ectopic activity in abdomen. The patient was taken to operation with the presumptive diagnosis of intestinal duplication. A dilated segment of ileum was encountered, and segmental resection and anastomosis were performed. RESULTS: Patient was discharged on the postoperative fourth day and remains well. Histopathologic examination of the specimen revealed SID. DISCUSSION: Segmental intestinal dilatation is an uncommon congenital anomaly. Most of the cases were operated on in newborn period because of intestinal obstruction, and their diagnoses were made perioperatively; the others were diagnosed in older ages during the investigation of nonspecific symptoms. The index patient is the second case of SID having hypoproteinemia in the literature. Gastrointestinal series suggested the diagnosis, and segmental resection and anastomosis were the adequate treatments.

Volvulus of a jejunal lymphatic vascular malformation presenting with bilious vomiting and the radiological appearances of malrotation.

Bile-stained vomiting in infancy is abnormal. Malrotation with small bowel volvulus is probably the most worrying of possible causes because if untreated for too long, it can result in massive bowel necrosis. As a consequence, if examination or plain radiography does not suggest another cause, an upper gastrointestinal (GI) series is often performed to exclude malrotation. If the examination is normal however and symptoms settle, should investigation stop, or how far should it be pursued? Small bowel lymphatic vascular malformations in children are extremely rare but carry a risk of obstruction, volvulus, and loss of small bowel. We present a case of a 5-year-old boy referred to our unit with bilious vomiting and the radiological findings of malrotation on upper GI series. Interestingly, a previous upper GI series undertaken during the first year of life for intermittent bilious vomiting was normal. Operative findings were of a 16-cm cystic lymphatic vascular malformation of the jejunal wall causing a 360 degrees volvulus. The mesentery was derotated, and the cyst removed with the attached section of jejunum. This case highlights the need to reinvestigate patients with bilious vomiting despite a previous normal upper GI series. The case raises the issue of whether bilious vomiting, with a normal upper GI series, should be investigated further with an abdominal ultrasound scan to identify more unusual causes such as jejunal lymphatic vascular malformations.

Primary mesenteric angiosarcoma in a child with associated lymphangiectasia: a case report.

Angiosarcomas are rare tumors in children, usually occurring in soft tissue and liver. By contrast, angiosarcoma in adults usually occurs in the extremities in conjunction with lymphedema. Mesenteric angiosarcoma has only rarely been reported. When angiosarcomas arise in this location, they usually represent a 2nd malignancy following Hodgkin's lymphoma. We report a child who presented to the emergency room with an acute abdomen and underwent emergency surgery for a mesenteric angiosarcoma with associated lymphangiectasia of the bowel and mesentery. A brief review of the literature and the nomenclature of these unusual tumors are discussed.

A case of recurrent gastrointestinal bleeding and protein-losing gastroenteropathy.

BACKGROUND: A 40-year-old male with pentalogy of Fallot (a congenital heart defect with five anatomical components) presented with recurrent gastrointestinal bleeding. He had recently recovered from a heart operation, which was performed to reconstruct the right ventricular outflow tract. INVESTIGATIONS: Laboratory tests and absorption tests, esophagogastroduodenoscopy, capsule endoscopy, human serum albumin scintigraphy, lymphoscintigraphy, CT and abdominal lymph-node histology. DIAGNOSIS: Intestinal lymphangiectasia with concurrent protein-losing gastroenteropathy and recurrent gastrointestinal bleeding. MANAGEMENT Despite a low-fat diet and surgical suturing of multiple small-bowel ulcerations the gastrointestinal bleeding continued. Serum albumin levels remained very low and severe lymphedema occurred. Unfortunately, the patient developed severe sepsis and died of multiple organ failure.