A rare histopathological fi nding after lung resection in a child.

BACKGROUND: The authors present a case of a patient with an extremely rare lung tumor in a child. CASE: A 9-year-old girl with a 3-day history of dyspnea and stabbing pain in the xiphoid region, irradiating to the area under the left costal margin, both in rest and in physical activities. She was primarily examined in a regional hospital with bounded homogenous focus of the superior right lung lobe on the X-ray. After initial treatment with antibio-tics and persistent finding on X-ray, a CT scan of the chest was performed. It revealed an irregular oval lesion of a non-homogenous structure with a contrast dye in the S3 region merged to pericardium and parietal pleura, which the presence of several micronodules. Based on the negative tumor markers, positive PET-CT scan and a negative etiology, bio-psy or eventually a lesion exstirpation were indicated. Right-sided thoracotomy, mass enucleation and exstirpation of nodular lesions were performed 2.5 months after the onset of difficulties. Postoperative recovery was uneventful, no sign of recurrence occurred during a follow-up period. The final histological finding was verified as an inflammatory myofibroblastic tumor - an extremely rare pulmonary pathology in the pediatric population. CONCLUSION: Inflammatory myofibroblastic tumor can be mimicking IgG4 sclerosing disease and inflammatory pseudotumor. It is essential to distinguish between these affections because of different (i.e. surgical vs. conservative) treatment approach.

Thyroid surgery in children: our experience.

INTRODUCTION: Thyroid surgery in children is a rare operation. The aim of our paper is to point out the specifics of thyroid surgery in children. METHODS: Retrospective analysis of patients hospitalized at the Department of Paediatric Surgery, Faculty of Medicine, Comenius University and National Institute of Childrens Diseases in Bratislava during a 10-year period (20072016) who underwent thyroid surgeries. RESULTS: The retrospective analysis included 81 patients: 66 (81%) girls and 15 (19%) boys. The mean age of the patients was 14 years ±8 months (range 418 years). The most common indications for thyroid surgery were: a nodule in 36 (44.4%) patients, Graves Basedow thyrotoxicosis in 19 (23.5%) patients, and suspected thyroid carcinoma in 11 (13.6%) patients. Cervical lymph node metastases (mts) were diagnosed in 9 (11.1%) patients, and distant pulmonary metastases in 5 (6.17%) patients. Total thyroidectomy (TTE) was performed in 43 (53%) patients, total lobectomy (TL) in 20 (24.7%) patients. Extended surgery on regional lymph nodes was performed in 9 (11.1%) patients. Eight (9.9%) patients underwent reoperation. A total of 12 (14.8%) patients experienced postoperative complications. Unilateral transient recurrent laryngeal nerve (RLN) paralysis occurred in 2 patients, and permanent in one patient. Transient postoperative hypoparathyroidism with hypocalcaemia was reported in 8 (9.9%) patients; no permanent condition of this type was observed. CONCLUSION: Multidisciplinary collaboration ensures that optimal surgical results are achieved in the patients. Experience of the surgeon performing thyroid surgery in children remains crucial.

Rare causes of respiratory insufficiency in newborns.

Congenital lung masses (CLM) the rare group of causes of acute respiratory insufficiency (RI) in newborns include congenital airway pulmonary malformation (CAPM), congenital overinflation, bronchopulmonary sequestration, and bronchial atresia. The presenting group consists of 13 newborns who were admitted to the Neonatal Department of Intensive Medicine (NDIM) during January 1st 2015-December 31st 2019 (8 males, 5 females, 2 premature/11 term newborns, spontaneous delivery: 2, caesarean section: 11) with positive prenatal diagnosis of CAPM in all cases. In 2 cases prenatal intervention was performed (drainage of the amniotic fluid, attempt of thoracentesis). Signs of acute RI immediately after delivery were seen in 5 newborns. Postnatal echocardiographic investigation confirmed the presence of increased pulmonary pressure in 8 patients, no patient had congenital heart abnormality. A thorax x-ray was positive also in asymptomatic patients. Computed tomography in patients brought detailed information about the position, size and character of CAPM. Six patients underwent surgery. In 15.4 % right lungs were affected by cystic malformation and in 23 % left lungs were affected. A final diagnosis of CAPM was confirmed in 5 patients using histopathologic examination. Multidisciplinary cooperation during prenatal as well as postnatal period is necessary.

Laparoscopic treatment of bowel perforation after blunt abdominal trauma (BAT) in children.

Minimally invasive techniques have now become standard for the treatment of many surgical conditions in children. There are a few studies that describe the utility of laparoscopy in BAT in children. In this article, we describe the complete laparoscopic surgical treatment of two patients after a single blunt abdominal trauma, both with bowel perforation. In both cases, the perforation was identified and closed, one laparoscopically with an ongoing suture, the second jejune perforation was closed by laparoscopic-assisted techniques. Both patients had an uneventful postoperative recovery. Therapeutic laparoscopic treatment of patients with upper gastrointestinal perforation is feasible. We hypothesize, that diagnostic laparoscopy provides important information for the treatment of children with abdominal trauma and is accompanied by improved diagnostic accuracy, reduction of nontherapeutic laparotomy rates, and a reduction of morbidity. Minimally invasive surgery in children after BAT is suitable for hemodynamic stable patients, could improve pain scores, cosmetic effect, shorter hospital stays, shorter operative times and shorter return to school/activities. However, at any point in the patients care, in case the unstable hemodynamic is encountered, exploratory laparotomy is the procedure of choice.Key words: miniinvasive surgery blunt abdominal trauma - laparoscopy bowel perforation.

An embryological point of view on associated congenital anomalies of children with Hirschsprung disease.

The most common congenital gut motility disorder is the Hirschsprung disease (HSCR). This anomaly is characterized by absence of neural crest-derived enteric neuronal ganglia. The aim of our study was to analyze the relationship between HSCR and other congenital anomalies or malfunctions. We examined 130 patients with Hirschsprung disease from Slovakia for last 10 years. During patients examination we focused not only on morphological abnormalities, but also functional anomalies. The incidence of associated congenital anomalies in our patients with HSCR was 26.1 %. But if we add functional defects (hypothyroidism, malfunction in cellular immunity, neurological deficit) to the morphological congenital abnormalities, the rate of the patients with HSCR with additional defects achieves 50.1 %. Nine of our patients (6.9 %) had syndromic HSCR. The most frequent disorder (13.6 % of patients) was primary deficiency in cellular immunity. More than 12.3 % of patients with HSCR had genitourinary abnormalities, in 10.0 % of patients variable degree of psychomotor retardation was observed, and skeletal, muscle and limb anomalies involved 7.7 % of patients. In 7.6 % cases of patients we found congenital hypothyroidism (including 2 cases of agenesis of thyroid gland). More than 6.1 % of patients presented with an associated anomaly in gastrointestinal tract (mostly anorectal malformations). Up to 5.5 % patients had congenital anomaly of heart, 3.8 % had ophthalmic and 3.1 % had craniofacial anomalies. Down syndrome was the main diagnosis in 3.8 % patients. We discussed  the relationship between HSCR and other anomalies, which are probably caused by abnormal migration, proliferation, or differentiation, of neural crest cells during embryogenesis (Tab. 1, Fig. 2, Ref. 75).

Laparoscopic cholecystectomy in children.

UNLABELLED: In 95 children (age 6-18 y) treated for gallstones between 2002-2010, 95 laparoscopic cholecystectomies were carried out. Symptomatic cholecystolithiasis was the indication for laparoscopic cholecystectomy in most of the cases. In three patients, there was a conversion to open cholecystectomy. Postoperative complications due to failure of bile drainage arose in three patients. Restored bile drainage was secured for two biliodigestive anastomosis. Bile leak in a third patient solved a temporary biliary stent. Laparoscopic cholecystectomy is the gold standard for gallstones (Ref. 8). KEYWORDS: gallstones, laparoscopic cholecystectomy, cholecystolithiasis, bile drainage.

[Laparoscopy in the management of invagination in pediatric patients]. / Miesto laparoskopie v liecbe invaginácie u detí.

AIM: During the last decade, many articles on indications, contraindications, benefits and risks of laparoscopic desinvagination procedures in children have been presented in scientific literature. However, this therapeutic procedure has become a standard therapeutic option within the range of pediatric laparoscopic precedures. The aim of the authors was to establish this miniinvasive procedure in their clinic. PATIENTS AND METHODS: From January 2006 to December 2009, the authors operated 19 patients with ileocolonic invaginations, in whom prior hydrostatic desinvagination had failed. 11 pediatric patients underwent laparotomic revision. In 9 subjects, idiopatic invagination was detected, while in 2 subjects secondary intususception caused by a Meckel's diverticle was confirmed during the procedure. In 7 subjects aged 8-51 months, desinvagination was achieved using laparoscopic procedures. 3 ports were used during the procedures--one for optic devices, measuring 10 mm, was placed in the infraumbilical incision and 2 working ports of 5 mm were introduced into the left hypogastrium and the left mesogastrium. The desinvagination was achieved using a grasper and gentle traction on the invaginated colon. In two subjects with relapsing invagination, desinvagination was followed by ileo-coecopexy. In one subject, conversion was required during the laparoscopic procedure, because repositioning of the invaginated colon was not feasible. OUTCOMES: The mean duration of these laparoscopic procedures was 68.6 minutes and no intraoperative complications were recorded. Reallimentation was introduced on the first postoperative day, no reinvagination episodes were recorded and the mean duration of hospitalization was 4.3 days. CONCLUSION: Laparoscopic desinvagination has become a part of the algorithm for the management of invaginations in cases, where conservative approach failed, and should be used before open laparoscopic revision is indicated. However, the miniinvasive procedure must be performed by a surgeon with experience in laparoscopy.

Devastating injury of the groin with vascular lesion.

A 13-year-old girl suffered an injury of the left inguinal region in October 2002. The mechanism of the injury was a fall off a merry-go-round with subsequent trailing by the iron frame, which resulted in devastation of the soft tissues and vessels of her left groin. The patient was transported to the Department of Paediatric Surgery in a state of haemorrhagic shock. A part of the iron frame was still sticking out of her groin. Emergent surgery and haemostasis was followed by reconstructive vascular surgery. Vascular ischaemic-reperfusion syndrome appeared 8.5 hours after the injury. A second episode of haemorrhagic shock developed on the 6th post-operative day, after a dislodgement of an arterial thrombus from a small artery, which was not obvious during the surgery. The situation required emergent wound exploration and surgical control of the bleeding. A third episode of haemorrhagic shock developed on the 9th post-operative day, after an excessive administration of heparin. This time no surgical bleeding was detected during wound exploration. The last episode of haemorrhagic shock appeared 6 months later, during the patient's menarche. She was ashamed to tell her parents about her heavy bleeding for several days. Authors of this article conclude that it is not necessary to increase the dose of Heparin above 20 mcg/kg/hour, even though the value of aPTT is not as high as we would expect. We should expect also the possibility of severe bleeding due to menarche on the anticoagulant therapy in adolescent female patients. Authors also consider both arterial and deep venous reconstructions to be adequate methods of treatment in case of extensive devastating injury of the groin region in the adolescent period (Fig. 5, Ref. 5). Full Text (Free, PDF) www.bmj.sk.

[Later age diaphragmatic hernia]. / Diafragmatická hernia manifestovaná v neskorsom veku.

INTRODUCTION: Congenital diaphragmatic hernia (CDH) is usually diagnosed antenatally or through the neonatal period at present. It is also used to treat and manage CDH in this date. However in rare cases CDH could be presented after finished neonatal period with non-specific and misleading symptoms. The aim of our presentation is to analyze the patients with late - presenting and diagnosed CDH. METHODS: During period 1996-2006 we operated on 54 children with CDH, 12 of them with late - presenting CDH, age variance was 9 months and 6 years. The spectrum of symptoms was from asymptomatic CDH (2 patients), mild respiratory (4 patients) and gastrointestinal symptoms (4 patients), combination of respiratory and gastrointestinal symptoms (2 patients) and finally the symptomatology of bowel incarceration (2 patients). RESULTS: Seven children presented with another associated malformations (m. Down, congenital heart failure, hydrocephalus, anorectal atresia). All 12 patients were operated on with a good results. The operation was composed of reposition of the herniated part of gastrointestinal tract (without need of resection) and diaphragmatic plastic. Two patients were reoperated on - once because of diaphragmatic relaxation and the second one due to consecutive heart operation. CONCLUSION: Congenital diaphragmatic hernia could be presented with chronic and non-specific respiratory or gastrointestinal symptoms. Time to time, presentation of diaphragmatic hernia as "acute abdomen" could be seen. The operation is indicated, also in asymptomatic patients as a prevention of life-threating events.

[Occlusive ileus caused by intestinal duplication]. / Obturacný ileus spôsobený crevnou duplikatúrou.

State with progressive development of intestinal obstruction may leads to the misinterpretation of acute gastroenteritis in infant period. After passing of newborn period the differential diagnosis is not easy. Loss of time which leads to the correct diagnosis is ufavourable for patient because of worsening of the condition. The authors present a case review of 5 months old girl in whom cystoid intestinal duplication caused complete ileo-coecal occlusion. Associated diagnosis in our patient were anus perinealis ventralis and diverticulum Meckeli. The treatment of enteral duplication and Meckel's diverticulum was done in one stage surgery.

Perinatal traumatism with gastroschisis.

Authors analyse 11 cases of perinatal traumatism in a group of 110 patients with gastroschisis during a period of 25 years. They point out its causes and consequences. To minimize perinatal traumatism, it is necessary to conduct suitable prenatal and perinatal management. In spite of preferring Caesarean section there are no differences between vaginal and Caesarean delivery when the delivery is properly managed (Tab. 1, Fig. 3, Ref. 12). Full Text (Free, PDF) www.bmj.sk.

[Exchange amnioinfusion in conceptus with laparoschisis (first experience)]. / Výmenná amnioinfúzia u plodov s laparoschízou (Prvé skúsenosti).

OBJECTIVE: During amnioinfusion exchange (AE) a certain amount of amniotic fluid is repeatedly extracted and the same amount of physiological solution is consequently instilled into the amniotic fetal cavity. The aim of this procedure is to dilute the amniotic fluid that surrounds the eviscerated organs of fetuses with laparoschisis so as to avoid the genesis of fibrous coating on these organs. DESIGN: Prospective study. SETTING: Gynekologicko-pôrodnícka klinika SZU, FNsP akad. L. Dérera, Bratislava, Slovakia. METHODS: We have executed AE in five fetuses with laparoschisis since June 2002. Two patients underwent the treatment 2 times during the 32nd and 36th weeks of gestation. Two other patients were treated once during the 32nd week and one patient once during the 36th week. Under ultrasound control we used a spinal needle to extract 120-180 ml of dense, cloudy amniotic fluid. Consequently, we instilled the same amount of physiological solution warmed up to the temperature of 37 degrees C into the amniotic cavity through antibacterial filter. The fetuses were monitored cardiotocographically and with the help of ultrasound flowmetry in umbilical vessels, before and after the treatment. RESULTS: The AE were successful and without complications in all five cases. All patients delivered via elective caesarean section during the 36th - 37th gestation week. One patient delivered 24 hours after second AE due to the danger of intrauterine fetal hypoxia that was verified cardiotocographically. The other patients delivered 1-4 weeks after AE. CONCLUSION: The significance of AE lies primarily in the reduction of the occurrence of fibrous coating on eviscerated organs. It enables postnatal primary surgical closure of the defect in the front abdominal wall, an earlier onset of intestine peristalsis, transition from parenteral to peroral nutrition and shorter hospitalization.

Separation of Siamese twins in Bratislava.

At the pediatric surgical clinic in Bratislava, in the year 2000, we successfully carried out the separation of Siamese twins, which we classified as "ischiopagus tetrapus"+. The pelvic ring, the gastrointestinal tract and the urogenital system were all malformed. There was a common terminal ileum and only a short segment of the large intestine with a blind end. This was a case of non-developed anal segment and rectum. Each of the twins had two kidneys and two commonly shared urinary bladders. There were two uteruses, which were both bicornuate in nature with a fallopian tube arising from each of the horns as well as an ovary. The orifices of both urinary bladders and those of the two uteruses joined into a common urogenital sinus. The external ostia of this sinus represented a bizarre fissure with a single skin fold similar to the labium majus, located dorsally at the point of fusion of the pelvic structures. The act of separation was performed in two stages. Separation of the gastrointestinal tract was urgent due to the severe ileus caused by aplasia of the anus and the rectum. This first stage procedure was performed on the third day of admission (24.2.2000) after the babies had spent a month in other health institutions. The definitive separation was carried out a month after the first procedure (28.3.2000) following the optimal stabilization of the functions of the gastrointestinal tract. The uropoietic and genital systems were separated. A new pelvic ring and a pelvic floor were formed. The anterior abdominal wall was reconstructed and plastic correction of the skin in the gluteal and perineal regions was performed. The colostomies are functioning well and the twins pass well-formed stools. Lucia has long dry intervals between mictions. Andrea has shorter dry intervals. The girls have recently celebrated their third birthday. Their mental development is excellent; they speak Slovak and Hungarian languages. They have a well-developed locomotive apparatus, without any movement impairments. (Tab. 1, Fig. 16, Ref. 10.)

[Maffucci's syndrome--case report of a 5-year-old female patient]. / Maffucciho syndróm--kazuistika 5rocnej pacientky.

Maffucci's syndrome is a congenital non-hereditary syndrome characterized by a combination of dyschondroplasia (enchondromatosis) and haemangiomatosis. It is a rare disease. During the last 140 years only 200 cases were described [5]. 109 cases are described in the literature by other authors [21]. This disease is associated with a high risk of development of neoplastic processes, in particular sarcomatous transformation of enchondromatoses as well as other mesodermal and non-mesodermal malignities [13]. Based on the incidence of spinocellular haemangioendothelioma with other congenital syndromes, the presence at a young age of patients and the multicentric incidence support the assumption that spinocellular haemangioendothelioma may be the manifestation of genetically based mesodermal disease which may be associated with the picture of Maffucci's syndrome [6]. In their case-history the authors present a 5-year-old patient. They describe the clinical course and findings taking into account possible manifestations and risks associated with the disease. With regard to the low incidence of the disease and its interdisciplinary character the authors pen the question of possible causal, symptomatic or palliative treatment.

[A pediatrician's views on the treatment of extensive hemangiomas in childhood]. / Pohlad pediatra na liecbu rozsiahlych hemangiómov v detskom veku.

Haemangiomas are the most frequent tumours of child age. They are found in 10-12% of infants. The authors summarize contemporary knowledge of the treatment of haemangiomas as well as their own experience with their treatment. They follow-up 18 children with haemangiomas at different sites, incl. ten with extensive haemangiomas. The latter were treated conservatively with prednisone and interferon alpha.

[Diastematomyelia and the tethered spinal cord syndrome. Case report]. / Diastematomyélia a syndróm pripútanej miechy. Kazuistika.

The authors present a patient with diastematomyelia and associated congenital malformations of the spinal cord. They caused tethering of the spinal cord. Patient had cutaneous marks of malformation from birth. The congenital malformation was operated at the age 5 years. MRI and surgical findings confirm the existence of diastematomyelia together with thick tethered filum terminale and lipoma of filum terminale. On surgery the bony septum was removed, the filum terminale was resected and the lipoma was excised. Each of the anomalies caused the tethering of the spinal cord and restricted its physiological ascent. Only careful surgical revision with subsequent removal of all causes of the tethering of the spinal cord produced good result. Diastematomyelia is a congenital malformation with some morphological variations and coexistence of other malformations. The presented case is a typical example of malformations associated with diastematomyelia.

[Supracondylar fractures of the humerus and disorders of circulation]. / Suprakondylická zlomenina humeru s poruchou cirkulácie.

Supracondylar fractures (SF) of the humerus are the third most common type of fractures in childhood. The highest rate of pulselessness fracture of the distal humerus is the extension-type. The aim of our treatment is to minimize potential complications. Our management of pulselessness supracondylar fracture is more aggressive compared with other dpts. 223 (100%) patients with SF were treated at the dpt. of Paediatric Surgery, University Children's Hospital in Bratislava, in the 5-year period of (1995-1999). 11 (4.93%) patients remained pulseless after reposition of bone fragments. These patients underwent surgical revision of the brachial artery. Spasms of the brachial artery (BA) occurred in six patients, a lesion of BA in three patients and thrombosis of BA in two patients. We performed revision of the brachial artery by a Fogarthy catheter in six patients--in four cases with thrombectomy. In two cases we released the vessel from bone fragments. In two patients was replaced the injured segment of the brachial artery by a venous allotransplant. Peroperative angiography was performed in six patients. We reoperated three patients with early postoperative complications. We did not record any case of Volkmann's contracture.

[Personal experience in the treatment of medial cervical cysts in children]. / Nase skúsenosti s liecbou mediálnych krcných cýst u detí.

Twenty-five patients with thyreoglossal cysts were treated during a 7 years long period. Most of the cysts (in 64 of patients) were typically located between the hyoid bone and the thyreoid gland. The complete Sistrunk's operation was performed in 40% of the patients and 48% patients underwent an extirpation of thyroglossal cyst with a resection of the hyoid bone body. Three of the patients (12%) suffered a relapse. In one of the patients, this was due to the lateral cyst swapping and in two patients an incomplete extirpation of the ductus thyreoglossus remnant happened. In another patient, an iatrogenic hypothyreossis occurred. To avoid relapse, authors recommend to resect at least 10 mm of the hyoid body and 5 mm of the ductus thyreoglossus remnant behind the hyoid bone.

[Treatment of blunt injuries of the spleen in children]. / Liecba tupého poranenia sleziny v detskom veku.

The authors report study of patients with blunt splenic trauma treated at Paediatric Surgery Department in years 1991-1999. During 9 years period 50 patients (36 males, 14 females) with demonstrated rupture of the spleen were analyzed. 46 patients (92%) were treated conservatively (group A). Complications appeared in 7 patients (15.22%), rebleeding in two patients, arterio-venous fistula in early posttraumatical period in one patient and during control in one patient, fluidothorax in two patients and posttraumatic pseudocyst of the spleen in one patient. Four patients (8%) were operated (group B). Two of them submitted splenectomy, one patient was treated by splenorrhaphy and one patient by partial splenectomy. Period of the hospitalization in average lasted in group A 12,67 days, in group B 15 days. With regard to the immunologic functions of the spleen especially in childhood authors prefer conservative treatment, in case that laparotomy is inevitable authors try to preserve the spleen.

[Subacute appendicitis in children]. / Subakútna apendicitída u detí.

Over the period of 4 years from 1993 to 1996 the authors recorded a 6% incidence of patients with periappendicular mass (PM) in a group of 786 patients with acute appendicitis. The aim of the study is to compare the two methods in the management of patients (operation-A, conservative treatment-B) by applying the same criteria. In the subacute phase of the disease 22 patients (group A) were operated on, while 24 patients (group B) were successfully treated conservatively, with recommendation of elective appendectomy after 6 months. In group A indication for operation was PM in 7 patients, diagnostic problems (tumour, hydronephrosis) or complications (peritonitis, intestinal obstruction) in 15 patients. Antibiotic therapy was nearly the same in both group. Peritoneal drainage in postoperative care was performed in 10 patients of group A for 4.7 days on the average. In this group, secondary wound healing was recorded in 3 patients. In group B, elective appendectomy was performed only in 12 patients (50%). In 2 patients, however, a diagnostic error was revealed, i.e. Crohn's disease and an ovarian cyst had been suspected to be a periappendicular mass. Conservative treatment with subsequent elective appendectomy after 6 months seems to be an effective method in the treatment of patients with a typical clinical picture and well bordered periappendicular mass. Appendectomy in the subacute phase of the disease appears to be a safety technique of PM treatment in patients with complications or diagnostic problems.