Anterior diaphragmatic hernias in children: long-term surgical outcomes

Anterior diaphragmatic hernia (ADH) is a rare congenital pathology that occurs in children with a frequency of 1:4800 (1–6% of all congenital diaphragmatic hernias). There are many controversial aspects in the surgical treatment of patients with ADH: the choice of surgical approach, the method of diaphragmatic repair and the feasibility of excision of the hernial sac. Objective. To conduct a comparative analysis of the surgical treatment of patients with ADH in different clinics, assessing longterm outcomes. Patients and methods. The medical records of 7 children with ADH who underwent surgical repair in different clinics (in time period from 2009 to 2019) were retrospecively reviewed. Evaluating the long-term results of ADH repair was made by telephone and online surveys of the parents of patients and by outpatient examination of children (chest x-ray in two projections). Results. In a ten-year period, 7 patients (4 boys and 3 girls) were operated on with a diagnosis of “anterior diaphragmatic hernia” at the age of 3 months to 12 years. In most children, a hernia was discovered accidentally by chest x-ray. Laparoscopic correction was performed in 5 (71%) cases, thoracoscopic correction – in 2 cases (29%). The main difference in surgical tactics in ADH patients was the manipulation with the hernial sac – the hernial sac was excised in 4 (57%) patients, but it was left in three cases (43%). The defect closure was performed by “full-thickness” separated sutures that fix the diaphragm to the anterior abdominal wall during laparoscopy (5) and to the chest tissue during thoracoscopy (2); in some cases, additional fixation to the rib (4) was performed. Sutures were tied extracorporeally and buried in the subcutaneous layer in 6 (86%) patients. Average follow-up was 7 years. While evaluating long-term outcomes no ADH recurrence were found. Conclusions. There are still many controversial aspects in the surgical treatment of ADH patients. In our opinion, multicenter studies with complex analysis of long-term results are required to standardize the surgical treatment of such patients. Key words: anterior diaphragmatic hernia, Larrey hernia, long-term outcomes, Morgani hernia

Current aspects of the development and progression of fluoride-related chronic kidney disease

Difficulties associated with the identification of a major risk factor for chronic kidney disease (CKD) (mainly in children without obvious anatomical causes), long-lasting asymptomatic phase, and irreversible kidney damage caused by chemical elements resulted in a particular interest to the problem of diagnosis and timely treatment of CKD. This review aims to summarize available information on the role of increased fluoride concentration in the development of CKD of uncertain etiology. We included more than 200 publications found in Scopus, Web of Science, Cochrane Library, and PubMed. We analyzed factors associated with an increased risk of fluoride-related CKD and identified possible mechanisms underlying disease progression. In addition to that, we proposed potential molecular markers to detect early stages of CKD. We described new promising therapeutic targets with the consideration of key elements of disease pathogenesis, poor prognosis, and age limits for existing nephroprotective drugs. Key words: fluoride, chronic kidney disease, risk factors, pathogenesis, molecular markers

Management strategy for children with dentoalveolar disorders against the background of chronic kidney disease

According to the European Dialysis and Transplantation Association (EDTA), the incidence of chronic kidney disease is 4–6 cases per million children under the age of 15 and it increases with the age of the child. In order to optimize dental care for children with chronic kidney disease (CKD), an algorithm for dental treatment and prevention was developed. A questionnaire was conducted among 57 children with CKD and 57 parents of this group of children to assess parental and child knowledge of the basics of preventive measures for kidney diseases. Based on the results of the questionnaire, nutritional recommendations were developed. It was found that 100% of parents know the basic nutritional guidelines for children with CKD. Particular attention was paid to children’s consumption of food containing salt. All parents surveyed (100%) were convinced that their children do not consume salty foods, while 23.1% of children claimed to consume them 3 times a day. Thus, it is advisable to implement the developed complex method of treatment and prevention of dental diseases with the inclusion of sanitary and hygienic education on the issues of nutrition for patients with CKD in order to further prevent not only recurrence, but also the progression of existing pathology. Key words: children, nutrition, dental care, chronic kidney disease

Difficulties associated with the diagnosis of tuberculous osteomyelitis of the ribs in children

Diagnosis of tuberculosis in children is challenging because of variable clinical symptoms and no specific signs. We report a case of tuberculous osteomyelitis of the ribs, which demonstrates difficulties associated with the diagnosis of generalized tuberculosis in an 8-year-old child (correct diagnosis was established after 1.3 years). Diagnostic procedures included: Mantoux test, skin test with recombinant tuberculosis allergen, computed tomography, pathomorphological and bacterioscopic testing. The patient presented with disease progression and lesions to the lung tissue, pleura, and ribs and did not respond to antibacterial therapy, hormones, and cytostatics. This required repeated diagnosis revision. The diagnosis of tuberculosis of the right 4th and 5th ribs was confirmed after surgery using bacterioscopic and immunohistochemical examinations. The child received comprehensive treatment in a specialized tuberculosis hospital and had positive dynamics. Despite the correct routing, low suspicion for tuberculosis in primary and secondary healthcare institutions has led to an insufficient use of currently available diagnostic methods and late diagnosis of tuberculosis in a child residing in a megapolis. Key words: generalized tuberculosis, children, tuberculosis of the rib, immunodiagnostics, diagnosis of tuberculosis, immunohistochemistry

Impact of cleft palate repair on speech and development of dentoalveolar deformities

Surgical repair of cleft palate is quite difficult because it aims not only to eliminate the anatomical defect of the palate, but also to ensure normal functioning, including speech. Moreover, successful surgery implies no or minimal deformation of the middle face that can be corrected in the late postoperative period. No doubt that primary surgery (both in terms of technique and time) is crucial for further growth and development of the maxilla. However, surgical techniques and the age of primary cleft palate repair vary between different clinics, which makes this literature review highly relevant. Key words: cleft palate repair, cleft palate, congenital cleft lip and palate

Marfan syndrome in combination with severe atopic dermatitis: importance of early diagnosis and treatment of patients with allergic dermatitis in childhood

We report a case of a rare combination of Marfan syndrome and severe atopic dermatitis (AD) in a 32-year-old male patient. He developed AD in early childhood and then had a relapse at the age of 18 years caused by stress. After this, AD became severe due to stress associated with aortic aneurysmectomy. The patient also had side effects caused by steroids, including multiple pustular eruptions, Kaposi's varicelliform eruption, edema and hyperemia of the periorbital area, pain and swelling of the small joints of the hands. These side effects, as well as specific cardiovascular symptoms typical of Marfan syndrome necessitated the search for alternative treatment strategies for severe AD. Eventually, we managed to find an effective treatment regimen that caused no side effects or adverse events, while the symptoms subsided. This case demonstrates the importance of early AD diagnosis in childhood in order to prevent its transition into severe chronic form that can hardly be managed in adulthood. Key words: atopic dermatitis, children, diagnostic criteria, clinical manifestations, hereditary diseases, Marfan syndrome

Nocturnal hyperalimentation via a gastrostomy tube in children with cystic fibrosis

Objective. To assess the efficacy of nocturnal hyperalimentation via a low-profile gastrostomy tube for in the improvement of nutritional status and lung function in children with severe cystic fibrosis (CF). Patients and methods. We used nocturnal hyperalimentation with a nutrient solution via a low-profile gastrostomy tube installed under endoscopic control to improve nutritional status of 16 CF children with severe protein-calorie malnutrition aged between 5.5 and 17.5 years. The mean duration of nocturnal hyperalimentation was 1.3 years (range: 7 months–54 months). The nutritional status was evaluated using the WHOAntho Plus software; pulmonary function was evaluated by assessing forced expiratory volume in 1 minute (FEV1) and the number of bronchopulmonary exacerbations per year. Results. All children demonstrated an improvement in their weight and height. After the first 12 months, the mean body weight gain was 5 kg; during the second and third years, children gained 4 kg and nearly 2 kg, respectively. The mean increase in height was 7 cm in the first year, 10 cm in the second year, and 4 cm in the third year. All patients except one had an increase in FEV1 and fewer exacerbations. Complications after gastrostomy were rare and easily addressed. Conclusion. Nocturnal hyperalimentation via a percutaneous endoscopic gastrostomy tube is an effective and safe method of improving nutritional status in children with CF, which delays the progression of bronchopulmonary lesions and lung function reduction, improves the prognosis and quality of life. Key words: cystic fibrosis, children, nutritional status, lung function, enteral nutrition, low-profile gastrostomy tube, nocturnal hyperalimentation

Clinical manifestations of Williams syndrome in children

Williams syndrome (WS), also known as Williams–Beuren syndrome, is a rare genetic disorder affecting many organs and caused by hemizygous deletions of the long arm of chromosome 7 (7q11. 23). In this article, we describe typical pathological changes in the cardiovascular, endocrine, urinary, digestive, and nervous systems, as well as phenotypic characteristics and psychological aspects of this disease. We also cover a correlation between clinical manifestations of WS and genes in the remote area. The article contains a summary of the main recommendations for the treatment of individual manifestations, i.e. pathological conditions associated with this syndrome. Key words: children, Williams syndrome, cardiovascular disorder, hypercalcemia, hypothyroidism, growth retardation, mental retardation

Variants of surgical treatment of chronic post-intubation laryngeal stenosis in children

Objective. To evaluate the effectiveness of a surgical tactics used for the treatment of children with chronic post-intubation laryngeal stenosis depending on the condition of their laryngeal cartilages. Patients and methods. We treated 47 children with chronic post-intubation laryngeal stenosis. Preoperative examination included endoscopy and computed tomography (CT) of the larynx and trachea. We evaluated the condition of their laryngeal cartilages in order to choose an optimal surgical tactics. Children with intact laryngeal cartilages (n = 20) have undergone endolaryngeal microsurgery using a CO2 laser. This method caused minimal injury due to submucosal excision of scar tissue, which enabled wound coverage with mucous microflaps to prevent recurrent stenosis. Surgery for extensive stenosis in the middle portion of the larynx was associated with a risk of recurrent scarring. In our study, we prevented it by using endografts during 21 days. However, patients with lesions in the subglottic larynx still had a high risk of recurrent stenosis (5 out of 12 patients) even if there was no evidence of damage to the laryngeal cartilages. Results. Twenty-seven patients with laryngeal post-intubation stenosis with lesions to the laryngeal cartilages detected before surgery have undergone extralaryngeal laryngotracheoplasty. Eighteen children have undergone laryngofissure with subsequent stenting between 6 and 18 months with a T-shaped silicone stent. Nine patients have undergone single-stage laryngotracheoplasty with autologous grafts. Conclusion. The methods used in this study have demonstrated high effectiveness, while the use of grafts reduced the number of stages of surgery and its duration. Key words: laryngotracheoplasty in children, laryngeal microsurgery, complications of tracheal intubation in children, chronic laryngeal stenosis, cartilage autologous grafts, CO2 laser

Idiopathic coccygodynia as a ‘mask’ of lesions caused by overload in sports associated with the art of movement

In this study, we analyzed specific manifestations of idiopathic coccygodynia in children and adolescents involved in rhythmic gymnastics and choreography. The experimental group consisted of 34 individuals aged 10 to 14 years with complaints of discomfort and/or pain in the pelvis, sacrum, coccyx, and hip joints, increasing during professional movements and who had no history of injuries. The control group comprised 12 healthy adolescents from the same teams. We evaluated local muscle hypertonia, pain severity (using a visual analog scale) in all study participants. In addition to that, they have undergone sports and choreographic testing and isokinetic examination (using the multiarticular complex ‘Biodex System 4 Pro’). Individuals in the experimental group had a 2-week rehabilitation course that included modification of training; intramuscular injections of 2.0 mL of 0.1% lidocaine solution into the area of muscle hypertonia; application of ointment (2.2 mL); manual therapy; special exercises to relax spasmed muscles). We identified significant difference in pain severity when performing basic choreographic elements between the two groups in the beginning of the study. We also observed a significant decrease in pain in the experimental group after the rehabilitation course. We obtained an indirect proof of the hypothesis that local muscle hypertonia is the leading cause of discomfort and pain. The results of isometric examination suggest that the number of patients with a bilateral difference (according to two of the three parameters studied) significantly differed between participants in the experimental group after a course of physical rehabilitation and controls. This fact can serve as an indirect evidence that hypertonia affects functional activation of muscles, and a 2-week course of physical rehabilitation is not sufficient. The introduction of the concept of ‘sports coccygodynia’ will allow orthopedic traumatologists and sports medicine doctors to focus on several therapeutic and diagnostic measures and to avoid unnecessary instrumental examination, which will significantly increase the effectiveness of patient rehabilitation. Key words: children and adolescents, muscle hypertonia, coccygodynia, lesions caused by overload, choreography, rhythmic gymnastics