Unilateral Pulmonary Agenesis Research Articles

Scoliosis correction surgery in a patient with unilateral pulmonary agenesis: A case report and review of literature

Secondary scoliosis is caused by diseases wherein there is a lack of support for the spine, resulting in unequal pressures on the sides of the spinal cord. Secondary scoliosis is usually due to a neuromuscular disorder such as cerebral palsy, poliomyelitis, spina bifida, or a muscular dystrophy and is rarely secondary to pulmonary abnormalities. In this paper, we will specifically focus on the spine deformities associated with pulmonary diseases. We report a case of an adolescent female presenting with shoulder imbalance and marked scoliosis with a history of pulmonary agenesis. The patient underwent scoliosis correction surgery with instrumentation and fusion. Postoperatively, the patient showed improvement in the Cobb angle as well as marked improvement in her respiratory function. This review may raise awareness of the association between severe scoliosis and childhood lung absence and stimulate further prospective research efforts.

Prenatal Ultrasound Diagnosis of Unilateral Pulmonary Agenesis

Abstract Pulmonary agenesis (PA) is a rare developmental malformation, with a frequency of approximately 1 in 10–15,000 pregnancies. Unilateral PA is often associated with other congenital anomalies, whereas bilateral PA is fatal. Prenatal diagnosis is rare and is diagnosed more frequently in the postnatal period than in the prenatal period. Is a challenge that may be difficult as it shares similar features with other more common pathologies. Ultrasound plays a crucial role in early diagnosis and management. Ultrasonographic findings for a correct prenatal diagnosis include mediastinum displacement with the absence of parenchymal or cystic tissue, decreased thoracic volume, an elevated diaphragm, cardiac axis deviation, and a hemithoracic cavity largely occupied by the heart. Cases of right PA have a worse prognosis compared to left PA, probably due to higher frequency of cardiac and great vessel abnormalities. A rare case of early prenatal ultrasound diagnosis of unilateral fetal PA, at 18 weeks of gestation, is reported.

A case report of prenatal diagnostics and surgical correction of pulmonary agenesis associated with total anomalous pulmonary venous connection and vascular ring

Abstract Pulmonary agenesis is a rare malformation associated with congenital heart disease and may lead to a life-threatening condition and death without pre-planned surgical and medical support. We report an unusual case of unilateral pulmonary agenesis in combination with total anomalous pulmonary venous connection, complete vascular ring, retroaortic, retroesophageal innominate vein, secondary atrial septal defect, which was prenatally identified by fetal ultrasound and magnetic resonance imaging, later confirmed by postnatal computed tomography.

Unilateral pulmonary agenesis, esophageal atresia, and tracheo esophageal fistula: no more a fatal condition

The occurrence of unilateral pulmonary agenesis (PA) and esophageal atresia with and without tracheo esophageal fistula (EA/TEF) is exceedingly rare. The common origin of the esophagus and the lungs from the primordial foregut explains this association. This study reports on the accuracy of the preoperative investigations, operative approach, and a review of the literature and our own series.

Airway performance in infants with congenital tracheal stenosis associated with unilateral pulmonary agenesis: effect of tracheal shape on energy flux.

Congenital tracheal stenosis (CTS) with unilateral pulmonary agenesis (UPA) is characterized by the absence of one or both lungs in the hemithorax and is often associated with airway distortion. Some UPA patients have high mortality and morbidity even postoperatively, and it remains unclear whether surgery increases the energy flux needed to drive airflow. Here, we used pre- and postoperative patient-specific airway models to numerically investigate tracheal flow in patients with CTS, especially flow associated with right UPA (CTS-RUPA). Airflow was simulated with the large-eddy model, and energy flux was investigated to quantify airway performance and the contribution of surgical intervention. Although energy flux decreased postoperatively, clinical respiratory status did not improve. Standard surgical intervention for CTS, which expands the minimal cross-sectional area, decreased energy flux, i.e., improved airway performance. The simulation also included artificial airways with a straightened bend or reduced tracheal lumen roughness. The numerical results clearly showed interindividual differences in the percent reduction of energy flux caused by straightening the tracheal bend versus correcting tracheal lumen roughness. Although this study was limited to small sample size, these numerical results indicated that energy flux alone is insufficient to evaluate breathing performance in patients with CTS-RUPA but it can be used to estimate airway performance.

Unilateral Pulmonary Agenesis with Mayer-Rokitansky-Kuster-Hauser Syndrome: A Rare Association

Unilateral pulmonary agenesis is a rare congenital condition characterized by the absence of one or both lungs. Most of the cases have unilateral presentation as bilateral agenesis does not support life. Simultaneous Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome also has very rare presentation. It is characterized by congenital aplasia of the uterus and the vagina with normal female karyotype (46XX). A 30-year-old female, presenting with dyspnea from last 6 months and amenorrhea for last 15 years was further evaluated with gynecological examination, contrast-enhanced computed tomography thorax with the abdomen and karyotyping. She was eventually diagnosed as a case of Type I pulmonary agenesis with Type I MRKH syndrome. K

Surgical management in tetralogy of Fallot with rare unilateral pulmonary anomalies: A literature review.

Tetralogy of Fallot with unilateral pulmonary anomalies such as the unilateral absence of pulmonary artery or unilateral pulmonary agenesis is an extremely rare complex congenital heart anomaly. There is no established surgical algorithm for tetralogy of Fallot with concomitant unilateral pulmonary anomalies. This condition is still challenging, especially in the surgical field. In this review we also present our experiences in our center, Dr Cipto Mangunkusumo General Hospital, Jakarta, Indonesia. This literature review aimed to discuss systematic treatment options and hoped to help the decision-making process when surgeons face these rare anomalies.

Late presentation of unilateral lung agenesis in adulthood

BackgroundPulmonary agenesis is a rare congenital anomaly with a reported prevalence of about 1 in 100,000 births. It may be bilateral or unilateral. Among the unilateral form, left lung agenesis is more common (70%); however, it is the right lung agenesis which carries a dismal prognosis due to the frequent association with a gamut of other congenital anomalies and greater degree of mediastinal shift leading to tracheo-bronchial and vascular distortion. The patients of unilateral pulmonary agenesis usually present in infancy or early childhood. Presentation in late adulthood as seen in our patient is rare. We present a case of left pulmonary agenesis that was diagnosed in 4th decade of life.Case presentationA 36-year-old male presented with gradually progressive exertional dyspnea of 1 month duration. Clinical examination revealed tachycardia and tachypnea. Chest radiograph showed opaque left hemithorax with ipsilateral mediastinal shift. Computed tomography clinched the diagnosis by demonstrating absence of left main bronchus, lung and left pulmonary artery with shift of heart, and great mediastinal vessels into left hemithorax. The patient was managed conservatively and discharged with attachment to out-patient department for regular follow-up.ConclusionPresentation of unilateral lung agenesis in late adulthood, as seen in the present case is extremely rare. This case report highlights that, a rare condition like unilateral pulmonary agenesis, should be considered in the list of differentials in an adult presenting with opaque hemithorax with ipsilateral mediastinal shift on radiography.

Posterior Lung Herniation in Pulmonary Agenesis and Aplasia: Chest Radiograph and Cross-Sectional Imaging Correlation.

ObjectiveTo describe the anatomic locations and imaging features of posterior lung herniation in unilateral pulmonary agenesis and aplasia, focusing on radiograph-CT/MRI correlation.Materials and MethodsA total of 10 patients (seven with pulmonary agenesis and three with pulmonary aplasia, male: female = 1:9, mean age 7.3 years, age range from 1 month to 20 years) were included. Chest radiographs (n = 9), CT (n = 9), and MRI (n = 1) were reviewed to assess the type of lung underdevelopment, presence of anterior and posterior lung herniation, bronchus origin, supplying artery, and draining vein of the herniated lung.ResultsPulmonary agenesis/aplasia more commonly affected the left lung (n = 7) than the right lung (n = 3). Anterior lung herniation was observed in nine of the 10 patients. Posterior lung herniation was observed in seven patients with left pulmonary agenesis/aplasia. Two patients showed posterior lung herniation crossing the midline but not beyond the aorta, and five patients showed the posteriorly herniated right lower lobe crossing the midline to extend into the left hemithorax farther beyond the descending thoracic aorta through the space between the esophagus and the aorta. This anatomical configuration resulted in a characteristic radiographic finding of a radiolucent area with a convex lateral border and a vertical medial border in the left lower lung zone, revealing a tongue-like projection on CT and MRI.ConclusionPosterior lung herniation occurs in unilateral left lung agenesis/aplasia. Approximately 70% of the cases of posterior lung herniation reveal a unique radiolucent tongue-like projection in the left lower lung zone on imaging studies, which is caused by the extension of the posteriorly herniated right lung farther beyond the descending aorta.

Expanded Goldenhar Complex with Multiple Rare Syndromic Associations

Goldenhar syndrome (GS) is the most severe form of the oculoauriculovertebral spectrum, a developmental disorder primarily involving the structures derived from the first and second branchial arches, intervening first pharyngeal pouch and branchial cleft characterized by microtia, facial asymmetry, ocular abnormalities, and abnormalities of the vertebral column. A multifactorial etiology involving genetic and environmental factors has been proposed and the majority of the cases are sporadic in occurrence. The clinical phenotype of GS is highly variable and multiple associated anomalies of various organ systems have been reported in the literature. The term “Expanded Goldenhar Complex” has been used when there are associated unusual extrafacial abnormalities involving major organ systems such as the central nervous system, cardiovascular system, and respiratory system. We report a case of Expanded Goldenhar Complex with multiple anomalies, some of which are very rarely reported associations. A 5-year-old boy presented with right-sided hemifacial microsomia, microtia, preauricular and malar skin tags, bilateral radial club hands, and scoliosis since birth. Imaging revealed additional abnormalities including aural atresia, unilateral pulmonary agenesis, dextrocardia, vertebral segmentation defects, left renal ectopia, and right undescended testis. Surgical corrections were undertaken for the right radial club hand and undescended testis and he is awaiting surgery for the left club hand. Complete imaging evaluation of children with dysmorphic facies and limb abnormalities helps in identifying the various associated organ anomalies and to arrive at an accurate syndromic diagnosis. These patients have to be managed by a multidisciplinary team of clinicians on a case-to-case basis and have a good prognosis if there are no major cardiac defects or intellectual disability.

Mandepudi Geethika5

Mandepudi Geethika5

Unilateral pulmonary agenesis, with esophageal atresia long term results

Unilateral pulmonary agenesis, with esophageal atresia long term results

Prenatal diagnosis and misdiagnosis of fetus unilateral pulmonary agenesis by ultrasonography

Objective To study the expression of nuclear factor-kappaB (NF-κB) and the counts of lymph vessels in laryngeal squarnous cell carcinoma and polyps of vocal cord tissues, and explore their clinicopathologic significance and correlation in the course of laryngeal carcinoma. Methods SP immuno-histochemical method was used to detect the expression of NF-κB and the counts of lymph vessels on the routinely paraffln-embedded sections of the specimens from 50 cases laryngeal squamous cell carcinoma and 10 cases of polyps of vocal cord tissues. Results The positive rate of NF-κB and the counts of lymph ves-sels in laryngeal carcinoma[60. 0% ,( 13.3±3.4)/HP]were significantly higher ( P <0. 05 and P <0. 01respectively) than those in polyps of vocal cord tissues[10.0 % ,(6. 1±3. 8)/HP]. The positive rate of NF-κB and the counts of lymph vessels in well differentiated adenocarcinoma and cases without metastasis were significantly lower( P < 0. 05, P <0. 01 ), compared with poor-differentiated adenoearcinoma and ca-ses with metastasis. The counts of lymph vessels in the NF-κB positive cases were significantly higher than thoseinNF-κBnegativecases[(14.9±4.1)/HPvs (9.8±3.1)/ HP, P <0.01] . Conclusions The expression of NF-κB and the counts of lymph vessels might be important markers to be used to monitor the progression, biological behaviors, metastatic status and prognosis of laryngeal carcinoma. NF-κB might pro-mote lympoangiogenesis in laryngeal squnmous cell carcinoma tissues. Key words: Laryngeal neoplasms/ME/PA; Carcinoma, squamous cell/ME; NF-kappa B/ME; Lymphangiogenesis

CT features of lung agenesis \u2013 a case series (6 cases)

Back groundLung agenesis is a rare congenital anomaly. The main etiology of the disease is unknown whereas genetic, iatrogenic and viral factors as well as vitamin A deficiency during early pregnancy may result in developmental failure of primitive lung bud causing unilateral pulmonary agenesis. Affected patients usually present with variable respiratory symptoms and recurrent chest infection at any age. Plain film demonstrates opaque unilateral lung while chest CT scan can definitely diagnosis the disease. The anomaly has three types. Type I is pulmonary agenesis, type II is called pulmonary aplasia and type III is pulmonary hypoplasia.Cases’ presentationSix patients with main complaint of dyspnea underwent contrast enhanced chest CT in radiology department of French Medical Institute for Mothers and children, Kabul and were diagnosed lung agenesis.Three patients were categorized as type II pulmonary agenesis (aplasia). Two patients, three months old boy and a seven year- old girl demonstrated right lung aplasia. Another patient boy of eighteen years old presented with left lung aplasia.Two boys of four and seven months of age were classified as type I pulmonary agenesis (agenesis).A boy of one year old was diagnosed pulmonary agenesis type III, right lung hypoplasia.ConclusionSix patients were diagnosed with pulmonary agenesis by Chest CT scan. The clinicians should consider possibility of congenital pulmonary agenesis in dyspneic patients with opaque unilateral hemithorax in plain film.

Complex surgical treatment of congenital tracheal stenosis with associated unilateral lung agenesis

Abstract Background The combination of congenital tracheal stenosis (CTS) and unilateral pulmonary agenesis is rare and fatal. Especially, right lung agenesis is associated with dextrocardia and often causes tracheal alignment disorder. The mortality is estimated with 12.5–65%. Slide Tracheoplasty (ST) has been improved the prognosis, however, the postoperative course of CTS with single lung remains unclear. Here, we describe the postoperative challenging situation with bronchial intervention and other devices. Case presentation A 9-month-old girl with CTS, right lung agenesis, dextrocardia, and bilateral superior vena cava underwent ST anterior to the aortic arch. On postoperative day 50, tracheostomy was performed for long-term airway pressure support. Six months after the ST, she showed severe bronchomalacia at the bifurcation of the lobar bronchi, which was sandwiched between the descending aorta and left pulmonary artery. Direct bronchopexy and mobilization of left pulmonary artery resolved the severe bronchomalacia. Additionally, the kinked trachea caused a tracheal ulcer and frequent accidental decannulations. The ulcer was made above the aortic arch by contact with the tracheal cannula. The customized Montgomery T-tube solved both problems and enabled the stable management for airway. Her condition eventually improved, and she was discharged home after 14 months of hospitalization. Conclusion This case presented a very rare and critical condition. This is the first report to address the severe condition after ST anterior to the aortic arch. Surgical interventions or other devices for treatment are thought to be modified according to each condition.

Pulmonary hypertension in pulmonary agenesis-a report of five pediatric cases and review of previous cases

Pulmonary Agenesis (PA) is a rare congenital malformation of lung development, and patients with comorbid Pulmonary Hypertension (PH) have substantially increased morbidity and mortality risks. We describe five pediatric cases born with unilateral PA who developed PH. By integrating our findings with those of 7 previously published case reports, we aim to provide a more complete description of disease features, prognosis, and the most effective treatment strategies. We found that male PA patients and those with right-side agenesis were more likely to develop PH. Moreover, half of these comorbid patients had congenital heart disease with left-to-right shunt. Early diagnosis and treatment, including surgical and medical interventions, are critical for preservation of cardiopulmonary function. All PA cases should receive regular cardiopulmonary function tests starting at an early age.

Anesthetic Challenges in a Child with Pulmonary Agenesis: A Case Report and Review of Literature

Pulmonary agenesis is a rare congenital anomaly, which results in a complete absence in the development of lung tissue, bronchi, and pulmonary vessels. Pulmonary agenesis can occur on its own or it can often be found in conjunction with other congenital defects as part of VACTERL association. Anesthesia for patients with pulmonary agenesis can be challenging particularly when other defects or comorbidities exist. Here, we describe the successful anesthetic and ventilation management of a pediatric patient with right-sided unilateral pulmonary agenesis with VACTERL association.

Autopsy findings of a child with left atrial isomerism associated with pulmonary agenesis

ABSTRACT Isomerism is a rare laterality defect. Our aim was to describe the autopsy findings of a child with left atrial isomerism (LAI) and pulmonary agenesis (PA), an association still not described in the literature. Fetal ultrasound revealed right renal agenesis, single umbilical artery and polyhydramnios. Echocardiography revealed a complex heart defect with LAI. The child died minutes after birth. Autopsy confirmed the prenatal findings and revealed the presence of unilateral PA, complex vascular abnormalities and polysplenia. Despite its rarity, health professionals should be aware for the possibility of such an association.

Unilateral Pulmonary Agenesis – As a cause of respiratory distress in newborn

Pulmonary agenesis is a rare developmental defect which occurs as a result of failure of development of primitive lung bud. The onset & mode of presentation is highly variable ranging from asymptomatic cases to cases diagnosed in adulthood due to recurrent respiratory tract infections. Around 50% of cases are associated with other congenital anomalies.Isolated U/L pulmonary agenesis, if not associated with other congenital abnormalities may have fewer symptoms & long survival. Hereby we report a case of 2 days old female baby with right lung agenesis who presented with respiratory distress in neonatal period.

Unilateral left pulmonary agenesis with the presence of bilateral accessory lobes of lung

Unilateral left pulmonary agenesis with the presence of bilateral accessory lobes of lung