Mesenteric and omental lymphatic malformations in children: seven-year surgical experience from two centers in China.

PURPOSE: To compare the clinical characteristics, surgical management and prognosis of mesenteric lymphatic malformations (ML) and omental lymphatic malformations (OL) in children. METHODS: This retrospective study included 148 ML patients and 53 OL patients who underwent surgical treatment at two centers between January 2016 and December 2022. Details about the patients' clinical characteristics, cyst characteristics, preoperative complications, surgical methods, and prognosis were retrieved and compared. RESULTS: No significant differences in sex ratio, prenatal diagnosis, or age of diagnosis were noted between ML and OL patients. Vomiting was more common in ML patients than in OL patients (46.6% vs. 22.6%, P = 0.002), but OL patients were more likely to be misdiagnosed (35.8% vs. 18.9%, P = 0.012). The size of the cysts in OL patients was significantly larger than that in ML patients (14.0 [4.0-30.0] vs. 10.0 [2.0-50.0] cm, P<0.001), and cysts with turbid fluid were more common in OL patients (38.0% vs. 20.6%, P<0.001). More OL patients than ML patients had preoperative hemorrhage or infection of cysts (41.5% vs. 31.8%, P<0.016). Cyst excision was performed in 137 (92.6%) ML patients and 51 (96.2%) OL patients, and the incidence of postoperative complications was lower (12.6% vs. 4.2%, P = 0.165) among OL patients. The main postoperative complications included adhesive ileus and recurrence of cysts. Additionally, more OL patients than ML patients were treated with laparoscopic surgery (69.8% vs. 39.2%, P<0.001). CONCLUSIONS: There were differences in clinical characteristics, cyst characteristics and preoperative complications between ML and OL patients. Cyst excision was the most common surgical method that was used to treat both ML and OL patients, and laparoscopic surgery could be a feasible surgical approach for treating OL patients with a good prognosis. TRIAL REGISTRATION: Retrospectively registered.

Operative Management of Intra-abdominal Lymphatic Malformations in Children: A Single Tertiary Center Experience.

BACKGROUND: Intra-abdominal lymphatic malformations (LMs) are relatively rare congenital anomalies and can be divided into intra- and retroperitoneal subgroups. This study aims to evaluate the outcomes after surgical resection of intraperitoneal LMs in children. METHODS: The records of all patients ≤16 years of age with intraperitoneal LMs managed at our tertiary center between 2007-2022 were reviewed. Patients with retroperitoneal LMs were excluded. Our preferred approach is surgical exploration for large (>5 cm), symptomatic lesions if they are potentially resectable on imaging. RESULTS: A total of 12 patients (10 males) were diagnosed with intraperitoneal LMs located in the small bowel mesentery or omentum at a median age of 6.6 (range, 0.6-14.4) years. Ten (83%) patients presented with acute symptoms, including abdominal pain (n = 8; 67%) and vomiting (n = 3; 25%). Two (17%) intraperitoneal LMs were found on imaging performed for other reasons. Surgical exploration was performed in 11/12 (92%) cases. Intestinal volvulus around a mesenteric macrocyst had occurred in 4 (36%) patients. Complete macroscopic resection was achieved in 10/11 (91%) patients. Most lesions were localized to a short intestinal segment. One mixed type LM with an extensive mesenteric involvement was biopsied as the lesion was deemed to be unresectable in preoperative imaging and the diagnosis was initially unclear. There was one recurrence (8%) during postoperative follow-up on imaging (median 3.6 (range, 1.5-6.9) years) but the patient has remained asymptomatic and not undergone interventions. 6/8 (75%) of screened intraperitoneal LMs were positive for a somatic PIK3CA mutation. CONCLUSIONS: Most large, pedunculated macrocystic LMs in the small bowel mesentery or omental location were amenable to macroscopic resection. Intraperitoneal LMs tend to present with acute symptoms including a risk for intestinal volvulus based on anatomical location. Our results suggest low recurrence rates or need for further interventions over follow-up. LEVEL OF EVIDENCE: III.

Congenital Mesenteric Defect with Transmesenteric Hernia in Children: A Case Series.

ABSTRACT: Transmesentric hernias are a type of internal hernia, in which there is herniation of bowel loops through a defect in the mesentery. They present with a wide variety of symptoms without any specific radiological features, leading to a delay in diagnosis and high mortality rate. Here, we present a case series of four patients with this rare but fatal cause of small bowel obstruction. Three children presented to the emergency department with small bowel obstruction. The other baby was a preterm neonate with an antenatal scan showing small bowel obstruction. All children underwent emergency laparotomy and were found to have a mesenteric defect with herniation and gangrene of the small bowel. Resection anastomosis of the gangrenous segment and closure of the mesenteric defect were done. The differential for small bowel obstruction in children should include transmesentric hernia. Laboratory or imaging investigations are often inconclusive. Timely exploration can save lives in this rare but life-threatening condition.

Absceso mesentérico inespecífico paniculitis intra-abdominal / Abscess nonspecific mesenteric panniculitis intra-abdominal

El objetivo del presente caso clínico es mostrar los signos y síntomas que puedan determinar diagnósticos presuntivos no claros, cuándo se trata de masas mesentéricas primarias, que pueden simular patologías de origen biliar, compromiso vascular mesentérico, síndromes de FID y patologías ginecológicas dependientes de anexo derecho. La TAC es el patrón diagnóstico, que no cuentan los hospitales de segundo nivel como el nuestro, de manera que las decisiones que se toman en el servicio de emergencias, frente a este tipo de abdomen agudo quirúrgico es una laparotomía exploradora; para evitar complicaciones, no solo en órganos de la cavidad abdominal, sino también de orden legal. Los resultados de la cirugía mostraron la presencia de un masa mesentérica correspondiente a un absceso mesentérico inespecífico compatible con una paniculitis intra-abdominal, patología rara dentro la estadística nacional e internacional. Los síntomas más frecuentes son dolor abdominal, anorexia, plenitud abdominal y pérdida de peso, a veces oclusión intestinal. Otras manifestaciones comprenden: estreñimiento, fiebre, diarrea y a la palpación; masa abdominal. La duración de los síntomas es mayor a dos semanas. El laboratorio a veces es normal con aumento del VES. El contraste baritado muestra la pared del colon de aspecto irregular sin lesiones intraluminales. La ecografía; masa hiperecogenica bien definida en la raíz. La TAC: aumento de la densidad grasa, del mesenterio, bien delimitada con halo graso o pseudocapsula que rodea a los ganglios y vasos (signo del anillo graso fat-ring sign).

The present study aims to display how the patient symptomatology does not help to determine a clear presuntive diagnoses, especially when we deal with primary mesenteric masses that can simulate a biliary pathology, mesenteric vascular disorder, right lower quadrant syndrome and dependent gynecological pathologies of right annex. CT is a diagnostic pattern that second level hospitals do not have, so that decisions are taken into the emergency room. An exploratory laparatomy is the option to face up such acute abdomen in order to avoid complications in organs of abdominal cavity as well as legal problems. This case report, surgery results displayed the presence of a mesenteric mass corresponding to a non-specific mesenteric abscess compatible with a intra-abdominal panniculitis, an unusual pathology according to national and international statistics data. The most frecuent symptoms are abdominal pain, anorexia, abdominal fullness, loss of weight and sometimes intestinal occlusion. Constipation, fever, diarrhea and abdominal tenderness are additional manifestations. Those symptoms last longer than two weeks. Laboratory results display normal but with an increased ESR. Barium test shows a colon wall of irregular appearance without intraluminal injury. Ultrasound shows a hyperechoic mass well defined in the root. CT reflects an increased density of the mesenteric fat, well defined with a pseudocapsule that rounds lymph nodes and vessels (fat-ring sign).

Abdomen agudo secundario a malformación congénita del mesenterio / Acute abdomen secondary to congenital malformation of the mesentery

Presentamos el caso de una primigesta de 19 semanas que comienza con un cuadro obstructivo intestinal secundario a una malformación congénita del mesenterio, que se complica con estrangulación, perforación y peritonitis fecaloidea. La evaluación de una embarazada con dolor abdominal debe ser llevada a cabo de manera rápida y eficaz. El abdomen agudo puede ser el resultado de causas gastrointestinales, ginecológicas, urológicas y obstétricas. El retraso en el diagnóstico y en la intervención quirúrgica solo empeora el resultado para la madre y el feto (AU)

We report the case of a primigravida at 19 weeks of pregnancy who presented with intestinal obstruction secondary to congenital malformation of the mesentery complicated by strangulation, perforation and fecal peritonitis. Pregnant women with abdominal pain must be evaluated quickly and efficiently. An acute abdomen may be the result of gastrointestinal, gynecological, urological or obstetric causes. Delay in diagnosis and surgical intervention only worsens the outcome for the mother and fetus (AU)

Hernia paraduodenal izquierda atípica / Atypical left paraduodenal hernia

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Hernia transmesentérica congénita en el anciano / Congenital transmesenteric hernia in the elderly

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Linfangiomiomatosis pulmonar y Linfangiomiomas: presentación de 8 casos y revisión de la literatura / Lymphangiomyomatosis and lymphangiomyomas: a study of 8 cases and review of the literature

Se presenta un estiduo clinicopatológico de 3 casos de linfangiomiomatosis pulmonar y mediastinal y de 5 linfangiomiomas de mediastino, retroperitoneo, mesenterio (2) y hombro. Seis pacientes fueron del sexo femenino y 2 del masculino, sus edades variaron de 19 meses a 47 años. Todos los pacientes presentaron grandes tumores por períodos que variaron de meses a muchos años, los cuales no afectaron su estado general. Cinco linfangiomiomas y un pulmón con linfangiomiomatosis se extirparon quirúrgicamente, a dos pacientes con linfangiomiomatosis solamente se les pudo biopsiar. Las lesiones midieron entre 12 y 32 cm de eje mayor, frecuentemente eran de aspecto multiquístico con pequeñas áreas sólidas. Microscópicamente, se observaron innumerables espacios quísticos, separados por septos tapizados con revestimiento endotelial y/o epitelial y dotados de músculo liso en haces. Un estudio inmunohistoquímico demostró que los quistes de la linfangiomiomatosis pulmonar tiene su orígen principalmente en conductos aéreos, mientras que los de los linfangiomiomas son exclusivamente de naturaleza vascular. Se informan 3 casos excepcionales en edad pediátrica (1 7/12, 9 y 11 años ) 2 localizaciones no descritas antes (mesenterio y hombro). Se presenta una revisión de las características clinicopatológicas de las dos formas de la enfermedad y su probale asociación con esclerosis tuberosa. Se discuten algunas ideas sobre la histogénesis y la etiopatogenia en estas lesiones de probable naturaleza hamartomatosa o corsistomatosa.

Mesenteritis retráctil con compromiso del colon, correlación radiológica y patológica

Se presenta el caso de un paciente de 48 años de edad, de sexo masculino, cuyo diagnóstico definitivo fue mesenteritis retráctil, con compromiso principalmente del colon y con una de las complicaciones inusuales de esta entidad, como es la estenosis del colon. Es éste un raro desorden caracterizado por engrosamiento del mesenterio bien sea local o difuso debido a inflamación crónica y fibrosis. Su incidencia es relativamente baja. (2 casos/1millón/año), y son pocos casos presentados en la literatura mundial