Treatment of the congenital thoracic deformity pectus excavatum.

Pectus excavatum is the most common chest wall deformity in the Czech Republic. This chest deformity is typically characterized by a wall depression with sternal rotation. If the excavation of the chest wall does not cause any physical or psychological problems, the patient does not need any specific treatment. However, if the deformity is painful, affects the function of the lungs, heart or results in psychological problems, we can propose an appropriate treatment for the specific age category of the patient. Up to 10 years, we choose a procedure that includes targeted exercises and rehabilitation; in the age group of 10-15 years, we can add to the exercises the vacuum bell therapy according to the patient's wishes and compliance; and in the age category of 16 years and above, the patient can be offered a surgical solution. The Nuss operation (so-called MIRPE - minimally invasive repair of pectus excavatum) is the gold standard in surgical treatment; during this surgery, a patient-shaped bar is inserted retrosternally into the patient's chest under thoracoscopic control and is left for 3 years. The aim of this article is to describe the most common modern methods used in the treatment of patients with pectus excavatum, supplemented by a historical overview.

Technical innovation in minimally invasive repair of pectus excavatum.

The aim of study was to introduce technical innovation of MIRPE which reduces the risk of cardiac injury. Modification of MIRPE method with semiflexible thoracoscope and sternum elevating technique has been used. Volkmann bone hook has been inserted percutaneously to the sternum. The hook elevates the sternum forward and enlarges the retrosternal space for safer passage of thoracoscopically guided introducer. Using semiflexible thoracoscope allows better view from various angles via one site of insertion. During the period 2005-2012, the MIRPE was performed on 29 girls and 151 boys; the mean age at the time of surgery was 15.9 years (range 13-18.7 years). The mean Haller index was 4.7 (range 2.7-20.5). The most common complication was pneumothorax (3.3 %) and the incidence of bar displacement was 2 %. The most serious complication was cardiac perforation when inserting Lorenz introducer. This occurred in a 16-year-old girl; she required urgent sternotomy with right atrial repair and recovered well. External elevation of sternum with the hook was used since this case. Subsequent 113 patients underwent surgery without any serious complications. Technical innovation using semiflexible thoracoscope and hook elevation of the sternum reduces the risk of cardiac injury. The hook opens the anterior mediastinum space effectively and makes the following dissection relatively safe and straightforward.

[Surgical technique changes in the repair of pectus excavatum between 1991 and 2012]. / Zmeny chirurgické techniky pri resení Pectus excavatum v letech 1991-2012.

INTRODUCTION: The aim of this article is a retrospective analysis of the results of three methods used for the repair of pectus excavatum between 1991 and 2012. MATERIAL AND METHODS: 508 patients were operated on at the Department of Paediatric Surgery of the Second Faculty of Medicine, Charles University, and the Teaching Hospital in Motol between 1991 and 2012. 16 patients were older than 19 years (19-28 years). The average age of paediatric and adolescent patients was 14.2 years (5-19 years range). Boys (408) prevailed over girls (82), the ratio being 4.9:1. Open Holcomb technique was used to operate on 87 patients (17%), the Welch method was applied in 208 patients (41%). Minimally invasive repair of pectus excavatum (MIRPE) was used in 213 patients (42%) during the entire period of time. RESULTS: As for the 295 patients operated on by the open Holcomb and Welch method, the outcome was excellent in 239 (81%) of them. A favourable result with a small deformity remaining occurred in 44 patients (15%). Unfavourable result was found in 7 patients (2.5%): in these children, a deep excavation occurred 3-5 years postoperatively. In 5 patients (1.5%) we evaluated the result as recurrent chest wall deformity. The minimally invasive method (MIRPE) produced an excellent result in 182 out of 213 patients (85%). A favourable outcome was observed in 27 (22%), unfavourable result in 1 (0.5%) patient. CONCLUSION: In the last 15 years, minimally invasive repair of pectus excavatum has become popular worldwide. Due to very favourable cosmetic results and a relatively small, gradually decreasing number of complications, the proportion of patients requiring surgery to correct their deformity has risen sharply.

[Stab wounds in children]. / Bodná poranení u detí.

Injuries are the most serious health risk in children. Injuries are the main cause of death and long term disabilities in children. Fortunately, stab wounds in children are very rare in our country, but they are, in most cases, very serious injuries. Presenting 3 case reports, this article aims to show the danger of stab wounds and how easily sharp objects can penetrate human tissue. In two out of the three case reports, a foreign object was remained inside the wound. When dealing with such injuries, this possibility must be considered the wound must be carefully inspected. When a revision is necessary, it should be carried out using minimally invasive methods in order to save the child from suffering extensive wounding which would result from the classical open approach revision.

Lipoblastoma in children: an analysis of 5 cases.

Lipoblastoma is a very rare benign tumour that is caused by embryonal fat. The present five cases of lipoblastoma operated on during the years 1996-2005. The localization of the lipoblastomas in our series were very unusual. A six-monthold girl with giant mediastinal lipoblastoma; a two-year old boy with very rare lipoblastoma of the kidney; a three-year old boy with mesenterial lipoblastoma; a seven-year old boy with mesenterial lipoblastoma; and an eight-week old girl with perineal localization. Histological diagnosis can be difficult. The basic differential diagnosis is to be made between lipoblastoma, myxoid, and round cell liposarcoma. In our sample group of patients all lipoblastomas were successfully and completely removed and we did not see any recurrence of the tumours. In only one case was more radical surgery needed. One patient with mesenterial lipoblastoma had to undergo a 30 cm long resection of the small intestine.

[Surgical complications following oesophageal replacement by stomach in childhood]. / Chirurgické komplikace po náhrade jícnu zaludkem v detském veku.

AIM: Assessment of surgical complications and options for their therapy following replacement of oesophagus by stomach. METHODOLOGY AND MATERIAL: A retrospective study of clinical and pathological data collected from 25 children, who underwent replacement procedures of their oesophagus by stomach during the 1992-2005 period. 18 subjects were indicated for the procedure for oesophageal atresia with large stubs distance and 7 subjects were indicated for extensive oesophageal strictures following acid burns. RESULTS: The study group included 25 children whose mean age at the time of the procedure was 12.5 years (the age range 8 to 13.3 years). Early postoperatively, a fistule in the oesophageal anastomosis region was confirmed in 7 subjects (28%), however, in all cases it healed successfully on conservative management. In five cases (20%), a stricture in the pyloric region developed and in 4 cases it was managed using balloon dilation. During the long-term follow up period, oesophageal anastomosis strictures were diagnosed in 9 children (36%). The problem was managed using repetitive balloon dilations. One child was operated for ileus with adhesions and in one child, a nutritional jejunostomy had to be re-conducted. One girl exited at home, a year following the procedure, due to respiratory arrest on aspiration. CONCLUSIONS: Transposition of the stomach is a suitable method of choice for oesophageal replacement in childhood, providing good quality of life for majority of patients. Early and late surgical complications correspond with a degree of seriousness of the disorder and require treatment and long term follow up to be conducted in a specialized clinic.

[Morgagni diaphragmatic hernia in childhood]. / Morgagniho bránicní kýly v detském veku.

The aim of this work was to retrospectively assess clinical and anatomical features of children with Morgagni congenital diaphragmatic hernias. The study group included 5 boys and 3 girls, aged between 5 months and 13 years, at the time of the diagnosis. Morgagni diaphragmatic hernia was diagnosed with non-acute symptoms in 8 children. A retrosternal defect of the diaphragm was left-sided in 7 subjects and bilateral in 1 boy. A hernial sac was found in 6 children. In 3 subjects, the hernial sac contained the liver, in 2 subjects the omentum, in 2 subjects the transverse colon and small intestinal loops and in one child it contained the transverse colon with the omentum. In all cases, the diaphragmatic defect was closed using primary plasty. All subjects healed without complications. A differential diagnosis of congenital Morgagni hernia must be considered in children with unusual respiratory and gastrointestinal symptoms with abnormal x-ray findings on chest examination. A stricture of the congenital Morgagni diaphragmatic hernia is rare, and was not recorded in our group.

[Trichobezoar-Rapunzel syndrome--case report]. / Trichobezoár-Rapunzelin syndrom--kazuistika.

Bezoars consist of swallowed foreign materials or indigestible organic matter and form a mass in gastrointestinal tract, usually in stomach. Trichobezoars are formed by swallowed hair, they are rare, and occur often in patients with psychiatric disorders. They may present with malabsorption, weight loss, abdominal pain, and signs of gastrointestinal obstruction or even perforation. Gastric trichobezoar with a tail reaching small intestine is called Rapunzel syndrom. Authors present a case of Rapunzel syndrome in a 13-year-old girl with only a short history of mild abdominal pain in who a gastric trichobezoar weighting 700g and measuring 24 x 16 x 10 cm was removed from laparotomy.

[Esophageal reconstruction using the stomach in children]. / Náhrada jícnu zaludkem v detském veku.

The authors present an account of their experience with oesophageal replacement by gastric transposition in 10 children during the period between November 1992 and January 1999. Indications for oesophageal replacement was in seven children atresia of the oesophagus at the age of 9-36 months and in three children (2.5, 3 and 14 years) a long stricture of the oesophagus after caustic injury which did not respond to balloon dilatation. Replacement after caustic injury was made in children aged 2.5, 3 and 14 years. In eight patients the replacement was made without thoracotomy, by blunt resection of the mediastinum, in two patients after caustic injury thoracotomy had to be performed. In three patients after surgery a temporary fistula developed at the site of anastomosis which healed spontaneously. Eight patients are in a satisfactory condition after surgery, one patient five months after operation swallows with difficulty, his birth weight was 1500 g. One female patient died one year after oesophageal replacement, apparently due to aspiration and respiratory arrest. Gastric transposition is a considerate and relatively simple method in child age. The functional results are favourable.

Long-term outcomes of balloon dilation of esophageal strictures in children.

RATIONALE AND OBJECTIVES: The purpose of this study was to determine the long-term success of the use of angioplasty balloons for dilation of esophageal strictures in children. MATERIALS AND METHODS: The authors studied 24 children who had undergone fluoroscopically guided angioplasty balloon dilation of esophageal strictures. Ten children had stricture of the anastomosis after surgical repair of esophageal atresia; five had achalasia; three had strictures due to gastroesophageal reflux; three had corrosive strictures; two had congenital stenosis; and one had stricture after radiation therapy. Success was defined as resolution of the dysphagia a year after dilation. RESULTS: A year after the last dilation, 15 of the 24 children had no dysphagia. Patients in whom treatment was successful included eight children with strictures after surgery for esophageal atresia, one with achalasia, three with strictures caused by gastroesophageal reflux, one with a corrosive stricture, one with congenital stenosis, and one with a stricture resulting from radiation therapy. CONCLUSION: The long-term results of balloon catheter dilation are highly successful in patients with stricture after surgical repair of esophageal atresia and stricture due to gastroesophageal reflux. Dilation is not as valuable in the treatment of esophageal strictures resulting from other causes.

[Late results after surgical treatment of gastroesophageal reflux in childhood]. / Pozdní výsledky po chirurgickém lécení gastroezofageálního refluxu v detském veku.

The authors present their long-term experience with surgical treatment of gastrooesophageal reflux (GER) in childhood. During the period between 1984-1993 they operated 243 children from neonatal age to 18 years. In 241 patients Nissen fundoplication was performed, once Thal's operation and once a modification of Boix-Ochoy's operation. Of 243 children 18 children (7.4%) died without any association with the operation, 5 children were re-operated on account of ileus, 5 developed a dehiscence of the surgical wound, 4 a paraoesophageal hernia and 2 a relapse of the reflux. The most frequent indication for surgery were relapsing bronchopneumonias 71 (29%), apnoe 44 (18%), impaired nutrition and growth 43 (17%), asthma bronchiale 38 (15%), oesophageal stricture 20 (8%), suffocating laryngitis 14 (6%) and incoordinated deglutition (5%). Two hundred and nine patients attended check-up examinations 3-13 years after surgery. 174 children (83%) do not have clinical manifestations of GER and no difficulties with swallowing. 27 children (13%) report marked improvement of the clinical manifestations of GER or slight difficulties when swallowing. Nissen fundoplication was a success in 96% children with GER. The authors recommend Nissen fundoplication for the surgical treatment of GER in child age because the long-term results with this method are favourable.

[Hematologic indications for splenectomy in childhood]. / Hematologické indikace k splenektomii v detském veku.

In 1988-1994 at the Pediatric Surgical Clinic, Faculty Hospital Motol splenectomy was performed in 66 children. Indication for splenectomy were haematological diseases (52), malignant and benign tumours (12) and congestive splenomegaly associated with cytopenia (2). The group does not comprise patients operated on account of injury. The most frequent haematological indications of splenectomy include haemolytic anaemia, chronic idiopathic thrombocytopenic purpura, hypersplenism and other conditions (haematological malignancies). The investigation is focused on haematological indications of splenectomy in childhood and its effect on treatment of the basic disease. Splenectomy on account of hereditary spherocytosis was performed in 31 patients. In all after surgery normalization of the haemogram was recorded. Of 11 patients, who were operated on account of idiopathic thrombocytopenic purpura (ITP) in nine normalization or marked elevation of the number of thrombocytes was observed. In two patients the rise of thrombocytes was slight however haemorrhagic manifestations of the disease receded. One patient operated on account of thrombocytopenia linked to the X chromosome died. In nine patients operated on account of other haematological indications after splenectomy improved values of the haemogram were observed. The immune state of the patients was not investigated.