Fetal Bronchoscopy as a Useful Procedure in a Case with Prenatal Diagnosis of Congenital Microcystic Adenomatoid Malformation

2014 ◽  
Vol 37 (1) ◽  
pp. 75-80 ◽  
Author(s):  
Rogelio Cruz-Martinez ◽  
Antonio Méndez ◽  
Oscar Perez-Garcilita ◽  
Araceli Monroy ◽  
Karla Aguilar-Vidales ◽  
...  

Massive microcystic congenital cystic adenomatoid malformation (CCAM) and bronchial atresia are associated with a high perinatal mortality secondary to lung hypoplasia and cardiac dysfunction, and fetal intervention should be considered to improve prognosis. Therapeutic options include open fetal surgery with pulmonary resection, fetal sclerotherapy and fetoscopy. We present a case with a severely enlarged left lung without ultrasound signs of dilated airways compatible with the diagnosis of microcystic CCAM, hydrops and severe contralateral lung hypoplasia that was treated successfully at 30 weeks of gestation by fetal bronchoscopy, through which bronchial atresia was identified at the end of the left mainstem bronchi and permeabilized by laser ablation. After fetal surgery, weekly follow-up showed a progressive decrease in the affected lung size and an increase in the contralateral hypoplastic lung size, demonstrating normal dimensions of both lungs at 34 weeks of gestation, reversal of the mediastinal shift, and complete disappearance of hydrops. A healthy neonate was delivered uneventfully at term with no need for respiratory support, and the boy is now doing well at 15 months of age. This report demonstrates that in cases with prenatal diagnosis of large microcystic CCAM, fetal bronchoscopy can be used to refine the diagnosis of bronchial atresia and as a therapeutic tool with good outcome.

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.


2017 ◽  
Vol 8 (2) ◽  
pp. 89-92
Author(s):  
Rema Nambiar ◽  
Dinesh Nayak ◽  
Krupa H Shah ◽  
Shashikala K Bhat

ABSTRACT Congenital pulmonary airway malformation (CPAM) is a rare developmental lung abnormality. It is also referred as a congenital cystic adenomatoid malformation. The exact etiology is not known yet. The majority of cases are detected during targeted prenatal scan or within 2 years of the birth. Here, we report a case of CPAM type II diagnosed at 23 weeks of gestation, the key diagnostic features of which were mediastinal shift and lung lesion. The fetus had transient polyhydramnios and pericardial effusion. Glucocorticoids were administered for threatened preterm labor and CPAM. Healthy neonate was delivered at term without any immediate neonatal complications. How to cite this article Bhat SK, Nambiar R, Nayak D, Shah KH. Prenatal Diagnosis and Postnatal Management of Congenital Pulmonary Airway Malformation. Int J Infertil Fetal Med 2017;8(2):89-92.


2017 ◽  
Vol 7 (1) ◽  
pp. 1130-1132 ◽  
Author(s):  
S Shukla ◽  
H Kini ◽  
ML Ilias ◽  
K Gautam

Congenital cystic adenomatoid malformation is a hamartomatous lesion. A 30 year old woman, G2P1L1, in the 29th week of gestation presented with pain abdomen, chest pain, cough with expectoration, fever and inability to appreciate fetal movements of 2 days duration. Clinically, she had pneumonia. An ultrasound revealed a single fetus in breech presentation with features of hydrops fetalis, hypoplastic left lung, mediastinal shift to the left and poor diastolic and systolic flow in the umbilical artery.A still born male baby delivered subsequently was found at autopsy to have hydrops fetalis, supradiaphragmatic right sided extralobar sequestration with associated congenital cystic adenomatoid malformation.The right lung also showed congenital cystic adenomatoid malformation. There were no other associated anomalies. We present a rare case of extralobar sequestrationwith congenital cystic adenomatoid malformation.


2018 ◽  
Vol 25 (2) ◽  
pp. 95-100
Author(s):  
Julita Račaitė ◽  
Alina Šumkovskaja ◽  
Audronė Arlauskienė ◽  
Ingrida Pilypienė ◽  
Elena Landsbergytė-Bukauskienė

Background. A congenital cystic adenomatoid malformation (CCAM) is a foetal pulmonary development abnormality caused by airway dysgenesis that is characterized by cystic or adenomatous lesions in the terminal bronchioles. The size of the mass, the degree of the mediastinal shift, and the presence of hydrops and polyhydramnios can all affect the severity of a case. Treatment can be initiated at early stages by applying prenatal and postnatal methods. Because CCAM is a rare pathology that is often only accidentally diagnosed during routine ultrasounds, we would like to share our case report to enrich the literature on this pathology and to present a case successfully treated at our hospital. Materials and methods. A patient with her first multiple pregnancy was seen for prenatal care and her first ultrasound at 17 weeks of gestation. One of the twins was diagnosed with a congenital cystic adenomatoid malformation of the left lung. At 20 weeks of gestation, an enlarged left lung with small cysts, a compressed right lung, a compressed and displaced heart, and oligohydramnios were observed. At 28 weeks of gestation, a fetoplacental circulation disorder appeared. At 32 weeks of gestation, due the unstable condition of the affected foetus, the twins were delivered via a C-section. The treatment of the newborn included antibiotics, caffeine citrate, and breathing therapy. Results and conclusions. CCAM are often diagnosed by accident when performing routine pregnancy ultrasound examinations. CT is the most reliable X-ray-based examination method for confirming a diagnosis. When CCAM is suspected in the foetus, amniocentesis and cariotype identification are performed, but chromosomal anomalies related to CCAM are often not identified. Currently, the best treatment results have been achieved by applying combined prenatal therapy and early surgical treatment.


VASA ◽  
2016 ◽  
Vol 45 (6) ◽  
pp. 497-504 ◽  
Author(s):  
Tom De Beule ◽  
Jan Vranckx ◽  
Peter Verhamme ◽  
Veerle Labarque ◽  
Marie-Anne Morren ◽  
...  

Abstract. Background: The technical and clinical outcomes of catheter-directed embolization for peripheral arteriovenous malformations (AVM) using Onyx® (ethylene-vinyl alcohol copolymer) are not well documented. The purpose of this study was to retrospectively assess the safety, technical outcomes and clinical outcomes of catheter-directed Onyx® embolisation for the treatment of symptomatic peripheral AVMs. Patients and methods: Demographics, (pre-)interventional clinical and radiological data were assessed. Follow-up was based on hospital medical records and telephone calls to the patients’ general practitioners. Radiological success was defined as complete angiographic eradication of the peripheral AVM nidus. Clinical success was defined as major clinical improvement or complete disappearance of the initial symptoms. Results: 25 procedures were performed in 22 patients. The principal indications for treatment were pain (n = 10), limb swelling (n = 6), recurrent bleeding (n = 2), tinnitus (n = 3), and exertional dyspnoea (n = 1). Complete radiological success was obtained in eight patients (36 %); near-complete eradication of the nidus was achieved in the remaining 14 patients. Adjunctive embolic agents were used in nine patients (41 %). Clinical success was observed in 18 patients (82%). Major complications were reported in two patients (9 %). During follow-up, seven patients (32 %) presented with symptom recurrence, which required additional therapy in three patients. Conclusions: Catheter-directed embolisation of peripheral AVMs with Onyx® resulted in major clinical improvement or complete disappearance of symptoms in the vast majority of patients, although complete angiographic exclusion of the AVMs occurred in only a minority of patients.


2019 ◽  
Vol 1 (4) ◽  
Author(s):  
Yustinus Robby Budiman Gondowardojo ◽  
Tjokorda Gde Bagus Mahadewa

The lumbar vertebrae are the most common site for fracture incident because of its high mobility. The spinal cord injury usually happened as a result of a direct traumatic blow to the spine causing fractured and compressed spinal cord. A 38-year-old man presented with lumbar spine’s compression fracture at L2 level. In this patient, decompression laminectomy, stabilization, and fusion were done by posterior approach. The operation was successful, according to the X-Ray and patient’s early mobilization. Pneumothorax of the right lung and pleural effusion of the left lung occurred in this patient, so consultation was made to a cardiothoracic surgeon. Chest tube and WSD insertion were performed to treat the comorbidities. Although the patient had multiple trauma that threat a patient’s life, the management was done quickly, so the problems could be solved thus saving the patient’s life. After two months follow up, the patient could already walk and do daily activities independently.


Author(s):  
Arshed Hussain Parry ◽  
Mujahed Abdulsattar Ibrahim Raheem ◽  
Hussam Hassan Ismail ◽  
Osama Sharaf

Abstract Background Pulmonary 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 presentation A 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. Conclusion Presentation 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.


1999 ◽  
Vol 34 (5) ◽  
pp. 794-799 ◽  
Author(s):  
Kathleen van Leeuwen ◽  
Daniel H. Teitelbaum ◽  
Ronald B. Hirschl ◽  
Edward Austin ◽  
Susan H. Adelman ◽  
...  

1997 ◽  
Vol 2 (2) ◽  
pp. E13
Author(s):  
Jefferson V. Proaño ◽  
Ignacio Madrazo ◽  
Luis García ◽  
Elia García-Torres ◽  
Dolores Correa

The purpose of this study was to determine the therapeutic efficacy of albendazole and praziquantel administration in the treatment of neurocysticercosis of the fourth ventricle. The authors report the results obtained in 10 patients with cystic neurocysticercosis of the fourth ventricle who were treated with albendazole at a dosage of 15 mg/kg/day for 2 weeks. Because of the failure of albendazole treatment, two of the patients received an additional course of praziquantel at a dosage of 100 mg/kg/day for 2 weeks. A total of 16 courses of albendazole and two courses of praziquantel were administered to the 10 patients. In seven patients (70%), there was complete disappearance of the cyst, in two others (20%) there was an important decrease in the size of the cyst, and in one (10%), no change was seen. None of the patients had complications during the follow-up period of between 6 and 26 months (average 15.7 months). The authors postulate that a regimen of albendazole is the treatment of choice for this type of neurocysticercosis, although praziquantel may also be useful.


Author(s):  
Christina L. Herrera ◽  
John J. Byrne ◽  
David B. Nelson ◽  
Rachel C. Schell ◽  
Jodi S. Dashe

Objectives Our primary objective was to evaluate how prenatal diagnosis of a major fetal structural anomaly and resulting pregnancy outcome affected postpartum depression risk, as assessed by the Edinburgh Postnatal Depression Scale (EPDS). Secondary objectives were to review the rate of mental health follow-up and subsequent diagnosis of postpartum depression in screen-positive women. Study Design Singleton pregnancies with prenatal diagnosis of one or more major fetal structural anomalies were ascertained from prospectively maintained databases that included perinatal outcomes and subsequent EPDS responses from January 2010 to May 2018. EPDS scores of 13 or higher were considered positive and prompted referral for mental health follow-up, which was verified by medical record review. Statistical analyses were performed using Student's t-test, χ2, and odds ratios (ORs) with p < 0.05 considered significant. Results A total of 1,306 women had a prenatal diagnosis of one or more major fetal structural anomalies, 896 (68%) also had a postpartum EPDS screening, and 82 (9.2%) screened positive. Positive EPDS screening was more common with anomalies of multiple organ systems (16.5 vs 7.8%, p = 0.002) and aneuploidy (17.1 vs 9.3%, p = 0.02). Pregnancies complicated by fetal death, neonatal death, and termination for anomaly were significantly more likely to screen positive than those with neonatal survival to discharge (OR, 3.1 [95% confidence interval [CI], 1.6–6.2], 3.0 [95% CI, 1.5–5.8], and 4.4 [95% CI, 2.1–8.9], respectively, p ≤ 0.002). Of the 35 (43%) screen-positive women who attended follow-up appointments with mental health providers, 18 (51%) were diagnosed with a depressive disorder, accounting overall for 22% of those with a positive EPDS screen. Conclusion Among women with a prenatal diagnosis of a major fetal structural anomaly, those experiencing a perinatal loss or pregnancy termination have an increased risk of positive EPDS screen result compared with who have a neonate surviving to discharge. A depressive disorder was diagnosed postpartum in 22% of these women with a positive EPDS screen. Our findings highlight the mental health needs in this vulnerable population. Key Points


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