Expansile pneumonia in children caused by Mycobacterium tuberculosis: clinical, radiological, and bronchoscopic appearances

A cohort of 24 children with expansile pneumonia caused by Mycobacterium tuberculosis is described in mostly HIV-noninfected children (n = 22). The children presented with nonresolving pneumonia and a swinging fever (83%). On chest radiography, they had dense opacification with bulging fissures mainly in the upper lobes (75%). On computed tomography, the lobes are consolidated, with areas of liquefacation. Other features visible are enlarged mediastinal lymph adenopathy with ring enhancement (100%), cavities (63%), and tracheal compression (71%). On bronchoscopy, bronchi were obstructed by more than 75% in 20 (83%) of cases. Lymph gland enucleation was required in 42% of cases. Phrenic nerve palsy was present in 3 children, of whom 2 underwent diaphragmatic plication. The children received standard antituberculous therapy, to which prednisone (2 mg/kg/day) was added for 1 month. The mortality was 4% after 6 months of therapy.     

An adult case of cervico-mediastinal tuberculous lymphadenitis]

We reported a case of cervico-mediastinal tuberculous lymphadenitis followed by the development of pulmonary lesions and recurrent laryngeal nerve palsy. A 48-year-old man was admitted to our hospital due to fever, dry cough, and loss of body weight. He had no medical history of tuberculosis or other significant diseases. On admission, a right cervical tumor was palpable and chest X-ray films revealed widened superior mediastinum. Chest computed tomography showed multiple swollen mediastinal lymph nodes, including multiple low-density areas and contrast medium-enhanced septa and margins. Gallium-67 scintigraphy demonstrated abnormal uptake in the right cervix and mediastinum. A PPD skin test was strongly positive and ribosomal RNA of tubercle bacilli was detected in aspirated gastric juice. Although anti-tuberculous chemotherapy was initiated, fever and cough persisted, and hoarseness due to left recurrent laryngeal nerve palsy developed. One month later, chest X-ray films showed abnormal infiltration in the left upper lung field. The patient was sero-negative for HIV. To confirm the diagnosis, right supraclavicular lymph node biopsy was performed. Microscopic examination of the biopsy specimen revealed acid-fast bacilli and granuloma with central caseous necrosis surrounded by Langhans' giant cells and epithelioid cells. After 9 months of extended anti-tuberculous chemotherapy, the cervical and mediastinal masses receded and the abnormal chest X-ray shadows disappeared. Because the incidence of tuberculosis in Japan is gradually increasing among young people as well as the elderly, the differential diagnosis of this disease will become more necessary.     

Ultrasound in the diagnosis of diaphragmatic paralysis after operation for congenital heart disease.

Phrenic nerve palsy is a recognised complication of operation for congenital heart disease in children. The accuracy of ultrasound in assessing diaphragmatic motion was prospectively compared with fluoroscopy in 16 patients in whom phrenic nerve palsy was suspected. Ultrasound successfully identified the five patients with phrenic nerve palsy; there were no false positive or false negative diagnoses. Ultrasound was as effective as fluoroscopy in the diagnosis of abnormalities of diaphragmatic motion.     

Ultrasound in the diagnosis of diaphragmatic paralysis after operation for congenital heart disease

Phrenic nerve palsy is a recognised complication of operation for congenital heart disease in children. The accuracy of ultrasound in assessing diaphragmatic motion was prospectively compared with fluoroscopy in 16 patients in whom phrenic nerve palsy was suspected. Ultrasound successfully identified the five patients with phrenic nerve palsy; there were no false positive or false negative diagnoses. Ultrasound was as effective as fluoroscopy in the diagnosis of abnormalities of diaphragmatic motion.     

Case of Heerfordt's syndrome with prolonged peripheral nerve involvement]

A 28-year-old man complaining of myiodesopsia was given a diagnosis of uveitis. Subsequently he complained facial nerve palsy and enlargement of parotid gland. Heerfordt's syndrome was diagnosed based on the results of several examinations. Facial nerve palsy, enlargement of the parotid gland and uveitis were improved by systemic corticosteroid therapy. At present he is receiving systemic corticosteroid therapy, but numbness in the mouth, thought to be the involvement of the trigeminal nerve, remains. Systemic corticosteroid therapy is usually effective for most cases with Heerford's syndrome. On the other hand, there are some cases with the prolonged peripheral nerve involvement despite systemic corticosteroid therapy, as seen in this case. If peripheral nerve involvement is prolonged, it is necessary to consider small-fibre neuropathy as one possible cause.     

儿童胸内淋巴结结核CT诊断分析

目的分析胸内淋巴结结核CT表现。方法对我院诊治120例胸内淋巴结结核进行分析。结果胸CT表现：肺门区淋巴结肿大共76例,右侧52例,左侧6例,双侧18例,炎症型37例,结节型39例,可发生在肺门上、中、下部。右肺门上部淋巴结肿多在上叶支气管分叉附近,中部淋巴结多在右肺动脉分叉附近,下部淋巴结在右肺下动脉干周围,伴有肺内播散8例。气管旁淋巴结肿大共44例,右侧40例,左侧3例,双侧1例,炎症型16例,结节型28例。结论儿童胸内淋巴结结核CT表现复杂,需认真分析,密切结合临床及实验室检查,可做出正确诊断。     

Diaphragmatic paralysis due to Lyme disease.

Lyme disease is a tick-borne spirochaete infection which, in a proportion of patients, can lead to neuropathy. This article describes a case of diaphragmatic paralysis due to Lyme disease. A 39-yr-old male presented to the hospital because of an acute left facial palsy. Six weeks prior to admission he had developed a circular rash on his left flank during a camping holiday. He also complained of shortness of breath and arthralgia for 1 week. His chest radiograph demonstrated a raised right hemi-diaphragm. Diaphragmatic paralysis was confirmed by fluoroscopy (a positive sniff test). Serology revealed evidence of recent infection by Borrelia burgdorferi. On the basis of the patient's clinical presentation, a recent history of erythema migrans, and positive Lyme serology, a diagnosis of neuroborreliosis was made. He received oral doxycycline therapy (200 mg x day(-1)) for three weeks. Facial and diaphragmatic palsies resolved within eight weeks. On the basis of this case, a diagnosis of Lyme disease should be considered in patients from endemic regions with otherwise unexplained phrenic nerve palsy.     

Surgical treatment of anomalous left pulmonary artery

We report our experience with the surgical treatment of anomalous origin of the left pulmonary artery in eight children between 2004 and 2009. The congenital heart disease most frequently associated with this condition was patent ductus arteriosus. Surgery was carried out with extracorporeal circulation in five children, and without, in three. The anomalous pulmonary artery was divided and translocated to the main pulmonary artery. One patient died soon after surgery because of hemodynamic instability and another died later because of respiratory complications. The other patients progressed satisfactorily during follow-up: the reimplanted artery remained patent in all cases and respiratory symptoms improved. However, one patient required endoscopic treatment.Full English text available from: www.revespcardiol.org     

Diaphragmatic paralysis in young children: A literature review

Diaphragmatic paralysis (DP) is a rare cause of respiratory distress in young children. In the first years of life, the main cause is phrenic nerve injury after cardiothoracic surgery or obstetrical trauma. DP usually presents as respiratory distress. Asymmetrical thorax elevation, difficulty weaning from mechanical ventilation, pulmonary atelectasis, and repeated pulmonary infections are other suggestive signs or complications. DP is usually suspected on chest X‐ray showing abnormal hemidiaphragm elevation. Although fluoroscopy was considered the gold standard for DP confirmation, it has gradually been replaced by ultrasound, which can be done at the bedside. Some electrophysiological tools may be useful for a better characterization of phrenic nerve injury and chance of recovery. The management of DP is mainly based on clinical severity. In mild asymptomatic cases, DP may only require close monitoring. In more severe cases, adequate ventilatory support and/or surgical diaphragmatic plication may be needed. Electrophysiological tools may help clinicians assess the ideal timing for diaphragmatic plication.     

Diaphragmatic paralysis caused by malposition of chest tube placement after pediatric cardiac surgery

Diaphragmatic paralysis is a recognized complication after pediatric cardiac surgery. It is universally acknowledged that direct phrenic nerve injury during surgery is the etiology. However, we experienced two unusual cases of diaphragmatic paralysis following malposition of chest tube placement after pediatric cardiac surgery. The malposition of too deeply placed chest tube with resultant phrenic nerve injury was presumably the underlying cause. One patient underwent successful diaphragmatic plication due to intractable respiratory distress. The other was asymptomatic. Our report highlights the previously unreported complication of chest tube-induced phrenic nerve injury following its malposition after pediatric cardiac surgery. Prompt recognition and correction of tube malposition or selection of a softer chest tube probably can ameliorate the problem.     

Determinants of rib motion in flail chest

We have previously developed a canine model of isolated flail chest to assess the effects of this condition on the mechanics of breathing, and these studies have led to the conclusion that the respiratory displacement of the fractured ribs is primarily determined by the fall in pleural pressure (Delta Ppl) and the action of the parasternal intercostal muscles. The present studies were designed to test the validity of this conclusion. A flail was induced in six supine anesthetized animals by fracturing both dorsally and ventrally the second to fifth ribs on the right side of the chest, after which the phrenic nerve roots were bilaterally sectioned in the neck. Sectioning the phrenic nerves caused a 34% decrease in Delta Ppl, associated with a 39% increase in parasternal intercostal inspiratory EMG activity (p < 0.05), and resulted in a marked reduction in the inspiratory inward displacement of the ribs. In three animals, the inward rib displacement was even reversed into a small outward displacement. When the airway was then occluded at end-expiration to increase Delta Ppl during the subsequent inspiration, all animals again showed a clear-cut inward rib displacement. These observations therefore confirm that in dogs with flail chest, the inspiratory displacement of the fractured ribs is set by the balance between the force related to pleural pressure and that generated by the parasternal intercostals. These observations also point to the critical importance of the pattern of inspiratory muscle activation in determining the magnitude of rib cage paradox in such patients.     

Diaphragm electromyogram measured with unilateral magnetic stimulation.

The purpose of this study was to establish the phrenic nerve conduction time (PNCT) for magnetic stimulation and further assess the relatively new technique of anterior unilateral magnetic stimulation (UMS) of the phrenic nerves in evaluating the diaphragm electromyogram (EMG). An oesophageal electrode was used to record the diaphragm compound muscle action potential (CMAP) elicited by supramaximal percutaneous electrical phrenic nerve stimulation (ES) and UMS from eight normal subjects. The oesophageal electrode used for recording the CMAP was positioned at the level of the hiatus and 3 cm below. The diaphragm CMAP was also recorded from chest wall surface electrodes in five subjects. All of the phrenic nerves could be maximally stimulated with UMS. A clear plateau of the amplitude of the CMAP was achieved for the right and left phrenic nerves. The mean amplitudes of the CMAP recorded from the oesophageal electrode were, for the right side, 0.74+/-0.29 mV (mean+SD) for ES and 0.76+/-0.30 mV for UMS with maximal power output, and for the left side 0.88+/-0.33 mV for ES and 0.80+/-0.24 mV for UMS. PNCT measured by the oesophageal electrode with ES and UMS with maximal output were, for the right side, 7.0+/-0.8 ms and 6.9+/-0.8 ms, respectively, and for the left side 7.8+/-1.2 ms and 7.7+/-1.3 ms, respectively. However, the CMAP recorded from chest wall surface electrodes with UMS was unsuitable for the measurement of PNCT. The results suggest that unilateral magnetic stimulation of the phrenic nerves combined with an oesophageal electrode can be used to assess diaphragmatic electrical activity and measure the phrenic nerve conduction time.     

Negative extrathoracic pressure ventilation for phrenic nerve palsy after paediatric cardiac surgery.

OBJECTIVE--To investigate the feasibility of negative extrathoracic pressure ventilation as a respiratory support following phrenic nerve palsy after cardiac surgery. DESIGN--An uncontrolled pilot study. PATIENTS--14 patients aged one week to 30 months (median 5.3 months) with phrenic nerve palsy diagnosed by phrenic nerve conduction tests and diaphragmatic electromyograms. Four had bilateral and 10 unilateral palsy. Before treatment all required oxygen and 10 were receiving positive pressure ventilation. One of the patients with bilateral and four of the patients with unilateral palsies had undergone a plication before negative pressure ventilation was started. INTERVENTION--Treatment was started 6-65 days (median 23) after operation with a newly designed system which included a Perspex chamber, which gave easy access to the child, and an elastic latex neck seal. Continuous negative pressure was used in conjunction with intermittent positive pressure ventilation while continuous or intermittent negative pressure ventilation was used in extubated infants. RESULTS--All four patients with bilateral palsy survived with long-term intermittent negative pressure ventilation and did not require further surgery. Of the 10 with unilateral lesions, seven required no further surgery, two underwent plication, and one had a re-plication. Three patients with unilateral palsy died of non-respiratory causes. The duration of positive pressure ventilation after starting negative pressure ranged from 0 to 23 days (median 6). Treatment with negative pressure lasted for 3-241 days (median 32) and was predominantly administered off the intensive care unit, including at home. CONCLUSIONS--Negative pressure ventilation may be an alternative to positive airway pressure ventilation in the management of phrenic nerve palsy. A multicentre randomised controlled trial is now required to assess further the role of negative pressure ventilation in phrenic nerve palsy.     

Negative extrathoracic pressure ventilation for phrenic nerve palsy after paediatric cardiac surgery.

OBJECTIVE--To investigate the feasibility of negative extrathoracic pressure ventilation as a respiratory support following phrenic nerve palsy after cardiac surgery. DESIGN--An uncontrolled pilot study. PATIENTS--14 patients aged one week to 30 months (median 5.3 months) with phrenic nerve palsy diagnosed by phrenic nerve conduction tests and diaphragmatic electromyograms. Four had bilateral and 10 unilateral palsy. Before treatment all required oxygen and 10 were receiving positive pressure ventilation. One of the patients with bilateral and four of the patients with unilateral palsies had undergone a plication before negative pressure ventilation was started. INTERVENTION--Treatment was started 6-65 days (median 23) after operation with a newly designed system which included a Perspex chamber, which gave easy access to the child, and an elastic latex neck seal. Continuous negative pressure was used in conjunction with intermittent positive pressure ventilation while continuous or intermittent negative pressure ventilation was used in extubated infants. RESULTS--All four patients with bilateral palsy survived with long-term intermittent negative pressure ventilation and did not require further surgery. Of the 10 with unilateral lesions, seven required no further surgery, two underwent plication, and one had a re-plication. Three patients with unilateral palsy died of non-respiratory causes. The duration of positive pressure ventilation after starting negative pressure ranged from 0 to 23 days (median 6). Treatment with negative pressure lasted for 3-241 days (median 32) and was predominantly administered off the intensive care unit, including at home. CONCLUSIONS--Negative pressure ventilation may be an alternative to positive airway pressure ventilation in the management of phrenic nerve palsy. A multicentre randomised controlled trial is now required to assess further the role of negative pressure ventilation in phrenic nerve palsy.     

Shrinking lung syndrome as a presenting manifestation of systemic lupus erythematosus in a female Kuwaiti

The shrinking lung syndrome (SLS) is a rare manifestation in patients with established systemic lupus erythematosus (SLE). Only two cases have been reported in which this syndrome was the presenting manifestation of SLE. We describe a 21-year-old female Kuwaiti who presented with SLS. In addition to clinical and serological features of lupus, she had dyspnea, respiratory muscle dysfunction, characteristic chest radiographic findings of small lung volumes, elevated right hemidiaphragm, and basilar atelectasis. There was no pulmonary parenchymal or pulmonary vascular involvement. Nerve conduction study showed right phrenic nerve palsy. She responded well to treatment with corticosteroids.     

Endobronchial Metastases from Colon Cancer without Liver Metastases: Report of Eight Cases

Purpose The liver is the most common site of hematogenous spread from colon tumors. Pulmonary metastases from colon cancer result, in most of the cases, from hepatic metastases. Methods We describe eight cases of colorectal cancers in which endobronchial metastases have been developed without any evidence of liver involvement. Results Median age was 62 years old. In most of the patients, the primary cancer developed in the left side. The median time from colorectal presentation to pulmonary onset was four years. Dyspnea was the major symptom in all cases. Pulmonary involvement included endobronchial metastasis in all cases. CT scan of the chest showed bilateral, diffuse, large, nodular infiltrates without lymph nodes enlargement and without pleural effusion. Endobronchial therapy brought symptomatic relief in all cases; however, two-year follow-up showed only 50 percent survival rate. Conclusions Endobronchial metastasis should be suspected in patients with colon cancer with respiratory symptoms, even without known liver metastasis. To the best of our knowledge, such a case series has not been published yet.     

Hypothyroidism presenting with respiratory muscle weakness

A 58-yr-old woman presented with recurrent chest infections, breathlessness, and orthopnea. She complained of nonspecific tiredness and aching limbs. A chest radiograph showed an elevated right hemidiaphragm. Thyroid function tests showed her to be severely hypothyroid (T4 = 23 nmol/L; TSH greater than 50 mU/L). Measurement of maximal respiratory mouth pressures (expiratory: 50 cm H2O, normal, 94 +/- 33; inspiratory: 15 cm H2O, normal, 71 +/- 27) suggested global respiratory muscle weakness. Severe bilateral diaphragm weakness was demonstrated by a greatly reduced maximal transdiaphragmatic pressure (Pdi) (Pdi Pimax = 0, normal, 65 +/- 31 cm H2O; sniff Pdi = 25 cm H2O, normal, 121 +/- 25). No Pdi was detectable on stimulation of the right phrenic nerve, whereas, on the left, it was 11 cm H2O (normal 7 to 15 cm H2O). Phrenic nerve conduction time was prolonged to both sides (right, 12 ms, left, 10 ms; normal, less than 9.5 ms). The relaxation rate of Pdi after a maximal sniff and after bilateral phrenic nerve stimulation was abnormally slow (7.4%/10 ms, 6.3%/10 ms, respectively). Three months after starting treatment with thyroxine she had become euthyroid, and phrenic nerve conduction times and Pdi relaxation rates had returned to normal. Maximal respiratory pressures, vital capacity, and maximal voluntary ventilation improved progressively on treatment, although maximal respiratory pressures still had not reached the normal range after six months. We conclude that hypothyroidism may present with breathlessness due to respiratory muscle weakness and/or phrenic nerve neuropathy and is reversible with treatment.     

Acute cervical motor radiculopathy induced by neck and limb immobilization in a patient with Parkinson disease

A 68-year-old woman with Parkinson disease (PD) presented with acute monoplegia of her left upper extremity after the neck and limb immobilization for several hours. Her sensory function was normal, and the chest X-ray showed left phrenic nerve palsy. Electrophysiological studies showed multi-segment muscle involvement (C3 to T1) including denervation potentials and reduced interference of motor units in needle electromyography. M wave amplitude in peripheral nerve stimulation was preserved except for the ulnar nerve, suggesting both axonal injury and conduction block at the anterior spinal roots. The patient showed fair recovery in several months, suggesting sufficient reinnervation and recovery of conduction block. Incomplete root avulsion was thought to be the pathomechanism of acute cervical motor radiculopathy.     

原发性干燥综合征合并结核病28例临床分析

目的 探讨结核病合并原发性干燥综合征的临床特点,注意肺结核与原发性干燥综合征导致的间质性肺疾病的鉴别。 方法 回顾性分析2005-2011年解放军第三O九医院28例合并原发性干燥综合征的结核病患者的临床资料。 结果 28例中男2例,女26例;40岁以上女性21例。临床上干咳、眼干、口干、发热多见。有6例首次诊断为原发性干燥综合征,均行自身抗体及腮腺X线增强造影检查。其中2例行下唇腺活检,结果阳性;余22例既往均有原发性干燥综合征病史,病史最短1年,最长14年,平均（2.7±1.6）年。经细菌学阳性确诊结核病10例,经组织病理学确诊结核病8例,经临床诊断结核病10例。28例患者中继发性肺结核11例,血行播散性肺结核3例,结核性胸膜炎2例,淋巴结结核2例,继发性肺结核合并支气管结核1例,继发性肺结核合并淋巴结结核2例,继发性肺结核合并结核性胸膜炎2例,继发性肺结核合并结核性心包炎2例,结核性多浆膜腔炎2例,继发性肺结核合并支气管结核及附件结核1例。所有患者均给予2HRZE/4HR抗结核治疗。至今28例患者中有5例仍在进行抗结核治疗,其余23例结核病患者均临床治愈。所有患者均未发生严重药物不良反应。4例肺结核误诊为原发性干燥综合征导致的间质性肺疾病。 结论 原发性干燥综合征合并结核病重点需注意原发性干燥综合征导致呼吸系统损害与原发性干燥综合征合并肺结核鉴别,减少临床上误诊、漏诊的发生。     

Phrenic nerve injury in infants and children undergoing cardiac surgery.

Fifty infants and 50 children less than 15 years undergoing palliative or corrective cardiac surgery in the Brompton Hospital between March and October 1988 had direct percutaneous stimulation of the phrenic nerve before and after operation. Ten patients, six under 1 year of age and four over, developed unilateral phrenic nerve injury. In those aged less than 1 year recovery after operation was prolonged because their diaphragmatic palsy made it difficult to wean them from the ventilator. Older children had symptoms but their rate of recovery did not seem to be affected by the phrenic nerve injury. Phrenic nerve damage was no more frequent after a lateral thoracotomy than after a median sternotomy. There was no significant association with the type of operation performed, the experience of the surgeon, the use of bypass or topical ice, the duration of bypass, circulatory arrest or aortic cross clamping, or the age of the patient at the time of operation. In patients who had cardiopulmonary bypass the risk of injury was significantly higher in those who had undergone previous operation. The 10% frequency of phrenic nerve injury determined in this prospective study was higher than that seen in earlier retrospective reports. Direct percutaneous stimulation of the phrenic nerve can be used at the bedside in infants and children to facilitate early and accurate diagnosis of phrenic nerve palsy, and the results may influence early management.