Metrical evaluation of Slovak patients with pectus excavatum.

PURPOSE: We have started to assess the severity of pectus excavatum by means of anthropometric methods prior to CT examination since 2012. The aim of the study was to establish a significance of anthropometry as first-line diagnostic method. Afterwards, we analyzed statistical significance of differences in selected anthropometric indicators before and after surgical intervention. The analysis was also focused on the data from CT scans. METHODS: The followed group represented 27 patients, including 6 girls and 21 boys aged 7-18 years (mean age 15.59 years). Evaluation of anthropometric measurements was realized by somatometry, and other metrical measurements were calculated from thoracic CT scans of patients. All measurements were managed with the approval of the Ethics Committee. RESULTS: The significant differences were found in sagittal diameter and thoracic index in 64.0 % of the patients. Data analyzed from CT showed that flat chest prevailed in both gender (100 %). The largest group represented asymmetric type of pectus excavatum (40.74 %). The superiority of the asymmetric type to the right was confirmed. The values of the Haller index were in range 2.00-5.17 (mean value 3.64). 81.5 % of patients met criterion for surgical correction. Most patients with pectus excavatum had a milder form of deformation. CONCLUSIONS: Anthropometry provides feasible and non-invasive method of pectus deformities evaluation. Based on the study results, anthropometry should be performed prior to CT examination in order to select patients for surgical treatment. Anthropometric measurements are helpful in accurate documentation of growth, longitudinal observation of the patient, and can support decision concerning the type of surgery.

Poland syndrome: from embryological basis to plastic surgery.

Poland syndrome is a rare congenital anomaly described by Sir Alfred Poland over 170 years ago. Combination of unilateral aplasia of the sternocostal head of musculus pectoralis major, and an ipsilateral hypoplastic hand with simple syndactyly and short fingers is typical for this condition. It occurs more frequent among males, and is usually situated on the right hemithorax in the unilateral form. The pathogenesis of Poland syndrome is not clear. Most of the authors assume that the etiologic insult is vascular in nature. During the sixth week of gestation, not only the pectoral mass splits (future muscles of the thorax) and intervening tissue between the finger rays of hands starts to disappear but also the vascular differentiation from six aortic arches begins. In our paper we report two cases of children with Poland syndrome, who underwent surgical procedure in the Department of Pediatric Surgery, Comenius University in Bratislava, Slovakia. Our case reports are focused on pre-operatively and also post-operatively imaging (RTG, CT, and 3D CT imaging) of the affected thorax and arm, as well as the operative reconstruction technique of abnormal ribs. We also discussed the possible embryonic backgrounds of this anomaly as well as the importance of plastic surgery resulting in patients' normal life.

Fetus in fetu from newborn's mediastinum: case report and a review of literature.

Fetus in fetu is a rare congenital anomaly, when the malformed monozygotic twin is found within the body of a living child or sometimes in an adult. We report a case of fetus in fetu diagnosed during the early third trimester of pregnancy, in the medial and inferior portions of anterior mediastinum of the dominant twin. The diagnosis was suspected by prenatal ultrasound and fetal magnetic resonance imaging. It was postnatally confirmed by CT and RTG, surgically removed after birth and histologically examined. Histologically we found well-developed organs and tissues derived from all germ layers. To our knowledge, we describe the fourth case of fetus in fetu located in the thorax, and the second case located in the anterior mediastinum. This case highlights the usefulness of prenatal ultrasound and MRI in the diagnosis of mediastinal masses, and the usefulness of complete histological examination for confirmation of diagnosis of fetus in fetu. We also reviewed the possible embryologic backgrounds of this congenital anomaly.

Rare peripheral femoral nerve neuropathy in children.

Peripheral neuropathy of the femoral nerve is extremely rare. In the literature, we found descriptions of only 50 similar patients, mainly as a complication of coagulopathies, and none of intrapelvic tumors. Three children with a rare peripheral neuropathy of the femoral nerve as a complication of extraperitoneal pelvic masses are described in this report. In all three, the neuropathy was caused by stretching of the femoral nerve over a huge intrapelvic mass. None was related to coagulopathy. After removal of the masses, full recovery from the neurological symptoms was observed in two children and the recovery of the patient with Ewing's sarcoma is satisfying, at the 2-year follow-up and at the time of writing. As early surgical debulking of the mass was a good influence on the recovery of the nerve paresis, we believe that is imperative in the treatment process. Medical staff should be aware that a serious illness can lie behind peripheral femoral nerve neuropathy.

First experience with single incision laparoscopic surgery in Slovakia: concomitant cholecystectomy and splenectomy in an 11-year-old girl with hereditary spherocytosis.

AIMS: Hereditary spherocytosis is an autosomal dominant inheritance disorder of the red blood cell membrane characterized by the presence of spherical-shaped erythrocytes (spherocytes) in the peripheral blood. The main clinical features include haemolytic anemia, variable jaundice, splenomegaly and cholelithiasis caused by hyperbilirubinemia from erythrocyte hemolysis. Splenectomy does not solve the congenital genetic defect but it stops pathological hemolysis in the enlarged spleen. If gallstones are present, it is appropriate to perform cholecystectomy at the time of splenectomy, although the patient has symptoms of gall bladder disease. We present the case of single incision laparoscopic surgical (SILS) concomitant splenectomy and cholecystectomy performed with conventional laparoscopic instruments in an 11-year-old girl with the diagnosis of hereditary spherocytosis. METHODS: A 2-3 cm umbilical incision was used for the placement of two 5 mm trocars and one 10 mm flexible videoscope. Conventional laparoscopic dissector, grasper, Ligasure, Harmonic Ace and hemoclips were the main tools during surgical procedure. We prefer Single Incision Laparoscopic Surgery Foam Port (Covidien) as the single umbilical device for introduction into the abdominal cavity. First, we performed cholecystectomy, then the gallbladder was put aside over the liver and after that we peformed splenectomy. To remove the detached spleen and gallbladder, a nylon extraction bag is introduced through one of the port sites. The spleen is than morcellated in the bag with forceps and removed in fragments. After that we removed them and the umbilical fascial incision was closed. RESULT: Splenectomy is the only effective therapy for this disorder and often it is performed in combination with cholecystectomy. Conventional surgery requires a wide upper abdominal incision for correct exposure of the gallbladder and spleen. Our experience shows that SILS splenectomy and cholecystectomy is feasible even in young children and despite the small number of cases in the world, we consider the combined laparoscopic approach safe and effective for the treatment of hereditary spherocytosis. CONCLUSION: According to actually published guidelines, the laparoscopic approach to concomitant splenectomy and cholecystectomy is recommended, but it depends on the availability of appropriately trained surgeons and suitable equipment.

The first histological and immunohistochemical examination of thymus in a case of fetus in fetu.

Fetus in fetu (FIF) is a rare condition with less than 150 cases reported in the world to the best of our knowledge. It is a malformed monozygotic twin ("non-dominant twin"), which is found inside the body of a living child or sometimes in an adult ("dominant twin"). Different organs can be seen in these fetuses; vertebral column limbs, central nervous system, gastrointestinal tract, vessels, and genitourinary tract. In the literature, we found only two cases of fetus in fetu with the present thymic tissue. In this paper, the thymus of non-dominant twin exteriorized from the mediastini of dominant twin, was analyzed by histological and imunohistochemical methods. Even though the majority of organs did not develop normally in the mentioned case, thymic tissue was proved to be present in many body parts of the non-dominant twin. In spite of the fact that the cortex and the medulla were not so distinguishable as in the normal thymuses, presence of many basic cell populations was demonstrated: thymic epithelial cells (AE1/AE3 positive cells), T (CD45RO positive) and B (CD20 positive) cells, macrophages (CD68 positive cells), dendritic cells (S100 positive cells) and myoid cells (desmin positive). The Hassall's bodies were localized mostly in the medulla, however in sporadic cases they occurred in the area close to the connective tissue septa. The superficial epithelial cells of the Hassall's corpuscules as well as their internal contents, were markedly stained by alcian blue, and the cystic formations, found inside the Hassall's bodies, contained PAS-positive substance, similar to Hassall's bodies of normal thymuses. This fact indicates that although development of the parasitic twin is incomplete, all three germ layers participate on its development.