IntroductionPrimary intestinal lymphangiectasia (PIL) is a very rare disorder usually diagnosed before the third year of life or later in adulthood, presenting with pitting edema, hypoproteinemia and low immunoglobulin levels. The location and the extent of the affected bowel greatly influence the clinical manifestation. The localized or segmental form of PIL is extremely rare with only five pediatric cases reported worldwide.Case presentationA 10 year-old Caucasian boy presented with 3 months history of recurrent abdominal pain and a 1 month history of diarrhea. An ultrasound scan was performed on two separate occasions 10 days apart, revealing a growing cystic mass on the right side of the abdomen, in front of the psoas muscle. Subsequently an MRI scan confirmed that the mass originated from the mesenteries and infiltrates a short segment of the small bowel. Surgical resection of the affected segment was performed. Histopathological examination of the removed segment of ileum was consistent with intestinal lymphangiectasia. We could not identify any associated genetic syndromes or any other conditions that could have caused secondary intestinal lymphangiectasia. The patient’s recovery from surgery was uneventful and no recurrence was observed in the following 4 years.ConclusionDespite being a benign condition, mortality of PIL can be as high as 13% due to the difficulties associated with the management of the disease. PIL should be considered as a rare but potential cause for an abdominal mass, even in the older child, when cystic mesenterial involvement might be seen on ultrasound or MRI. In selected cases of PIL affecting only a short segment of the bowel or following unsuccessful conservative treatment, surgical resection of the affected bowel segment can be curative.

Objectives: Minimal invasive repair of pectus excavatum (MIRPE) described by Nuss is the most popular correction nowadays of this deformity. During the introduction of the bars, they can hurt or compress the internal mammary arteries (IMA). The aim of this study was to observe the prevalence of IMA compression in children after MIRPE. Also, we examined if IMA obstruction increases the risk of complications at bar removal, and if these vascular changes are reversible.Materials and Methods: All patients operated on pectus excavatum in our tertiary pediatric surgical center between 2013 and 2019 were involved in the study. Data of age, sex, number of bars and characteristics of the deformity were examined. IMA flow was checked by Doppler ultrasound (DUS) after MIRPE and after bar removal, too.Results: Among 41 patients with mean age of 15.2 years there were 18 asymmetrical deformities, 23 sternal rotations. Mean pectus index was 4.01. After the Nuss procedure 7(9%) stenoses and 10(12%) occlusions of IMA were found on DUS. After bar removal 3 of 4 stenoses have resolved, but all examined occlusions (3/3) persisted. There were no complications during bar removals.Conclusion: IMA compression after MIRPE in children is uncommon, and is not influenced by severity of deformity. Obstruction of these vessels does not increase the risk of hemorrhagic complications at bar removal. Data of larger cohort are needed to determine reversibility of these changes.

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