A case of neonatal bilateral diaphragmatic paralysis requiring surgery

Abstract Diaphragmatic paralysis may cause life threatening respiratory distress especially in infancy. A case is reported of a 32 week gestation infant with bilateral phrenic nerve palsies and associated brachial plexus injury who remained severely compromised despite spontaneous recovery of the right hemidiaphragm after 13 weeks. Operative plication of the left hemidiaphragm produced a marked improvement. Surgery should be considered after an adequate trial of expectant management in symptomatic patients.     

Accessory diaphragm associated with respiratory distress in a neonate

Supernumerary diaphragm is a rare congenital abnormality. This paper describes a neonate with respiratory distress caused by an overdistended left upper lobe and atelectasis of the left lower lobe entrapped below the accessory hemidiaphragm. Excision of the membrane dividing the hemithorax led to an uneventful recovery. Offprint requests to: I. Milovic     

A case of neonatal bilateral diaphragmatic paralysis requiring surgery

Diaphragmatic paralysis may cause life threatening respiratory distress especially in infancy. A case is reported of a 32 week gestation infant with bilateral phrenic nerve palsies and associated brachial plexus injury who remained severely compromised despite spontaneous recovery of the right hemidiaphragm after 13 weeks. Operative plication of the left hemidiaphragm produced a marked improvement. Surgery should be considered after an adequate trial of expectant management in symptomatic patients.     

Vascular ring formed by right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum: a rare cause of respiratory distress in newborn infants

Vascular ring, in which the trachea and esophagus are completely surrounded by vascular structures, is one of the causes of respiratory distress in children. Right aortic arch with aberrant left subclavian artery is a common aortic arch anomaly; however, respiratory distress due to vascular ring is seldom associated with this anomaly. We report herein a newborn infant treated surgically because of severe respiratory distress caused by vascular ring formed by right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum. As laryngomalacia was first thought to be the reason for respiratory distress, we suggest that patients with respiratory distress diagnosed with laryngomalacia be evaluated for possible vascular ring.     

Amebic liver abscess in children: clinical and epidemiologic features

Amebiasis, that is, infection with Entameba histolytica, continues to be endemic in the United States, with liver abscess occurring as an infrequent but constant complication. Seven cases are reported, with epidemiologic investigation of two. Reliable findings in hepatic abscess include fever, abdominal pain, respiratory distress, tender abdomen, and large, tender liver. Anemia, elevated white count with left shift, and the radiographic findings of an elevated right hemidiaphragm are constant. Epidemiologically, amebiasis occurs in clusters in the United States with person-to-person transmission predominant in spread. Infection is associated with poor sanitation and crowding. Investigation of the families of two patients documented 9/21 carriers and an additional 3/21 who were seropositive, as well as crowding and poor sanitation. In this country, treatment of a patient with amebic disease should include investigation of his home and family.     

Central hypoventilation and diaphragmatic eventration: diagnosis and management

Congenital central alveolar hypoventilation syndrome (CCAHS) is a disorder of ventilatory control that occurs without neuromuscular blockade or pulmonary disease. It is characterized by a lack of response to habitual respiratory stimulants, especially hypercapnia. In this article, the management of this syndrome by diaphragmatic pacing is discussed. Paralysis of the phrenic nerve in small children usually results from injury during birth or during a cardiothoracic operation and results in eventration of the hemidiaphragm. Alternatively, eventration of the diaphragm may be a congenital condition. In both cases it may lead to respiratory distress in the newborn. In this article, we review the diagnosis of these conditions and their management, focusing on the surgical indications. We also discuss outcome.     

Phrenic nerve paralysis of obstetrical origin: favorable course using continuous positive airway pressure]

INTRODUCTION: Isolated diaphragmatic paralysis due to obstetrical factors is rare and therapeutic management modalities are not quite clear. CASE REPORT: A neonate born by breech delivery presented with respiratory distress due to isolated paralysis of the right hemidiaphragm. The clinical course was progressive, his condition worsening with oxygen supplementation. Continuous positive airway pressure (CPAP) delivered via a nasal cannula was started in the one-month-old child, inducing gradual improvement towards recovery at the age of two months and a half. CONCLUSION: Non-invasive nasal CPAP should be proposed for the treatment of phrenic nerve obstetrical palsy before introducing more invasive ventilation techniques. Surgical plication should be delayed until the child reaches the age of at least three months.     

Antidiuretic hormone response in children with bronchopulmonary dysplasia during episodes of acute respiratory distress

We investigated the antidiuretic hormone (ADH) response in 12 infants with bronchopulmonary dysplasia during acute respiratory distress. All of the infants had hypoxemia with air-trapping in the chest at the time of admission to the hospital. None had documented infection. There was a dramatic increase in the plasma levels of ADH during acute respiratory distress, with a subsequent reduction of levels toward normal when the respiratory distress decreased to the preadmission well state. Three of 12 infants manifested hyponatremia at 24 hours after admission, with two of them exhibiting persistent hypertension for up to three days. The mechanism for elevated ADH levels is air-trapping in the chest, causing pulmonary hypovolemia and decreased left atrial filling and/or decreased transmural pressure of the left atrium.     

Atrial right-to-left shunt in infants with respiratory and cardiac distress but without congenital heart disease

Summary Contrast echocardiography was used to detect atrial right-to-left shunts in 20 term infants; there were 10 cyanosed infants who had severe respiratory distress and signs of cardiac failure but no apparent congenital heart disease and 10 noncyanosed infants with no respiratory distress and no signs of cardiac failure who underwent exchange transfusion for hyperbilirubinemia.All of the venous contrast echocardiograms in the cyanosed infants showed an atrial right-to-left shunt with a dense contrast effect in the left atrium and aorta but little in the right ventricle. A sparse effect in the left atrium and a dense one in the right ventricle were recorded in infants with resolving respiratory distress and in three infants from the control group. The other seven infants in the control group showed no right-to-left shunt. Conventional M-mode echocardiograms showed no evidence of congenital heart disease in any of the 20 infants.This contrast echocardiographic technique was useful for demonstrating an atrial right-to-left shunt in cyanosed term infants with neonatal respiratory problems and signs of congestive failure but no apparent congenital heart disease.     

Spontaneous rupture of the diaphragm.

A 2.5 year old girl with metachromatic leukodystrophy presented with acute respiratory distress and was initially wrongly diagnosed with pneumothorax. Barium meal showed bowel loops in the left hemithorax, which prompted surgical intervention; spontaneous rupture of the diaphragm was diagnosed at surgery.     

Unusual Clinical Presentation of Rare Adult Survivors With a Missing Pulmonary Valve Associated With a Missing Left Pulmonary Artery

Tetralogy of Fallot with absent pulmonary valve syndrome is associated with absence of the left pulmonary artery in one-seventh of cases. Infants with this disorder commonly present very early in life with respiratory distress and heart failure. Because the pulmonary blood flow is primarily to the right lung only, severe respiratory distress occurs. Survival to late childhood without surgery is very rare due to clinical worsening in infancy. This report describes two adult natural survivors with this rare association who presented with extremely unusual unreported clinical features. The aneurysmally dilated main pulmonary artery was compressed on the left coronary ostium, causing angina in the first patient. The second patient had recurrent ventricular tachycardia originating from the dilated thinned out right ventricular outflow tract. Both patients had successful surgical correction with valved xenograft conduits.     

Spontaneous rupture of the diaphragm

A 2.5 year old girl with metachromatic leukodystrophy presented with acute respiratory distress and was initially wrongly diagnosed with pneumothorax. Barium meal showed bowel loops in the left hemithorax, which prompted surgical intervention; spontaneous rupture of the diaphragm was diagnosed at surgery.

     

Extralobar pulmonary sequenstration presenting as intractable pleural effusion

A pre-term baby girl presented at birth with respiratory distress and pleural effusion on the left. A soft tissue mass was found via lateral x-ray and CT scan; the mass proved to be extralobar pulmonary sequestration.     

Utility of B-Type Natriuretic Peptide in Differentiating Congestive Heart Failure from Lung Disease in Pediatric Patients with Respiratory Distress

Plasma B-type natriuretic peptide (BNP) has been reported to be helpful in differentiating cardiac from pulmonary etiologies of dyspnea in adults. We investigated whether BNP concentration could be applied similarly in children with respiratory distress. BNP levels were measured using a rapid immunoassay in 49 infants and children presenting with acute respiratory distress. The patients history, symptoms, physical exam, chest x-ray, and an echocardiogram were used to identify patients with congestive heart failure (CHF) from noncardiac causes of respiratory distress. Results are reported as mean ± SD. Patients with CHF (n = 23) had BNP levels of 693.0 ± 501.6 pg/ml, significantly higher than those of the group of patients with lung disease (n = 26), whose BNP was 45.2 ± 64.0 pg/ml (p < 0.001). There was no significant difference in age between the two groups (29.7 ± 59.3 vs 13.1 ± 22.6 months; p = 0.12). A BNP level of 40 pg/ml was 84% accurate in differentiating CHF from pulmonary disease. Fifteen of 23 CHF patients had ventricular volume overload from left-to-right shunting congenital heart defects and 8/23 had left ventricular systolic dysfunction. Age-adjusted comparison of the two subgroups of CHF patients revealed that children with left ventricular systolic dysfunction had significantly higher mean BNP levels than those with left-to-right shunts (1181 ± 487 vs 433 ± 471 pg/ml, p = 0.0074). We conclude that BNP level is of value in differentiating cardiac from pulmonary causes of respiratory distress in children.     

Severe respiratory distress at birth caused by a hairy polyp of the Eustachian tube: Transoral endoscopy‐guided treatment

Hairy polyps are rare developmental lesions, which present as masses mainly consisting of fatty tissue covered by skin, seldom localized in the nasopharynx, causing respiratory obstruction. We describe the case of a female newborn affected by a hairy polyp arising from the left Eustachian tube, who presented severe respiratory distress soon after birth. The polyp was successfully removed transorally under videoendoscopic guidance. This case highlights the importance of including hairy polyp in the differential diagnosis of respiratory distress at birth because this type of tumor can be lethal and requires prompt treatment. A transoral endoscopy‐guided approach can allow successful and minimally invasive excision even in a newborn.     

Endocardial fibroelastosis with coronary artery thromboembolus and myocardial infarction

We report a case of an 18-month-old male, born to a woman with third trimester febrile illness, who had a history of congestive heart failure and respiratory distress, cardiomegaly, and electrocardiographic (ECG) findings suggestive of cardiomyopathy and myocarditis. After gradual improvement in heart size and function with pharmacologic therapy, he developed a terminal episode of respiratory distress and cardiogenic shock, with ECG findings of an anterolateral infarct. At autopsy it was found that endocardial fibroelastosis with mural thrombi in the left ventricle had been complicated by thromboembolism to the left anterior descending coronary artery, resulting in transmural infarction of the anteroseptal region of the left ventricle. Myocardial infarction is a potential but unusual thromboembolic complication of endocardial fibroelastosis. A high index of suspicion for coronary artery thromboemboli should be maintained in pediatric patients with cardiomyopathy and suspected myocardial infarction.     

Surgical management of congenital lobar emphysema

Here in nine patients with congenital lobar emphysema who had been treated surgically in the previous 10 years are reported. The ages of the patients at diagnosis ranged from 26 days to 11 months. The six patients whose symptoms started in the neonatal period had more severe dyspnea, cyanosis and respiratory distress. Tube thoracostomy was performed in two of three patients who had been misdiagnosed initially. The affected side was the left upper lobe in five patients, the right upper lobe in three, and the right middle and upper lobes in one patient. Lobectomy was performed in all cases. Dysplasia of the bronchial cartilage was found in six patients and bronchial atresia of the left upper lobe was found in another infant as the etiologic cause of the condition. Although the possibility of conservative management in congenital lobar emphysema has been reported recently, we believe that surgery is the treatment of choice in patients who have persistent or progressive, severe respiratory distress in spite of medical treatment.     

Echocardiographic abnormalities in infants of diabetic mothers.

In order to evaluate the presence of myocardial hypertrophy and/or abnormalities of postnatal cardiovascular adaptation, echocardiograms were performed on 34 infants of diabetic mothers. Based on cardiopulmonary assessment, the IDM were divided into three groups: Group I with congestive heart failure predominating: Group II with respiratory distress predominating: Group III asymptomatic. Hypertrophy of the interventricular septum and of the walls of left and right ventricles was frequently present in IDM: this change was most notable in association with clinical CHF. Six IDM, four of whom were found to have CHF, had additional echocardiographic signs of subaortic stenosis. All IDM had normal indices of left ventricular performance, despite the presence of CHE. In IDM with respiratory distress, the right ventricular pre-ejection period to ventricular ejection time ratio was elevated, suggesting an abnormality of the transitional pulmonary circulation. Poor maternal diabetes control and maternal systemic hypertension were closely correlated with evidence of myocardial hypertrophy in the infants.     

选择性剖宫产与新生儿呼吸系统疾病相关性分析

目的 评估不同胎龄足月儿选择性剖宫产（ECD）分娩后呼吸系统疾病的发生风险,探讨减少新生儿呼吸系统疾病发生的ECD时机.方法 对2008年6月至2013年6月在郑州人民医院行ECD分娩的新生儿进行回顾性分析.入选标准：胎龄37 ～ 39周,单胎,母亲无妊娠期合并症和并发症,剖宫产前无胎膜早破及临产征象.分析胎龄37、38、39周三组新生儿的一般情况及呼吸系统疾病发生情况.结果 共有1152例胎龄37～39周的ECD新生儿纳入研究,37周组136例,38周组401例,39周组615例,因发生呼吸系统疾病转入新生儿科的病例94例,其中37周组20例,呼吸系统疾病发生率14.7％;38周组38例,呼吸系统疾病发生率9.5％;39周组36例,呼吸系统疾病发生率5.8％.胎龄越大,ECD新生儿呼吸系统疾病发生率越低,差异有统计学意义（P＜0.05）.胎龄37 ～ 39周ECD新生儿中,发生的呼吸系统疾病以湿肺、窒息及肺炎为主,湿肺共51例,占54.2％;窒息共9例,占9.6％;肺炎共17例,占18.1％.胎龄37周和38周进行ECD的新生儿呼吸系统疾病发生率均较胎龄39周增高（OR分别为2.5和1.8,P均＜0.05）.结论 胎龄37 ～ 38周进行ECD可增加新生儿呼吸系统疾病的发生率,ECD尽可能在妊娠39周后进行.     

Costal osteochondroma presenting as haemothorax and diaphragmatic laceration

We report the unusual case of an 11-year-old girl who suffered a large haemothorax and lacerated left hemidiaphragm because of a costal osteochondroma arising from the left sixth rib near the costochondral junction. Surgical excision of the osteochondroma was performed and the lacerated diaphragm was repaired.