scholarly journals Comorbidity between Klinefelter syndrome and diaphragmatic hernia. A case report

2014 ◽  
Vol 132 (5) ◽  
pp. 311-313
Author(s):  
Carolina Melendez Valdez ◽  
Stephan Philip Leonhardt Altmayer ◽  
Adyr Eduardo Virmond Faria ◽  
Aline Weiss ◽  
Jorge Alberto Bianchi Telles ◽  
...  

CONTEXT: Intrathoracic cystic lesions have been diagnosed in a wide variety of age groups, and the increasing use of prenatal imaging studies has allowed detection of these defects even in utero.CASE REPORT: A 17-year-old pregnant woman in her second gestation, at 23 weeks of pregnancy, presented an ultrasound with evidence of a cystic anechoic image in the fet al left hemithorax. A morphological ultrasound examination performed at the hospital found that this cystic image measured 3.7 cm x 2.1 cm x 1.6 cm. Polyhydramnios was also present. At this time, the hypothesis of cystic adenomatoid malformation was raised. Fet al echocardiography showed only a dextroposed heart. Fet al magnetic resonance imaging produced an image compatible with a left diaphragmatic hernia containing the stomach and at least the first and second portions of the duodenum, left lobe of the liver, spleen, small intestine segments and portions of the colon. The stomach was greatly distended and the heart was shifted to the right. There was severe volume reduction of the left lung. Fet al karyotyping showed the chromosomal constitution of 47,XXY, compatible with Klinefelter syndrome. In our review of the literature, we found only one case of association between Klinefelter syndrome and diaphragmatic hernia.CONCLUSIONS: We believe that the association observed in this case was merely coincidental, since both conditions are relatively common. The chance of both events occurring simultaneously is estimated to be 1 in 1.5 million births.

2019 ◽  
Vol 18 (1) ◽  
Author(s):  
Nurashidah Musa ◽  
Ruben Gregory Xavier

Traumatic diaphragmatic hernia (TDH) is uncommon and it can be a result from both blunt and penetrating trauma. About to 1% to 7% of patients with blunt trauma sustained TDH. Left sided traumatic diaphragmatic hernia are much common compared to right side.TDH can present acutely or delayed with signs of respiratory distress of intestinal obstruction. The diagnosis was made with the aid of chest radiograph and computed topography (CT) abdomen. A coiled nasogastric tube within the hemithorax is a pathognomonic for TDH. We are presenting a case of high impact injury resulting in a TDH in a 19-year-old, malay male with unsure mechanism injury. He presented with generalised abdominal pain and in respiratory distress with a clinical evidence of abdominal tenderness and type 1 respiratory failure. Subsequently, he underwent exploratory laparotomy and repair of left diaphragmatic hernia. Intraoperatively, noted large linear tear of left hemidiaphragm posterolaterally extending medially until the insertion of falciform ligament. Stomach, left lobe of liver, spleen and splenic flexure of colon were herniated into the left hemithorax. The left diaphragmatic tear was repaired in 2 layers using prolene. A left subdiaphgramatic drain and a chest tube were inserted. Post operatively, the patient was nursed in ICU and recovered well. Repeated chest x -ray showed left lung was fully expanded. With aggressive chest physiotherapy and incentive spirometry, he recovered well and was discharged home. In trauma, there should be a high index of suspicion in patients with both respiratory and abdominal symptoms. Conclusion: Prompt recognition and early definitive management can improve patient outcomes.


2001 ◽  
Vol 56 (6) ◽  
pp. 173-178 ◽  
Author(s):  
Uenis Tannuri

PURPOSE: In previous papers, we described a new experimental model of congenital diaphragmatic hernia in rabbits, and we also reported noninvasive therapeutic strategies for prevention of the functional and structural immaturity of the lungs associated with this defect. In addition to lung hypoplasia, pulmonary hypertension, biochemical, and structural immaturity of the lungs, the hemodynamics of infants and animals with congenital diaphragmatic hernia are markedly altered. Hence, cardiac hypoplasia has been implicated as a possible cause of death in patients with congenital diaphragmatic hernia, and it is hypothesized to be a probable consequence of fetal mediastinal compression by the herniated viscera. Cardiac hypoplasia has also been reported in lamb and rat models of congenital diaphragmatic hernia. The purpose of the present experiment was to verify the occurrence of heart hypoplasia in our new model of surgically produced congenital diaphragmatic hernia in fetal rabbits. METHODS: Twelve pregnant New Zealand rabbits underwent surgery on gestational day 24 or 25 (normal full gestational time - 31 to 32 days) to create left-sided diaphragmatic hernias in 1 or 2 fetuses per each doe. On gestational day 30, all does again underwent surgery, and the delivered fetuses were weighed and divided into 2 groups: control (non-surgically treated fetuses) (n = 12) and congenital diaphragmatic hernia (n = 9). The hearts were collected, weighed, and submitted for histologic and histomorphometric studies. RESULTS: During necropsy, it was noted that in all congenital diaphragmatic hernia fetuses, the left lobe of the liver herniated throughout the surgically created defect and occupied the left side of the thorax, with the deviation of the heart to the right side, compressing the left lung; consequently, this lung was smaller than the right one. The body weights of the animals were not altered by congenital diaphragmatic hernia, but heart weights were decreased in comparison to control fetuses. The histomorphometric analysis demonstrated that congenital diaphragmatic hernia promoted a significant decrease in the ventricular wall thickness and an increase in the interventricular septum thickness. CONCLUSION: Heart hypoplasia occurs in a rabbit experimental model of congenital diaphragmatic hernia. This model may be utilized for investigations in therapeutic strategies that aim towards the prevention or the treatment of heart hypoplasia caused by congenital diaphragmatic hernia.


Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Atsuko Kato ◽  
Christian Drolet ◽  
Shi-Joon Yoo ◽  
Andrew Redington ◽  
Lars Grosse-Wortmann

Introduction: The left pulmonary artery (LPA) contributes more than the right (RPA) to total pulmonary regurgitation (PR) in patients after tetralogy of Fallot (TOF) repair, but the mechanism of this difference is not well known. We hypothesized that unilaterally increased pulmonary vascular resistance (PVR), resulting from lung compression by the enlarged and levorotated heart leads to greater PR in the LPA. This study aimed to analyze the interplay between heart and lung size, mediastinal geometry, and differential PR. Methods: This is a single-center retrospective analysis of 50 magnetic resonance studies in patients after TOF repair. Patients with more than mild discrete branch pulmonary artery stenosis were excluded. Blood flow was measured by phase-contrast velocity encoding within the branch pulmonary arteries. On the axial image with the largest total cardiac surface area, cardiac angle (α) between the thoracic anterior-posterior line and the interventricular septum, right and left lung areas as well as right and left hemithorax areas were measured (Figure). Results: There was no difference in LPA and RPA diameters. The LPA showed significantly less total forward flow (p=0.04), smaller net forward flow (p=<0.001), and greater RF (p=0.001) than the RPA. Left lung area was smaller than the right (p<0.001). RVEDVi correlated with LPA RF (R=0.48, p<0.001), but not with RPA RF. Larger RVEDVi correlated with a larger α angle (R=0.46, p<0.001), i.e. a more leftward cardiac axis and with smaller left lung area (R=-0.58, p<0.001). LPA RF, but not RPA RF, correlated inversely with left lung area indexed to the left hemithorax area (R=-0.34, p=0.02). Conclusions: An enlarged and levorotated heart - as a result of PR - is associated with smaller left lung size, and augments diastolic flow reversal in the LPA, presumably via increased left PVR. By imposing a further volume load on the RV, LPA regurgitation may thus close a positive feed-back loop of PR and RV dilatation.


Open Medicine ◽  
2014 ◽  
Vol 9 (1) ◽  
pp. 141-143
Author(s):  
Camelia Diaconu ◽  
Bianca Paraschiv ◽  
Răzvan Lungu ◽  
Daniela Bartoş

AbstractWe report the case of a 73 year old woman who presented for progressive dyspnea. Her medical history included thyroidectomy 15 years ago, myocardial infarction, recurrent paroxysmal atrial fibrillation and femoral fracture two weeks previously, conservatively treated. Physical examination revealed absent breath sounds in the left hemithorax, up to the apex, and crackles in the right hemithorax. The acid-base balance showed acute hypoxemic respiratory failure. The chest X-Ray revealed left diaphragmatic paralysis. Thoracic CT-scan was performed, which excluded the pulmonary embolism and revealed left diaphragmatic relaxation, ascension of the splenic angle of the colon, stomach and spleen up to the projection of left lung hilum, and right postero-basal alveolar condensation process. Diaphragm dysfunction can be caused by various disorders, including phrenic paralysis. This pathology should be considered in the differential diagnosis of acute respiratory failure.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Fanyi Gan ◽  
Liang Xia ◽  
Yushang Yang ◽  
Qiang Pu ◽  
Lunxu Liu

Abstract Background Congenital lobal emphysema (CLE) is a developmental lung abnormality usually diagnosed in the neonatal period and is rarely observed in adults. Adults with CLE are usually asymptomatic and only a small fraction may present with coughing, recurrent pneumonia and respiratory distress. In imaging studies, the most frequently affected lobe of CLE is the left upper lobe, followed by the right middle lobe. However, multilobar involvement with severe mediastinal shift is extremely rare. Case presentation We report a case of fatal CLE in a 28-year-old puerpera with postpartum respiratory failure. Chest computed tomography (CT) revealed emphysema of the right upper, middle and lower lobes resulting in adjacent atelectasis. Hyperinflation of the right upper lobe crossed the midline, leading to a deviation of the mediastinal structure to the left hemithorax and severe compression of the left lung. Conclusions Early and timely diagnosis of CLE with routine follow-up is necessary for patients. CLE, especially with multilobar involvement or mediastinal shift, could be life-threatening and should be promptly and aggressively treated to prevent severe complications.


Author(s):  
Aaron Teel ◽  
Benjamin Kwan ◽  
Mousumi Bhaduri

Introduction: Heteropagus twins are a set of conjoined twins with one being grossly abnormal, the ‘parasite’, and the other being relatively normal, the ‘autosite’. Case Report: A pair of heteropagus twins were initially identified on prenatal ultrasound and subsequently confirmed using prenatal MRI at 25 weeks gestational age. Prenatal imaging identified supernumerary limbs, accessory small bowel loops in the parasitic twin, a hypoplastic left heart in the right hemithorax of the autosite, and a shared liver and bowel between the two. Delivery occurred at 38 + 5 weeks gestation via C-section with an APGAR score of 9/9 for the autosite at both 1 and 5 minutes. MRI confirmed an interconnected autosite-parasite liver that was herniating into the left hemithorax as well as continuous autosite-parasite bowel loops. The patient underwent surgical management including resection of the parasitic twin and repair of the congenital heart defect and is now thriving. Discussion: Heteropagus twins include a spectrum of clinical entities from nonconjoined twins to intact conjoined twins. The predominant theory regarding etiology is an incomplete cleavage of a monozygotic embryo at approximately 2 weeks gestation. Following differentiation, ischemia-induced atrophy leads to the embryonic death of the parasite and hemodynamic changes in the autosite to support parasite tissues. The use of antenatal and postnatal imaging is crucial in establishing the diagnosis, management plan, and longterm prognosis secondary to the congenital cardiac malformations and degree of interconnectedness. Conclusion: This is the first reported case of heteropagus twins with co-existing hepatobiliary and gastrointestinal continuity.


1982 ◽  
Vol 53 (6) ◽  
pp. 1650-1652
Author(s):  
R. E. Drake ◽  
D. K. Adcock ◽  
R. L. Scott ◽  
J. C. Gabel

We have developed an electromechanical feedback system to control blood flow to the lower left lung lobe of dogs. Blood flow is measured with an electromagnetic flowmeter. The feedback system compares the blood flow signal to an adjustable reference voltage and causes a motor to turn. The direction of motor rotation depends on the relative magnitude of the flow signal and the reference. The motor pushes the plunger of a syringe that is attached to a balloon in the right pulmonary artery. Inflation of the balloon causes increased blood flow to the lower left lobe. We have used the system to control lobe blood flow in three dogs.


2021 ◽  
Vol 9 (4) ◽  
pp. 15-22
Author(s):  
V.I. Trykhlib ◽  
K.P. Bieliaieva ◽  
N.R. Tsyurak ◽  
L.O. Palatna

Literature and personal data on community-acquired pneumonia during the epidemic of a new coronavirus infection COVID-19 are presented. It was found that men and patients with moderately to severe form were treated for community-acquired pneumonia more often. The most common localizations were as follows (in descending frequency): bilateral multisegmental, right-sided lower lobe, left-sided lower lobe. Men as compared to women more often had the following localization of pneumonia: right-sided lower lobe pneumonia at the age of 20–30, 51–60; right-sided multisegmental in all age groups; left-sided lower lobe pneumonia at the age of 51–60, left-sided upper lobe pneumonia at the age of 20–50; left-sided multisegmental pneumonia in all age groups; slightly more frequent bilateral lower lobe pneumonia in different age groups; bilateral multisegmental pneumonia at the age of 20–30 and over 60. Conversely, the most common localization in women was as follows: right-sided lower lobe pneumonia at the age 31–50, right-sided upper lobe pneumonia at the age 20–30, left-sided lower lobe pneumonia at the age 20–50 and over 60, left-sided upper lobe pneumonia at the age over 60, bilateral lower lobe pneumonia at the age 31–40, bilateral multisegmental pneumonia at the age 41–60. Right-sided lower lobe pneumonia was equally often registered irrespective of gender in persons older than 60 years old. Right-sided upper lobe pneumonia was not registered in patients aged 31–40 and 41–50 years, and over 60 years old. The most frequent localization of pneumonia in mild form was left-sided lower lobe (35.3 %), right-si­ded lower lobe (20.6 %), and bilateral lower lobe (14.7 %). The least frequent were upper lobe localization of pneumonia in the right, left, and both lungs (2.94 % each), and left multisegmental pneumonia was not observed at all. The most frequent localization of pneumonia in the moderate form was the lower lobes of the right lung (29.4 %), left lung (18.3 %), and multisegmental in both lungs (28.8 %). It was least frequently registered in the upper lobe of the right lung (2.6 %), left lung (3.9 %) but was not registered in the upper lobes of both lungs. The most frequent localization of pneumonia in severe form was bilateral multisegmental (37.5 %). Right-sided multisegmental, left-sided lower lung and bilateral lower lung were the least common — 12.5 % for each. Subfebrile fever was registered more frequently in almost all localizations. More often normal or subfebrile temperature was registered in all localizations, except for right-sided upper lobe pneumonia, in which higher (febrile, pyretic) temperature was registered more often. In all localizations within three days after hospitalization most patients had normal leukocyte count, leukopenia was slightly more frequent in left-sided lower lobe pneumonia, leukocytosis — in the bilateral upper lobe, bilateral lower lobe, and right-sided upper lobe. In general, leukocytosis in community-acquired pneumonia was registered in 38.8 % of patients. In the first three days after hospitalization, a normal amount of lymphocytes was registered in the majority of patients, lymphopenia was observed in one-third of patients with right-sided upper and left-sided lower lobe localization.


2020 ◽  
Vol 8 (1) ◽  
pp. 420
Author(s):  
Indrajit Anandakannan ◽  
Shanthi Ponnandai Swaminathan ◽  
Vikas Kawarat ◽  
Rajeshwari Mani ◽  
Arul Kumar Chinnappan ◽  
...  

A traumatic diaphragmatic hernia is uncommon which accounts for 0.8 to 1.6%. In Blunt or penetrating abdominal injury, the patient presents as early or delayed respiratory distress or intestinal obstruction. We present the 55-year old female with a road traffic accident (pedestrian versus two-wheeler) with left-sided chest pain and breathlessness, left shoulder and leg pain referred to our institute. On examination, left hemithorax decreased breath sound and bowel sound was present, chest compression test positive, normal bowel sound in the abdomen, restricted left shoulder movement and abnormal mobility of shaft of left tibia and fibula. A plain X-ray of the chest and abdomen showed bowel shadow in the left hemithorax up to the apex. Computed tomography (CT) of thorax and abdomen shows herniation of stomach, transverse colon, omentum in the left hemithorax with collapsed left lung. A plain X-ray of the left shoulder shows neck of scapula fracture, left leg both bone fracture. Suggesting traumatic diaphragmatic hernia took emergency surgery, laparotomy was made intact stomach, transverse colon, omentum reduced with no injuries, radially placed diaphragmatic rent of size 10 cm × 5.5 cm through which left lung inferior lobe visualized, medial edge of rent close to the pericardial pad of fat. Other solid organs normal, left thoracic drain was fashioned. Rent was closed with interrupted polypropylene with intraabdominal drain. Left leg both bone fracture was done with tibial nailing and left neck of scapula fracture managed conservatively. Abdominal approach is sufficient rather than a thoracoabdominal approach given associated intraabdominal injuries, nowadays minimal access approaches preferred.


2020 ◽  
Vol 24 (4) ◽  
pp. 234-238
Author(s):  
Yu. Yu. Sokolov ◽  
D. V. Khaspekov ◽  
Oleg G. Topilin ◽  
M. I. Ayrapetyan ◽  
A. M. Efremenkov

A surgical management of extensive diaphragmatic defects is an actual problem in pediatric thoracic surgery. Despite of a large number of existing diaphragmatic prostheses, the search for an ideal plastic material is still going on. Material and methods. The authors analyze the effectiveness of xenopericardium in 15 children, aged 2 days - 17 years, with extensive diaphragmatic defects. Congenital hernia was in 8 (53.3%) patients, recurrence of congenital hernia - in 4 (26.7%) children, acquired hernia - in 3 (20.0%) children. The thoracoscopic approach was applied in 10 (66.7%) patients, thoracotomy - in 2 (13.3%) patients, laparotomy - in 3 (20.0%). Results. Diaphragmatic integrity was restored in all operated children. There were no any relapse of diaphragmatic hernia at the late postoperative period. There were no any xenopericardium rejection either. The article describes two clinical observations. In the first case, a 15-years-old girl with an extensive defect in her left dome of the diaphragm after the resection of lower lobe in the left lung and the diaphragmatic dome because of alveolar rhabdomyosarcoma had retoracotomy on the left and prosthetics of the diaphragm with a xenopericardium plate. In the second case, a thoracoscopic plastic surgery on the diaphragmatic dome with a xenopericardium plate was made in a newborn baby with aplasia of the right diaphragm on the 3rd day of his life. A long-lasting effect was obtained in both cases. Conclusions. Diaphragmatic plasty with xenopericardium can be successfully applied in children with extensive congenital and acquired diaphragmatic defects. The surgical technique with xenopericardium is possible both in open surgeries and in thoracoscopic interventions.


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