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Lung Pathology: Embryologic Abnormalities
Chapter2C Lung Pathology: Embryologic Abnormalities Content and Objectives Pulmonary Sequestration 2C-3 Chest X-ray Findings in Arteriovenous Malformation of the Great Vein of Galen 2C-7 Situs Inversus Totalis 2C-10 Congenital Cystic Adenomatoid Malformation of the Lung 2C-14 VATER Association 2C-20 Extralobar Sequestration with Congenital Diaphragmatic Hernia: A Complicated Case Study 2C-24 Congenital Chylothorax: A Case Study 2C-37 Continuing Nursing Education Test CNE-1 Objectives: 1. Explain how the diagnosis of pulmonary sequestration is made. 2. Discuss the types of imaging studies used to diagnose AVM of the great vein of Galen. 3. Describe how imaging studies are used to treat AVM. 4. Explain how situs inversus totalis is diagnosed. 5. Discuss the differential diagnosis of congenital cystic adenomatoid malformation. (continued) Neonatal Radiology Basics Lung Pathology: Embryologic Abnormalities 2C-1 6. Describe the diagnosis work-up for VATER association. 7. Explain the three classifications of pulmonary sequestration. 8. Discuss the diagnostic procedures for congenital chylothorax. 2C-2 Lung Pathology: Embryologic Abnormalities Neonatal Radiology Basics Chapter2C Lung Pathology: Embryologic Abnormalities EDITOR Carol Trotter, PhD, RN, NNP-BC Pulmonary Sequestration pulmonary sequestrations is cited as the 1902 theory of Eppinger and Schauenstein.4 The two postulated an accessory he clinician frequently cares for infants who present foregut tracheobronchia budding distal to the normal buds, Twith respiratory distress and/or abnormal chest x-ray with caudal migration giving rise to the sequestered tissue. The findings of undetermined etiology. One of the essential com- type of sequestration, intralobar or extralobar, would depend ponents in the process of patient evaluation is consideration on the timing of the accessory foregut budding (Figure 2C-1). -
Current Management and Outcome of Tracheobronchial Malacia and Stenosis Presenting to the Paediatric Intensive Care Unit
Intensive Care Med 52001) 27: 722±729 DOI 10.1007/s001340000822 NEONATAL AND PEDIATRIC INTENSIVE CARE David P.Inwald Current management and outcome Derek Roebuck Martin J.Elliott of tracheobronchial malacia and stenosis Quen Mok presenting to the paediatric intensive care unit Abstract Objective: To identify fac- but was not related to any other fac- Received: 10 July 2000 Final Revision received: 14 Oktober 2000 tors associated with mortality and tor. Patients with stenosis required a Accepted: 24 October 2000 prolonged ventilatory requirements significantly longer period of venti- Published online: 16 February 2001 in patients admitted to our paediat- latory support 5median length of Springer-Verlag 2001 ric intensive care unit 5PICU) with ventilation 59 days) than patients tracheobronchial malacia and with malacia 539 days). stenosis diagnosed by dynamic con- Conclusions: Length of ventilation Dr Inwald was supported by the Medical Research Council. This work was jointly trast bronchograms. and bronchographic diagnosis did undertaken in Great Ormond Street Hos- Design: Retrospective review. not predict survival. The only factor pital for Children NHSTrust, which re- Setting: Tertiary paediatric intensive found to contribute significantly to ceived a proportion of its funding from the care unit. mortality was the presence of com- NHSExecutive; the views expressed in this Patients: Forty-eight cases admitted plex cardiac and/or syndromic pa- publication are those of the authors and not to our PICU over a 5-year period in thology. However, patients with necessarily those of the NHSexecutive. whom a diagnosis of tracheobron- stenosis required longer ventilatory chial malacia or stenosis was made support than patients with malacia. -
The Role of Larygotracheal Reconstruction in the Management of Recurrent Croup in Patients with Subglottic Stenosis
International Journal of Pediatric Otorhinolaryngology 82 (2016) 78–80 Contents lists available at ScienceDirect International Journal of Pediatric Otorhinolaryngology jo urnal homepage: www.elsevier.com/locate/ijporl The role of larygotracheal reconstruction in the management of recurrent croup in patients with subglottic stenosis a,b,c, a,b a,d,e Bianca Siegel *, Prasad Thottam , Deepak Mehta a Department of Pediatric Otolaryngology, Childrens Hospital of Pittsburgh of UPMC, Pittsburgh, PA, USA b Children’s Hospital of Michigan, Detroit, MI, USA c Wayne State University School of Medicine Department of Otolaryngology, Detroit, MI, USA d Texas Children’s Hospital, Houston, TX, USA e Baylor University School of Medicine Department of Otolaryngology, Houston, TX, USA A R T I C L E I N F O A B S T R A C T Article history: Objectives: To determine the role of laryngotracheal reconstruction for recurrent croup and evaluate Received 16 October 2015 surgical outcomes in this cohort of patients. Received in revised form 4 January 2016 Methods: Retrospective chart review at a tertiary care pediatric hospital. Accepted 6 January 2016 Results: Six patients who underwent laryngotracheal reconstruction (LTR) for recurrent croup with Available online 13 January 2016 underlying subglottic stenosis were identified through a search of our IRB-approved airway database. At the time of diagnostic bronchoscopy, all 6 patients had grade 2 subglottic stenosis. All patients were Keywords: treated for reflux and underwent esophageal biopsies at the time of diagnostic bronchoscopy; 1 patient Laryngotracheal reconstruction had eosinophilic esophagitis which was treated. All patients had a history of at least 3 episodes of croup Recurrent croup in a 1 year period requiring multiple hospital admissions. -
Extralobar Pulmonary Sequestration Infected with Mycobacterium Gordonae
Brief Communications Extralobar pulmonary sequestration infected with Mycobacterium gordonae Yukio Umeda, MD, PhD,a Yukihiro Matsuno, MD, PhD,a Matsuhisa Imaizumi, MD, PhD,a Yoshio Mori, MD, PhD,a Hitoshi Iwata, MD, PhD,b and Hiroshi Takiya, MD, PhD,a Gifu, Japan Pulmonary sequestration is a malformation composed of lung was found except around the left lower pulmonary dysplastic lung tissue without normal communication with vein. We diagnosed it as extralobar sequestration and the tracheobronchial tree and with an anomalous systemic planned a simple excision of the sequestrated lung. We di- arterial supply.1 Few cases of pulmonary sequestration in- vided the aberrant artery and drainage vein and excised the fected with tuberculous or nontuberculous mycobacterium sequestrated lung using a linear stapler. The postoperative have been reported.2-4 However, all of those reports were course was uneventful. of intralobar pulmonary sequestrations. In the present article, Histopathologic study revealed destruction of alveolar we describe the first case of extralobar sequestration infected and reconstruction of respiratory epithelium within its own with Mycobacterium gordonae. pleura. The alveolar spaces were filled by mucoid or mono- nuclear cells. Caseating epithelioid granulomas and Lan- ghans’ giant cells were also observed (Figure 2). The CLINICAL SUMMARY diagnosis was of an extralobar pulmonary sequestration in- In August of 2005, a 72-year-old woman was referred to fected with Mycobacterium. M. gordonae was identified the Gifu Prefectural General Medical Center for abnormal from the culture of preoperatively collected sputum and sur- shadow on chest x-ray. Contrast media-enhanced com- gical specimen by the DNA–DNA hybridization method. -
Recurrent Pneumonia (Recurrent Lower Respiratory Tract Infections)
Recurrent Pneumonia (Recurrent lower respiratory tract infections) Guideline developed by Gulnur Com, MD, and Jeanne Velasco, MD in collaboration with the ANGELS team. Last reviewed by Jeanne Velasco, MD, on May 15, 2017. Key Points A single episode of uncomplicated pneumonia in an otherwise healthy child does not require investigation. Recurrent pneumonia is not an uncommon presenting symptom in general pediatric practice and one of the most common reasons for referral to pediatric pulmonologists. Recurrent pneumonia is usually defined as ≥2 episodes of pneumonia in a year or ≥3 in life.1 Many children with recurrent pneumonia do not need a full diagnostic work up, either because pneumonia episodes are not frequent or severe enough or because eventually children become asymptomatic. Evaluation of children with recurrent pneumonias begins by taking a careful history, an examination while the child is sick, and confirmation that the child is truly experiencing recurrent pneumonia. The majority of recurrent pneumonia causes in children have predictable risk factors (e.g., psychomotor retardation with feeding problems). Extensive investigations may not identify an underlying cause in up to 30% of children with recurrent pneumonia.1 The initial step in evaluating a child with recurrent respiratory symptoms includes distinguishing between recurrent wheezing versus recurrent infections. Studies show that asthma is being over diagnosed in children with recurrent respiratory symptoms. Patients with atypical asthma that does not respond to therapy should be investigated further. The evaluation of children with recurrent pneumonia should not be focused only on the respiratory tract. 1 Investigation for other organ system involvement may help for ultimate diagnosis (e.g., cystic fibrosis). -
Subglottic Stenosis: Current10.5005/Jp-Journals-10001-1272 Concepts and Recent Advances Review Article
IJHNS Subglottic Stenosis: Current10.5005/jp-journals-10001-1272 Concepts and Recent Advances REVIEW ARTICLE Subglottic Stenosis: Current Concepts and Recent Advances 1Oshri Wasserzug, 2Ari DeRowe ABSTRACT Signs and Symptoms Subglottic stenosis is considered one of the most complex and The symptoms of SGS in children are closely related to challenging aspects of pediatric otolaryngology, with the most the degree of airway narrowing. Grade I SGS is usually common etiology being prolonged endotracheal intubation. The asymptomatic until an upper respiratory tract infection surgical treatment of SGS can be either endoscopic or open, but recent advances have pushed the limits of the endoscopic occurs, when respiratory distress and stridor, which are approach so that in practice an open laryngotracheal surgical the hallmarks of SGS, appear. Grades II and III stenosis approach is considered only after failed attempts with an can cause biphasic stridor, air hunger, dyspnea, and endoscopic approach. In this review we discuss these advances, suprasternal, intercostal, and diaphragmatic retractions. along with current concepts regarding the diagnosis and Prolonged or recurrent episodes of croup should raise treatment of subglottic stenosis in children. suspicion for SGS. Keywords: Direct laryngoscopy, Endoscopic surgery, Laryngo- At times, the appearance of SGS may be insidious and tracheal surgery, Prolonged intubation, Subglottic stenosis. progressive. It is important to recognize that a compro- How to cite this article: Wasserzug O, DeRowe A. Subglottic Stenosis: Current Concepts and Recent Advances. Int J Head mised airway in a child can lead to rapid deterioration Neck Surg 2016;7(2):97-103. and require immediate appropriate intervention to avoid Source of support: Nil a catastrophic outcome. -
Stridor in the Newborn
Stridor in the Newborn Andrew E. Bluher, MD, David H. Darrow, MD, DDS* KEYWORDS Stridor Newborn Neonate Neonatal Laryngomalacia Larynx Trachea KEY POINTS Stridor originates from laryngeal subsites (supraglottis, glottis, subglottis) or the trachea; a snoring sound originating from the pharynx is more appropriately considered stertor. Stridor is characterized by its volume, pitch, presence on inspiration or expiration, and severity with change in state (awake vs asleep) and position (prone vs supine). Laryngomalacia is the most common cause of neonatal stridor, and most cases can be managed conservatively provided the diagnosis is made with certainty. Premature babies, especially those with a history of intubation, are at risk for subglottic pathologic condition, Changes in voice associated with stridor suggest glottic pathologic condition and a need for otolaryngology referral. INTRODUCTION Families and practitioners alike may understandably be alarmed by stridor occurring in a newborn. An understanding of the presentation and differential diagnosis of neonatal stridor is vital in determining whether to manage the child with further observation in the primary care setting, specialist referral, or urgent inpatient care. In most cases, the management of neonatal stridor is outside the purview of the pediatric primary care provider. The goal of this review is not, therefore, to present an exhaustive review of causes of neonatal stridor, but rather to provide an approach to the stridulous newborn that can be used effectively in the assessment and triage of such patients. Definitions The neonatal period is defined by the World Health Organization as the first 28 days of age. For the purposes of this discussion, the newborn period includes the first 3 months of age. -
A Case of Tracheal Stenosis As an Isolated Form of Immunoproliferative Hyper-Igg4 Disease in a 17-Year-Old Girl
children Case Report A Case of Tracheal Stenosis as an Isolated Form of Immunoproliferative Hyper-IgG4 Disease in a 17-Year-Old Girl Natalia Gabrovska 1,*, Svetlana Velizarova 1, Albena Spasova 1, Dimitar Kostadinov 1, Nikolay Yanev 1, Hristo Shivachev 2, Edmond Rangelov 2, Yanko Pahnev 2, Zdravka Antonova 2, Nikola Kartulev 2, Ivan Terziev 3 and Kaloyan Gabrovski 4 1 Department of Pulmonary Diseases, Multiprofile Hospital for Active Treatment of Pulmonary Diseases “St. Sofia”, Medical University–Sofia, 1431 Sofia, Bulgaria; [email protected] (S.V.); [email protected] (A.S.); [email protected] (D.K.); [email protected] (N.Y.) 2 Department of Pediatric Thoracic Surgery, Pediatric Surgery Clinic, University Multiprofile Hospital for Active Treatment and Emergency Medicine “N. I. Pirogov”, 1606 Sofia, Bulgaria; [email protected] (H.S.); [email protected] (E.R.); [email protected] (Y.P.); [email protected] (Z.A.); [email protected] (N.K.) 3 Department of Pathology, University Hospital ‘’Tsaritsa Uoanna–ISUL”, 1527 Sofia, Bulgaria; [email protected] 4 Department of Neurosurgery, University Hospital “St. Ivan Rilski”, Medical University–Sofia, 1431 Sofia, Bulgaria; [email protected] * Correspondence: [email protected]; Tel.: +359-887-931-009 Abstract: Immunoglobulin G4-related disease (IgG4-RD) is a lymphoproliferative disease which is described almost exclusively in adults. There are only a few pediatric patients who have been observed with this disorder. Here, we describe a rare case of IgG4-RD in a 17-year-old girl with Citation: Gabrovska, N.; Velizarova, a single manifestation—tracheal stenosis without previous intubation or other inciting event. -
Respiratory Complications and Goldenhar Syndrome
breathe case presentations.qxd 06/03/2007 17:56 Page 15 CASE PRESENTATION Respiratory complications and Goldenhar syndrome Case report W. Jacobs1 A 29-year-old female was referred to hospital A. Vonk Noordegraaf1 with progressive asthmatic complaints. On pre- R.P. Golding2 sentation, the patient had been experiencing J.G. van den Aardweg3 orthopnoea, an audible wheeze during daily P.E. Postmus1 activities, and sporadic coughing without spu- tum production. The patient had a history of recurrent airway infections and a 5-kg weight Depts of 1Pulmonary Medicine loss during the previous year, and had stopped and 2Radiology, Vrije Universiteit smoking several years before. She was known to Medisch Centrum, Amsterdam, have oculo-auriculo-vertebral (OAV) syndrome, and 3Dept of Pulmonary i.e. Goldenhar syndrome, which is a develop- Medicine, Medisch Centrum mental disorder involving mainly first and sec- Alkmaar, The Netherlands. ond branchial arch anomalies. On physical examination, she was not dys- pnoeic at rest, and had a respiratory rate of Correspondence: -1 -1 14 breaths·min , pulse 80 beats·min , blood W. Jacobs pressure 110/70 mmHg and temperature Dept of Pulmonary Medicine Figure 1 37.9°C. The left hemifacial structures and the Vrije Universiteit Medisch Centrum Postero-anterior chest radiograph. left hemithorax were underdeveloped. A chest Postbus 7057 examination revealed a systolic heart murmur 1007 MB Amsterdam grade 2/6 over the apex, and inspiratory and The Netherlands expiratory wheezing over both lungs. There was Task 1 Fax: 31 204444328 E-mail: [email protected] no oedema or clubbing. Arterial blood gas analy- Interpret the chest radiograph. -
Adult Outcome of Congenital Lower Respiratory Tract Malformations M S Zach, E Eber
500 Arch Dis Child: first published as 10.1136/adc.87.6.500 on 1 December 2002. Downloaded from PAEDIATRIC ORIGINS OF ADULT LUNG DISEASES Series editors: P Sly, S Stick Adult outcome of congenital lower respiratory tract malformations M S Zach, E Eber ............................................................................................................................. Arch Dis Child 2002;87:500–505 ongenital malformations of the lower respiratory tract relevant studies have shown absence of the normal peristaltic are usually diagnosed and managed in the newborn wave, atonia, and pooling of oesophageal contents.89 Cperiod, in infancy, or in childhood. To what extent The clinical course in the first years after repair of TOF is should the adult pulmonologist be experienced in this often characterised by a high incidence of chronic respiratory predominantly paediatric field? symptoms.910 The most typical of these is a brassy, seal-like There are three ways in which an adult physician may be cough that stems from the residual tracheomalacia. While this confronted with this spectrum of disorders. The most frequent “TOF cough” is both impressive and harmless per se, recurrent type of encounter will be a former paediatric patient, now bronchitis and pneumonitis are also frequently observed.711In reaching adulthood, with the history of a surgically treated rare cases, however, tracheomalacia can be severe enough to respiratory malformation; in some of these patients the early cause life threatening apnoeic spells.712 These respiratory loss of lung tissue raises questions of residual damage and symptoms tend to decrease in both frequency and severity compensatory growth. Secondly, there is an increasing with age, and most patients have few or no respiratory number of children in whom paediatric pulmonologists treat complaints by the time they reach adulthood.13 14 respiratory malformations expectantly; these patients eventu- The entire spectrum of residual respiratory morbidity after ally become adults with their malformation still in place. -
Idiopathic Subglottic Stenosis: a Review
1111 Editorial Section of Interventional Pulmonology Idiopathic subglottic stenosis: a review Carlos Aravena1,2, Francisco A. Almeida1, Sanjay Mukhopadhyay3, Subha Ghosh4, Robert R. Lorenz5, Sudish C. Murthy6, Atul C. Mehta1 1Department of Pulmonary Medicine, Respiratory Institute, Cleveland Clinic, Cleveland, OH, USA; 2Department of Respiratory Diseases, Faculty of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile; 3Department of Pathology, Robert J. Tomsich Pathology and Laboratory Medicine Institute, 4Department of Diagnostic Radiology, 5Head and Neck Institute, 6Department of Thoracic and Cardiovascular Surgery, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, USA Contributions: (I) Conception and design: C Aravena, AC Mehta; (II) Administrative support: All authors; (III) Provision of study materials or patients: All authors; (IV) Collection and assembly of data: C Aravena, AC Mehta; (V) Data analysis and interpretation: All authors; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors. Correspondence to: Atul C. Mehta, MD, FCCP. Lerner College of Medicine, Department of Pulmonary Medicine, Respiratory Institute, Cleveland Clinic, 9500 Euclid Ave, A-90, Cleveland, OH 44195, USA. Email: [email protected]. Abstract: Idiopathic subglottic stenosis (iSGS) is a fibrotic disease of unclear etiology that produces obstruction of the central airway in the anatomic region under the glottis. The diagnosis of this entity is difficult, usually delayed and confounded with other common respiratory diseases. No apparent etiology is identified even after a comprehensive workup that includes a complete history, physical examination, pulmonary function testing, auto-antibodies, imaging studies, and endoscopic procedures. This approach, however, helps to exclude other conditions such as granulomatosis with polyangiitis (GPA). It is also helpful to characterize the lesion and outline management strategies. -
Gastroesophageal Reflux in Patients with Subglottic Stenosis
ORIGINAL ARTICLE Gastroesophageal Reflux in Patients With Subglottic Stenosis David L. Walner, MD; Yoram Stern, MD; Mark E. Gerber, MD; Colin Rudolph, MD; Constance Y. Baldwin, BS; Robin T. Cotton, MD Objectives: To determine the incidence of gastroesopha- for reflux-associated symptoms have not been estab- geal reflux in patients with subglottic stenosis (SGS) and lished. Therefore, patients were grouped as having a pH to determine if upper esophageal reflux occurs in addi- of less than 4.0 in the upper esophagus for 0%, 0.1% to tion to lower esophageal reflux in these patients. 0.9%, 1.0% to 1.9%, 2.0% to 3.0%, or more than 3% of the study time. Design: Esophageal pH probe studies were reviewed in patients diagnosed as having SGS. Results: Thirty-seven of the 74 patients who under- went lower probe testing had a pH of less than 4.0 more Setting: A tertiary care pediatric medical center. than 5% of the study time, and 24 had a pH of less than 4.0 more than 10% of the study time. Twelve of the 55 Patients: All patients diagnosed as having SGS be- patients who underwent upper probe testing had no mea- tween January 1990 and July 1996 who had undergone surable reflux; 27 of the 55 had a pH of less than 4.0 more monitoring with an overnight esophageal pH probe. Sev- than 1% of the study time; 14 had a pH of less than 4.0 enty-four patients qualified for the study. All 74 pa- more than 2% of the study time, and 11 had a pH of less tients underwent lower probe testing, and 55 of the 74 than 4.0 more than 3% of the study time.