Mediastinal ganglioneuroblastoma-secreting vasoactive intestinal peptide causing secretory diarrhoea

In this case report we describe a case of mediastinal ganglioneuroblastoma-secreting vasoactive intestinal peptide (VIP), causing secretory diarrhoea in an 18-month-old child.An 18-month-old girl presented with a 2-month history of diarrhoea, abdominal distension and weight loss. Investigations revealed secretory diarrhoea with hypokalaemia, hyponatraemia and hypochloraemia and metabolic acidosis. Her stool output was 2.5–3.l day^?1 with increased stool sodium. VIP levels were strikingly high with normal glucagon and gastrin levels. X-ray of the chest revealed a well-defined mass in the right upper zone with tracheal shift, which was confirmed with computed tomography (CT) of the chest. The mass was resected and the patient became asymptomatic.This case shows that secretory diarrhoea caused by VIP and produced by ganglioneuroblastoma indicates a favourable prognosis, provided it is resectable.     

Chest Wall Abscess likely due to BCG Vaccination in a Child

Abstract   Bacillus Calmette-Guérin (BCG) vaccine has a good safety profile in immune competent children and considerable risks such as disseminated BCG disease in individuals with immune deficiencies. However, some complications including local subcutaneous infection, regional suppurative lymphadenitis may occur after BCG vaccination in immune competent children. A 17-month-old female child was admitted with a painless protruding mass on the anterior chest wall. There was no evidence of previous tuberculous infection and contact with a tuberculosis patient. Chest-computed tomography revealed an oval shaped low attenuated 1.8 × 1.0 cm mass at subcutaneous and muscle layers just below sternum, but the pulmonary parenchyma seemed to be normal. We excised the mass and the histopathologic examination revealed granulomatous lesions that suggestive of mycobacterial disease process. We considered that the chest wall abscess was a complication of BCG vaccination occurred by hematogenous dissemination of the inoculated mycobacteria. She recovered without any complications and was treated with isoniazid (150 mg/day) and rifampin (150 mg/day) for 6 months as an outpatient, not directly observed therapy but monthly follow-up.     

Computed tomography with normal chest radiography in childhood tuberculosis

During the recent 2 years we experienced 5 children (2 months-5 years old) with lung tuberculosis, all of whom had positive tuberculin skin-tests, and were in close contact with their family members who had active tuberculosis. However, no abnormal findings on chest X-ray films were pointed out by 2 independent roentogenologists, and no increased levels of inflammatory markers including WBC numbers, CRP, and ESR were detected in all but one child. Moreover, mycobacterial examination of sputa and/or gastric aspirates by microscope, culture technique, and PCR amplification revealed no causative bacilli in 4 children. To clarify whether these children were affected by Mycobacterium tuberculosis, chest CT scan was applied. Surprisingly, all 5 children were revealed to have abnormal changes including primary complexes in the lung field. Taken together, it is important to pursuit the apparent lung tuberculosis in children with a positive family history and positive tuberculin skin-test.     

Successful repair of a left ventricular aneurysm after blunt chest trauma in a child

A 5-year-old boy sustained multiple serious injuries, including compression trauma to the chest, when he was struck by a piece of timber. Two-dimensional echocardiography and left ventricular angiography revealed a left ventricular apical aneurysm, which was successfully repaired with the use of cardiopulmonary bypass. To our knowledge, this is only the 5th case in which a left ventricular aneurysm resulting from closed-chest injury in a child has been successfully treated.     

Case of varicella-zoster pneumonia with bronchioalveolar lavage confirmed by the detection of VZV DNA in the bronchial washing by the polymerase chain reaction]

A 32-year old male was admitted to our hospital complaining of cough, fever, and skin eruptions. He was coctacted with a child who had chickenpox 3 weeks before the onset. He showed the elevating of antibody to varicella-zoster virus. Despite of the administration of Acyclovir for four days, cough was not relieved and a chest X-ray film showed infiltrative shadow in right middle lobe of the lung. Bronchoscopic examination revealed vasicle and edema, and the varicella-zoster virus (VZV) DNA was detected in the bronchoalveolar lavage by the polymerase chain reaction. The patient in first case confirmed by the virus DNA in the bronchial washing by the PCR.     

Successful endovascular treatment of type B aortic dissection in a 15-year-old child

Aortic dissection is a rare condition in the pediatric and young adult population [1]. Here, we present an unusual case of a 15-year-old male patient with sudden onset of chest and back pain аnd numbness in the left leg. Chest and abdominal CT revealed Stanford type B (DeBakey type III) aortic dissection, leading to subocclusive stenosis of the superior mesenteric artery (causing critical intestinal dysfunction with ileus) and total occlusion of left iliac and left renal arteries. The child was admitted 48 h after chest pain onset in critical clinical condition. Revascularization was achieved by several consecutive endovascular procedures, and the patient was discharged after favorable clinical evolution with full restoration of flow in the target organ arteries. The first interventional treatment included stenting of the superior mesenteric artery, stenting of the abdominal aorta, and balloon dilatation of the left iliac artery. Due to resistant renovascular hypertension, stenting of the left renal artery, which was occluded, was conducted 20 days later, leading to optimal blood pressure control. Thirty-three days following the initial procedure, an endovascular endograft prosthesis implantation was performed to close the primary tear in the thoracic aorta. Genetic samples revealed ACTA2 mutation. This case is extraordinary because of its combined life-threatening aortic and vessel pathology, treated for the first time with endovascular means in a child, and highlights the feasibility of endovascular treatment in the pediatric population. We discuss the imaging, management, and successful outcome of this severe condition.     

Esophageal Trachealization: A Feature of Eosinophilic Esophagitis

Eosinophilic esophagitis (EE) is an inflammatory condition characterized by intense eosinophilic infiltration of the esophagus. EE is frequently misdiagnosed as gastroesophageal reflux disease. Here, we present a child with EE and a characteristic endoscopic finding, “ringed esophagus”. An 11-year-old Saudi boy presented with dysphagia for 1 year. He had experienced an intermittent sensation of solid food sticking in his chest, which was relieved by drinking liquids. A barium swallow excluded anatomical causes of dysphagia, but revealed multiple-ringed esophagus. Endoscopy showed a furrowing and trachealizing appearance of the entire esophagus. Hisologically, extensive eosinophilic infiltration was a feature in biopsies obtained from the esophagus. The child responded well to a 2-month course of inhaled fluticasone. Symptoms recurred 3 months after discontinuation of therapy, which necessitated resumption of inhaled fluticasone. The endoscopic appearance of multiple esophageal rings should raise suspicion of EE and be confirmed by esophageal biopsies.     

Acute aortic dissection in a 34 year old pregnant woman--a case report

A case of a 34 year old pregnant woman at 38 weeks gestation, who was admitted to the hospital with acute chest pain, is presented. Electrocardiogram showed ST segment changes suggestive of acute myocardial infarction. Echocardiography, however, revealed acute aortic dissection. The patient was transferred to another hospital where caesarean section followed by cardiac surgery were successfully performed. A subsequent one-year follow-up period was uneventful, both for mother and child. Diagnosis and treatment of acute aortic dissection during pregnancy are discussed.     

Aortic aneurysm after patch aortoplasty repair of coarctation: a prospective analysis of prevalence, screening tests and risks

Twenty-nine children were evaluated prospectively for the presence of an aortic aneurysm at the repair site 1 to 19 years after patch aortoplasty repair of coarctation of the aorta. In each child, noninvasive evaluation included a chest X-ray film, computed tomography of the chest and two-dimensional echocardiography. The presence and size of an aortic aneurysm were determined quantitatively by measuring the ratio of the diameter of the thoracic aorta at the repair site to the diameter of the aorta at the diaphragm (aortic ratio). An aortic ratio of greater than or equal to 1.5 was judged abnormal and was shown to be significantly greater than the aortic ratio of a normal control group. An aortogram was obtained in each child if any noninvasive screening test was found to be abnormal. As assessed by the aortogram, the prevalence of aortic aneurysm was 24% in this patient group. The sensitivity of echocardiography and chest computed tomography for detecting an aneurysm was 71% and 66%, and the specificity 76% and 85%, respectively. The chest X-ray film was 100% sensitive and 68% specific in determining the presence of an aneurysm. Although the data are not statistically significant, they suggest that children undergoing patch aortoplasty as the primary procedure (rather than a reoperation after earlier resection), and children in whom a Dacron patch is utilized may be at increased risk for aneurysm formation.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pompe's disease or type IIa glycogenosis

This is the report of a five-month-old child presenting clinical evidence of Pompe's disease: severe hypotonicity, hyporeflexia and congestive heart failure. The ECG showed a short PR interval, the chest radiography disclosed marked cardiomegaly, and the echocardiogram revealed marked left ventricular hypertrophy - the most typical finding of this disease. A skeletal muscle biopsy led to final diagnosis, because in the histopathologic study marked increased glycogen accumulation was evident. Death occurred two months after symptom onset.     

Morgagni hernia: an unexpected cause of respiratory complaints and a chest mass

Morgagni hernia (MH) is the least common type of congenital diaphragmatic hernias. Although its course is often asymptomatic, it may be associated with various respiratory and gastrointestinal symptoms. We describe 7 children with MH during a 5-year period in three pediatric centers in Turkey. All children had acute or chronic respiratory symptoms; cough was the most frequent. The diagnosis was made by posterior-anterior (PA) and lateral chest X-rays. The PA chest X-rays showed a homogenous mass in 2 and a gas-filled cystic image in 3 children in the right cardiophrenic angle. A retrocardiac homogeneous density in one child, and bilateral consolidation in lower lung areas in another child were also seen. All lateral chest X-rays showed gas-filled bowel loops above the diaphragm. The diagnosis was confirmed by barium-contrast radiograph. Four patients had five additional anomalies, i.e., ventricular septal defect, right inguinal hernia, congenital hip dislocation, pectus carinatum, and obstruction of the uretero-pelvic junction. All of the hernias were repaired by an abdominal approach. There were no complications or recurrences during follow-up. In conclusion, MH should be considered in the differential diagnosis of cases of long-standing respiratory symptoms and/or when an unexplained radiological image, especially on the right cardiophrenic area, is present.     

Maximal static pressures and lung volumes in a child with Guillain-Barré syndrome ventilated by a cuirass respirator

Serial maximal static pressure and lung volume measurements were made in a child with Guillain-Barré syndrome and respiratory failure. The patient received successfully ventilatory support for seven weeks with the use of a cycled negative-pressure cuirass-type respirator obviating prolonged intubation and tracheostomy. The fall in VC preceded the loss of respiratory muscle strength (RMS) and lagged behind RMS in the return towards normal values. There was a disproportionate loss of lung volume (lowest VC, 220 to 250 ml, 11 to 12 percent of normal) as compared to maximal static pressures (lowest PImax, 60 cm H2O, lowest PEmax, 47 cm H2O). The disproportion was greater than that previously reported in adults. The greater loss in lung volume may be due to greater chest wall compliance in the child, resulting in less outward recoil of the chest wall and subsequently more microatelectasis with denervation of the chest wall musculature. Loss of lung volume resulted in a smaller surface area over which the respiratory muscles exerted their force. Thus, pressure being equal to force divided by surface area, could be relatively maintained and not reflect the extent of the loss of muscle force.     

Bronchiolitis obliterans organizing pneumonia associated with Evans syndrome.

The association of bronchiolitis obliterans organizing pneumonia (BOOP) with insulin-dependent diabetes mellitus (IDDM) and Evans syndrome (autoimmune pancytopenia) has not been reported previously. We describe the case of a 4-year-old child diagnosed with IDDM and Evans syndrome who presented malaise, fever and nonproductive cough for several months. The chest radiograph revealed several patchy alveolar opacities with peripheral and bilateral distribution and multiple hilar and mediastinal adenopathies. An open lung biopsy established the diagnosis of BOOP. During the follow-up over the next 7 years, the patient had chronic relapses in spite of corticosteroid treatment and developed restrictive lung disease.     

Lung volume reduction surgery in lieu of pneumonectomy in an infant with severe unilateral pulmonary interstitial emphysema

A male infant with a prenatal diagnosis (at 20 weeks' gestation) of cystic adenomatoid malformation was delivered after 38 weeks' gestation (birth weight, 3 kg) and admitted to the neonatal intensive care unit. During the first few days of life, he developed mild respiratory distress; a chest radiograph and computed tomography scan showed multiple cystic areas in the left lower lobe with hyperinflation and herniation of the upper lobe across the midline. At 3 weeks of age, a left lower lobectomy was performed for presumed cystic malformation. To our surprise the pathology reports revealed pulmonary interstitial emphysema. The postoperative chest radiograph was unchanged, and mechanical ventilation was necessary and required progressively increasing ventilatory settings to provide adequate support. High-frequency oscillatory ventilation and selective right bronchus intubation failed to improve lung function. After 3 weeks, a left thoracotomy was repeated and lung volume reduction was performed with removal of 50' of the peripheral hyperinflated parenchyma. Postoperative recovery was rapid; the child was weaned from the ventilator after 3 days and discharged after 3 weeks. Follow-up chest X-rays showed a normally expanded right lung with mediastinal structures back to midline and a small left lung. Favorable results persisted at 3 years of follow-up. This first and successful experience with lung volume reduction in a neonate suggests that infants who need removal of a large portion of lung parenchyma to achieve adequate ventilation and gas exchange, lung volume reduction surgery should be considered as an alternative to pneumonectomy.     

Left ventricular obstruction caused by a large hiatal hernia

A 76‐year‐old man was admitted to our emergency department owing to chest pain, which started immediately after lunch. Although electrocardiogram revealed ST‐segment elevation with hyperacute T‐wave changes in the anterior lead tracings, emergency coronary angiography revealed normal coronary arteries. Echocardiography revealed left ventricular (LV) compression with left ventricular obstruction (LVO) caused by an echogenic mass. Computed tomography clearly revealed compression of both left atrial (LA) and LV by a large hiatal hernia. A large hiatal hernia can induce cardiac symptoms resulting from cardiac compression. This case highlights a possible association between chest pain and LVO caused by a hiatal hernia.     

Unilateral hypertransradiancy in a child with recurrent chest infections

A 3-year-old female child was treated for six recurrent episodes of cough, high grade fever and breathlessness during past one year. Every time total leucocyte counts used to range between 17,000 - 25,000 per mm3 with neutrophils over 80%. Except for diminished breath sounds in left basal region, systemic and chest examinations were normal. Her chest roentgenogram is shown in figure 1. A good quality chest roentgenogram could not be done because of persistent movements of the child.     

Bronchial mucoepidermoid carcinoma in children

Three cases of children, aged 7, 12 and 13 years, respectively, with bronchial mucoepidermoid carcinoma are presented. All patients were primarily misdiagnosed as pneumonitis following common symptoms of prolonged fever, cough, expectoration, and indirect signs of bronchial obstruction by chest roentgenogram, before the tumours in the main bronchi were revealed by chest computed tomography and bronchoscopy. After undergoing lobectomy and systematic lymph node dissection, all three patients recovered uneventfully. Histologically, all the cases were low-grade mucoepidermoid carcinoma; one case additionally had mediastinal lymph node metastasis. The patients have remained in good health during a follow-up of between 1.5 and 6 years. Despite its extremely rare occurrence in childhood, the possibility of bronchial mucoepidermoid carcinoma should be kept in mind when encountering a child presenting with symptoms of recurrent pneumonia.     

Massive parapneumonic effusion caused by Mycoplasma pneumoniae in a child: a case report

Mycoplasma pneumoniae infection is usually mild course and self-limited. Parapneumonic effusion is not a common feature of M. pneumoniae. The treatment of parapneumonic effusion is macrolides and chest tube with adequate pleural drainage. We report here on a child with M. pneumoniae infection complicated by massive parapneumonic effusion. Protracted course of fever and respiratory distress was noted in patient. Pneumothorax was occurred subsequent to chest tube drainage.     

MRI after removal of central venous access device reveals a high number of asymptomatic thromboses in children with haemophilia

Central venous access devices (CVADs) are often required in children with haemophilia to secure venous access for prophylactic treatment or immune tolerance therapy. Complications of CVADs include infections, thrombosis and mechanical problems. This study sought to determine the outcome of the vessels by magnetic resonance imaging (MRI) in children with haemophilia and to assess risk factors for development of catheter-related deep venous thrombosis (DVT). After the removal of CVAD an MRI of the chest and neck was performed to 20 boys with haemophilia who each had 1-3 (total number 27) CVADs placed. MRI revealed DVT in five children (25%). As their CVADs were functional at the time of the removal, the DVTs were clinically silent. However, there had been suspicion of DVT leading to replacement of the CVAD in one case. All the children with DVT had their CVADs inserted initially below the age of 1 year. The clinical signs of mild post-thrombotic syndrome (PTS) were common: dilated chest wall veins were observed in 11 (55%) children and were associated with DVT in three cases. Arm circumference discrepancy was observed in one child with DVT. No correlation between the duration or number of CVADs and DVT was detected. None of the patients had subjective symptoms of PTS. Silent DVT is a common complication of CVAD. Catheter insertion at a young age seems to predispose to thrombosis. The long-term consequences of the DVTs remain unknown.     

Infantile cardiac fibromatosis: correlation of antemortem and necropsy findings

In the case presented here of a 2-month-old boy with symptoms of upper respiratory infection, the physical examination, chest X-ray film and electrocardiogram led us to suspect congestive heart failure caused by either a primary or infectious cardiomyopathy or by a structural left ventricular outflow tract obstruction. The echocardiographic findings were the first evidence to suggest the presence of an intracardiac tumor. A cardiac computerized axial tomography scan supported the findings. Operation was performed, but the tumor was unresectable. Biopsies of the mass revealed fibromatosis. The infant was discharged on diuretics, procainamide and propranolol. Approximately 3 weeks following discharge, while at rest, the child suddenly became unresponsive and could not be resuscitated. Postmortem examination verified the degree of precision that can be achieved by noninvasive techniques.