Vladimir Ahel

Aortopexy and bronchopexy for the management of severe tracheomalacia and bronchomalacia.

Thre infants with severe tracheomalacia and thre infants with isolated bronchomalacia undervent aortopexy or bronchopexy.The authors experience with this treatment is positive and itis in concordance with published reports.

Down syndrome and Crohn's disease: an extremely rare association.

trisomy 21, but normally concerns developmental abnormalities of the gastrointestinal tract, Hirschsprung disease or intestinal pathologies with verosimilar genetic basis, such as gluten enteropathy.1 Clustering of cases of Crohn’s disease according to family, race and ethnicity, as well as increased incidence of some human leukocyte antigens (HLA) loci in these patients supports the role of genetic factors in the disease.2 Further indirect evidence for a genetic basis for Crohn’s disease is the reported association with Ullrich– Turner syndrome, Hermansky–Pudlak syndrome, mucoviscidosis and other disorders with known genetic etiologies.3–5 Since we found only one previous report of the presence of Crohn’s disease in a patient with trisomy 21,6 we would like to report on this extremely rare association (trisomy 21, anal atresia and Crohn’s disease) found in our patient.

Pulmonary tuberculosis with gallbladder involvement: a review and case report.

illness and death worldwide. The incidence of TB in western countries has steadily declined, but this trend is reversing. Over the past decade the number of TB cases has increased dramatically in developing countries. This is attributed to an increasing proportion of cases among immigrants and refugees born in countries where TB is endemic, and to the increase in the number of cases of AIDS.1,2 The occurrence of TB in children implies recent transmission in the community and reflects the effectiveness of TB control programs. Around the world, almost 500 children die of TB each day and it has been estimated that 170 000 children die each year from the two most severe forms of disseminated disease, meningeal and miliary TB. In this paper a patient with primary progressive pulmonary tuberculosis who developed clinical signs of acute cholecystitis with inspissate bile (gallstones) is presented. To our knowledge such association is extremely rare in children and it remains unclear whether it was tuberculosis in origin or a side-effect of chemotherapy.

Complete transcatheter closure of a patent arterial duct with subsequent haemolysis

We report a development of severe haemolysis after complete transcatheter closure of patent arterial duct. Aortography and echocardiography revealed no signs of residual shunt. Haemolysis occurred a day after the implantation. Aortography was performed and the extrusion of coil in aorta was evident. The extruded part of the coil was surgically removed. No signs of haemolysis remained.

Laryngeal cyst and tracheomalacia in neonate: coincidence or complication?

The authors are presenting a case of a female infant with a large congenital laryngeal cyst. After operation, tracheomalacia with inspiratory stridor has been noticed.

Forces that fracture teeth during extraction with mandibular premolar and maxillary incisor forceps

Our aim was to measure the forces that fracture teeth during extraction based on the effectiveness of the extraction forceps, and to compare them with data collected about forces applied to extracted teeth that did not fracture. We studied 208 patients whose teeth fractured during both the standard and our new method of extraction: maxillary incisors (n=79) extracted with forceps 1 (maxillary incisor forceps), and both maxillary (n=95) and mandibular incisors (n=34) extracted with forceps 13 (mandibular premolar forceps). Forces needed to fracture were assessed with a specially-designed instrument for measuring pressure and rotation. Mean (SD) pressure at the fracture site was significantly higher in maxillary incisors extracted with forceps 1 (1.26 (0.26) bar) then in both maxillary and mandibular incisors extracted with forceps 13 (0.96 (0.19) and 0.98 (0.16), p<0.001). Pressure at dislocation and both left and right rotation showed similar patterns. Pressure correlated to root surfaces of teeth ranging from r=0.35-0.54 but the correlation coefficients did not differ significantly between the teeth-forceps groups. Pressure was higher in fractured than in extracted teeth, and this varied from 3%-48%. In conclusion, forces that break teeth during extractions are sometimes only slightly higher than the extraction forces, so caution is needed during extraction.

Overt yet unrecognized cyanosis in a 3-year old boy with right pulmonary artery-to-left atrium communication: malpractice or ignorance?

We present the case of a 3 year-old boy with obvious and severe blue discoloration of the skin and mucous membranes, pre-sent since birth, accompanied by clubbing of the fingers. He lives in the city and has been seen regularly by his pediatrician, and yet was never recognized as cyanotic. Cardiac examination revealed a soft systolic heart murmur. Electrocardiography (ECG) and blood pressure were normal and all peripheral pulses were present. Laboratory tests showed arterial hypoxia and polycythaemia. Echocardiography (ECHO), Doppler, angiography and multi-slice computed tomography pulmonary angiogram were performed. A right pulmonary artery-to-left atrium communication was diagnosed. Other possible causes of cyanosis were excluded. The child underwent surgery. At follow-up, one year later, the child was in excellent health. Labo-ratory tests were within referent values and oxygen saturation was normal. Repeated ECHO studies showed a progressive decrease in the size of the left atrium. This case illustrates the importance of careful physical examination during everyday practice and emphasizes the importance of collaboration between the physician and parents to ensure child wellbeing.

Preoperative management of hypoplastic left heart syndrome

Pediatricians are frequently involved in the care of cyanotic newborns in the labor and delivery room, as well as in the well baby nursery. Causes of hypoxia and cyanosis in the term newborn can be found within all physiological systems. Congenital heart structural diseases account for the largest diagnostic category. There have been significant advances during the past years in the diagnosis and treatment of neonates with critical congenital heart disease, especially in the field of pre- and post-operative intensive care. The term hypoplastic left heart syndrome (HLHS) describes a spectrum of cardiac structural abnormalities characterized by marked hypoplasia of the left ventricle and ascending aorta. Prenatal diagnosis, initial resuscitation and optimal preoperative management are key elements that allow the best opportunity for low mortality and normal neurodevelopment in affected newborns. Preoperatively, the goal is to achieve adequate systemic oxygen delivery. Patency of the ductus arteriosus (DA) is critical for survival until surgery. Blood flow to the pulmonary and systemic circulations should be nearly balanced (goal Q p /Q s ratio of 1). The immediate therapy for all infants with HLHS is an intravenous infusion of prostaglandin E 1 (PGE 1 ) in order to manipulate the DA and maintain ductal patency. Oxygen saturations of 75% to 85% by pulse oximetry suggest an adequate balance between systemic and pulmonary blood flow. Judicious use of inotropic support is initiated if evidence of low cardiac output is detected. Diuretics may be necessary to help alleviate the increased volume load on the right ventricle. The goal of respiratory management is to increase pulmonary vascular resistance and decrease systemic vascular resistance. Infants with HLHS who are born with a severely restricted or no inter-atrial communication, a rare occurrence, have profound hypoxemia. The severe restriction of blood flow across the atrial septum results in a life-threatening situation and these patients, which present with severe cyanosis and hemodynamic instability, require urgent postnatal cardiac catheterization to relieve the septal obstruction and improve oxygenation. Special attention should be paid to the prevention of brain injury and poor neurodevelopmental outcome. Care for infants with HLHS is complex, and often multiple specialists are involved. Despite an increase in the number of newborns with complex congenital heart disease and a growing percentage of patients with single-ventricle physiology, it is possible to care for this particular group of patients and achieve acceptable mortality risks, even in centres with no pediatric cardiac surgery facilities, if good preoperative management protocols are followed.