一例罕见膈疝影像学表现

Daniel Horton, HMS III Gillian Lieberman, MD An Unusual Presentation of Diaphragmatic Hernia Daniel B. Horton Harvard Medical School Year III Gillian Lieberman, MD January 2007 Daniel Horton, HMS III Gillian Lieberman, MD Patient LG: Clinical Presentation, Nov. 2004 • 52 year old woman presents with new nonproductive cough & dyspnea • PMH: Obesity, recurrent bronchitis • ROS: No GI or GU complaints, no history of prior trauma or major surgery • PE: Decreased breath sounds at left lung base Daniel Horton, HMS III Gillian Lieberman, MD Patient LG: Chest Radiograph, 11/26/04 ElevatedElevated hemidiaphragmhemidiaphragm Bowel gasBowel gas ShiftedShifted mediastinummediastinum PACS, BIDMC Bowel gasBowel gas Crowded vessels?Crowded vessels? AtelectasisAtelectasis?? Daniel Horton, HMS III Gillian Lieberman, MD DDx: Elevated Hemidiaphragm • Lung conditions “pulling” up diaphragm – Atelectasis – Prior lobectomy – Pulmonary disease, e.g., pulmonary fibrosis • Abdominal (and other) conditions “pushing” up diaphragm – Organ enlargement, e.g., splenomegaly, distended stomach – Inflammatory or infectious process, e.g., subphrenic abscess – (Subpulmonic effusion-“pushes” up lung from above diaphragm; not a truly elevated diaphragm) • Diaphragmatic defects – Eventration-muscular defect causing weakness – Paralysis-elevation and paradoxical movement – (Hernia-not a truly elevated diaphragm) PACS, BIDMC Daniel Horton, HMS III Gillian Lieberman, MD In fact, after a recent colonoscopy was limited by a redundant colon, patient LG underwent virtual CT colonoscopy which revealed her diagnosis… Daniel Horton, HMS III Gillian Lieberman, MD Patient LG: Virtual Colonoscopy Coronal CT, 8/3/04 PACS, BIDMC SCOUT VIEW Herniated bowelHerniated bowel Daniel Horton, HMS III Gillian Lieberman, MD Patient LG: Virtual Colonoscopy Axial CT, 8/3/04 AscAsc AortaAorta Main Main PulmPulm ArtArt FatFat SOFT TISSUE WINDOW ColonColon AtelectasisAtelectasis LUNG WINDOW Courtesy of Dr. Khasgiwala Daniel Horton, HMS III Gillian Lieberman, MD Amazingly, when this large diaphragmatic hernia was first diagnosed in August 2004, at age 52, LG was asymptomatic. Her respiratory symptoms would only begin several months later. Daniel Horton, HMS III Gillian Lieberman, MD Patient LG: Hospital Admission 12/28/04-1/7/05 • From November to December 2004, LG continued to have progressive dyspnea and cough, increasingly productive of greenish sputum, and intermittent fevers • Outpatient CT on 12/28/04 demonstrated pneumonia with LUL abscess and pleural effusion • Patient was admitted to BIDMC later that day for further work-up and management • CT-guided abscess drainage was performed on 1/1/05 Daniel Horton, HMS III Gillian Lieberman, MD Patient LG: CT-Guided Abscess Drainage, 1/1/05 PigtailPigtail CatheterCatheter PACS, BIDMC NON-CONTRAST AXIAL CT Daniel Horton, HMS III Gillian Lieberman, MD Patient LG: Axial Contrast CT, 1/3/05 Abscess w/ catheterAbscess w/ catheter EmpyemaEmpyema KidneyKidney CT, 5 months ago Courtesy of Dr. Khasgiwala Daniel Horton, HMS III Gillian Lieberman, MD Patient LG: Coronal and Sagittal Contrast CT, 1/3/05 AbscessAbscess w/ catheterw/ catheter EmpyemaEmpyema CORONAL SAGITTAL KidneyKidney ColonColon Courtesy of Dr. Khasgiwala ColonColon DiaphragmDiaphragm Daniel Horton, HMS III Gillian Lieberman, MD Patient LG: Hospital Admission 12/28/04-1/7/05 • Diagnosis – Bochdalek diaphragmatic hernia – Streptococcus milleri pneumonia complicated by LUL abscess and multiloculated empyema • Treatment – Abscess drainage – Antibiotics (CTX) • Elective diaphragmatic repair was deferred pending resolution of infection Daniel Horton, HMS III Gillian Lieberman, MD Let’s put our patient’s unusual presentation into a broader context Daniel Horton, HMS III Gillian Lieberman, MD Diaphragmatic Hernias (DH): Classification • Congenital – Bochdalek – Morgagni – Hiatus • Idiopathic • Acquired – Traumatic – Iatrogenic Daniel Horton, HMS III Gillian Lieberman, MD Diaphragmatic Hernias (DH): Classification • Congenital – Bochdalek – Morgagni – Hiatus • Idiopathic • Acquired – Traumatic – Iatrogenic Daniel Horton, HMS III Gillian Lieberman, MD Development of Diaphragm Week 14 Adapted from Sadler, Langman’s Medical Embryology, 9th Ed, 218. Week 9Week 7 GESTATIONAL AGE Pleuroperitoneal membrane Septum Transversum Pleuroperitoneal canal Body wall muscle ingrowth IVC Es Ao Defect leads toDefect leads to BochdalekBochdalek herniahernia Anterior defect leadsAnterior defect leads to to MorgagniMorgagni herniahernia Defect leadsDefect leads to hiatus herniato hiatus hernia Dorsal mesentery of esophagus Pleuroperitoneal fold Daniel Horton, HMS III Gillian Lieberman, MD Mature Diaphragm Diagram Esophageal HiatusEsophageal Hiatus MorgagniMorgagni foraminaforamina BochdalekBochdalek foraminaforamina INFERIOR SURFACE R L Moore and Agur, Essential Clinical Anatomy, 2nd Ed, 188. IVC Aorta Daniel Horton, HMS III Gillian Lieberman, MD Congenital DH: Bochdalek • Posterolateral defect through Bochdalek foramen • Most common congenital diaphragmatic hernia (1:2200 live births) Moore and Agur, Essential Clinical Anatomy, 2nd Ed, 188. Sadler, Langman’s Medical Embryology, 9th Ed, 220. • L>R>>bilateral – L: bowel, stomach, fat, spleen, kidney – R: liver, fat, kidney • Neonates with large hernias often present with respiratory distress due to poor fetal lung development Daniel Horton, HMS III Gillian Lieberman, MD Congenital DH: Morgagni • Anteromedial defect through Morgagni foramen • Rare (1:1,000,000 live births) • R>L • Often small, containing only fat, and asymptomatic Moore and Agur, Essential Clinical Anatomy, 2nd Ed, 188. Daniel Horton, HMS III Gillian Lieberman, MD Congenital DH: Hiatus • Herniation of stomach through esophageal hiatus • Overall rare in children • Of the three different subtypes of hiatus hernia (see below), paraesophageal is most common congenital form – Congenital paraesophageal hernias not often associated with complications (e.g., obstruction), as in adults Moore and Agur, Essential Clinical Anatomy, 2nd Ed, 188. http://coastalsurgery.com/news-hiatal_hernias.htm Type I “Sliding” Type II Paraesophageal Type III Mixed Daniel Horton, HMS III Gillian Lieberman, MD Imaging Pediatric DH • Prenatal ultrasound (US) • Neonatal radiographs – Contrast studies may help Daniel Horton, HMS III Gillian Lieberman, MD Companion Patient #1 Prenatal US: Congenital DH Hedrick & Adzick, UpToDate, 2006. DiaphragmDiaphragm StomachStomach LiverLiver DiaphragmDiaphragm Findings suggestive of DH: •Abdominal organs in thorax •Contralateral mediastinal shift •Small abdominal circumference HeadHeadHeadHead SAGITTAL HIGH-RESOLUTION FETAL ULTRASOUND Daniel Horton, HMS III Gillian Lieberman, MD Prenatal US: Congenital DH • Advantages – Routinely performed – Safe for woman and fetus – Early diagnosis • Search for other associated anomalies (prenatal karyotype, echo) • Opportunity for prenatal intervention (e.g., fetal tracheal occlusion) • Plan for delivery and critical postnatal care at tertiary hospital • Prepare parents psychologically • Disadvantages – User dependent – Limited resolution – May not detect smaller abnormalities Daniel Horton, HMS III Gillian Lieberman, MD Courtesy of Dr. Khasgiwala ShiftedShifted mediastinummediastinum BowelBowel CompressedCompressed LungLung ECG leadsECG leads ET TubeET Tube AP DDx: Bochdalek diaphragmatic hernia Congenital cystic adenomatoid malformation Cystic pulmonary interstitial emphysema Companion Patient #2 Neonatal Radiograph: Congenital Bochdalek DH Daniel Horton, HMS III Gillian Lieberman, MD Companion Patient #3 Neonatal Radiograph: Congenital Morgagni DH BowelBowel BowelBowel MediastinumMediastinum CompressedCompressed LungLung AP Lateral Courtesy of Dr. Khasgiwala Daniel Horton, HMS III Gillian Lieberman, MD Companion Patient #4 Neonatal Radiograph: Congenital Hiatus DH AP Karpelowsky, Wieselthaler, Rode, J Pediatr Surg 2006 41:1588-93. Lateral Cystic mass inCystic mass in posterior posterior mediastinummediastinum Daniel Horton, HMS III Gillian Lieberman, MD Companion Patient #5 Neonatal Barium Study: Congenital Hiatus DH Esophageal hiatusEsophageal hiatus Gastric Gastric fundusfundus EsophagusEsophagus Barium study performed to distinguish from other posterior mediastinal cystic masses, such as: •Epiphrenic diverticulum •Pulmonary cyst •Cystic tumor Karpelowsky, Wieselthaler, Rode, J Pediatr Surg 2006 41:1588-93. Daniel Horton, HMS III Gillian Lieberman, MD Neonatal Radiograph: Congenital DH • Advantages – Widely available and cheap – Demonstrates anatomy – Contrast studies (e.g., barium) may be used for confirmation – Can track progress and complications of interventions (e.g., lines, catheters, pulmonary disease, pre/post-op) • Disadvantages – Exposes child to radiation – Limits to identifying involved structures – If small hernias are missed by US and asymptomatic, they will not be detected Daniel Horton, HMS III Gillian Lieberman, MD And now let’s turn to other presentations of diaphragmatic hernias in adults, which sometimes recapitulate (if not represent) congenital phenotypes Daniel Horton, HMS III Gillian Lieberman, MD Diaphragmatic Hernias (DH): Classification • Congenital – Bochdalek – Morgagni – Hiatus • Idiopathic • Acquired – Traumatic – Iatrogenic Daniel Horton, HMS III Gillian Lieberman, MD Imaging Adult DH • Radiographs ± contrast • Cross-sectional studies: CT, MR – Characterize anatomy of hernia – Identify potential complications (e.g., respiratory, GI) – Directly identify diaphragmatic defect Daniel Horton, HMS III Gillian Lieberman, MD Idiopathic DH: Hiatus Hernias • Most common diaphragmatic hernia overall, usually of unclear etiology http://coastalsurgery.com/news-hiatal_hernias.htm Type I “Sliding” Type II Paraesophageal Type III Mixed – Type I (sliding) predominates • About half present with GERD • Usually medically managed – Other types (e.g., paraesophageal) less common • May present with obstruction due to volvulus • Surgical repair is indicated, even if incidental and asymptomatic Daniel Horton, HMS III Gillian Lieberman, MD Kahrilas, Pandolfino. GI Motility online 2006 | doi:10.1038/gimo48 Gastric Gastric rugalrugal foldsfolds EsophagusEsophagus A ringA ring Esophageal hiatusEsophageal hiatus Companion Patient #6 Barium Study: Type I Hiatus Hernia Daniel Horton, HMS III Gillian Lieberman, MD Idiopathic DH: Other Types • There are multiple case reports of Bochdalek and Morgagni hernias of unclear etiology in adults, which are diagnosed incidentally or because of symptoms • A retrospective review of 13,138 abdominal CT reports for adults patients at a large urban hospital identified incidental Bochdalek hernias in 0.17%, of which 27% involved solid or enteric organs Megremis et al., J Clin Ultrasound 2005;33:412-7. Mullins et al., AJR 2001;177:363-366. Daniel Horton, HMS III Gillian Lieberman, MD Companion Patient #7 Radiographs and CT: Morgagni DH in Asymptomatic 64yo Female Schubert H and Haage P. N Engl J Med 2004;351:e12 PA Lateral PARASAGITTAL CONTRAST CTRADIOGRAPH Arrow=R anterior cardiophrenic mass Asterisk=herniated mesenteric fat Arrowheads=anteromedial diaphragmatic defect Daniel Horton, HMS III Gillian Lieberman, MD Acquired DH: Trauma • Penetrating trauma – Direct injury to diaphragm causes rupture – Often undergo quick surgical repair • Blunt trauma – Impact may lead to direct or indirect injury of diaphragm • Increased abdominal pressure may push abdominal structures through a weakened diaphragm – Many hernias are missed early, and patients can present late with respiratory illness or GI complication (e.g., obstruction) – L>R>bilateral (R-sided protection of liver) Daniel Horton, HMS III Gillian Lieberman, MD Companion Patient #8 Radiograph & Axial CT: Traumatic DH in 47yo Male s/p MVA PA CHEST RADIOGRAPH (CONED DOWN) RibRib fracturesfractures BowelBowel EffusionEffusion AXIAL CONTRAST CT Eren, Kantarcı, Okur. Clinical Radiology 2006; 61:467-477. DiaphragmDiaphragm defectdefect RibRib fracturefractureHemothoraxHemothorax BowelBowel FatFat Daniel Horton, HMS III Gillian Lieberman, MD Acquired DH: Iatrogenesis • Thoraco-abdominal surgeries, esp. esophago- gastrectomy (e.g., for esophageal cancer), may lead to acquired DH – Similar presentation and complications to traumatic hernias Daniel Horton, HMS III Gillian Lieberman, MD Diaphragmatic Hernia Repair • Medical management suffices for most sliding hiatus hernias and small idiopathic hernias • Surgical repair is indicated for most pediatric, acquired, and otherwise symptomatic adult DH Daniel Horton, HMS III Gillian Lieberman, MD So what finally happened with our patient? Daniel Horton, HMS III Gillian Lieberman, MD Patient LG: Clinical Course • After resolution of infection with many months of antibiotic therapy (CTX followed by Levo/Clinda), LG underwent successful surgical repair of the diaphragm in July 2005 • Surgery revealed: – extensive adhesions from previous empyema – 8 x 5 cm defect in the posterolateral diaphragm consistent with “a congenital Bochdalek type hernia” (per operative report) Daniel Horton, HMS III Gillian Lieberman, MD PRE-OP 7/6/05 POST-OP 9/8/05 PleuralPleural effusioneffusion LinearLinear atelectasisatelectasis PACS, BIDMC PA Lateral Patient LG: Chest Radiograph Before and After Diaphragm Repair Key findings: •Repaired diaphragm •Normal mediastinum •Minor post-op changes Daniel Horton, HMS III Gillian Lieberman, MD Summary • Congenital diaphragmatic hernias can be classified as Bochdalek, Morgagni, or hiatus types • Prenatal imaging followed by neonatal radiographs represent the best tests to identify congenital diaphragmatic hernias and track the children’s clinical course • For adults with suspected idiopathic or acquired diaphragmatic hernias, radiographs ± contrast and cross-sectional imaging can best characterize the defects and their associated complications Daniel Horton, HMS III Gillian Lieberman, MD Acknowledgments • Gillian Lieberman, MD • Vaibhav Khasgiwala, MD • David Roberts, MD • Molly Collins • Alex Herrera • Pamela Lepkowski • Larry Barbaras, webmaster Daniel Horton, HMS III Gillian Lieberman, MD References • Eren S, Kantarcı M, and Okur A. Imaging of diaphragmatic rupture after trauma. Clinical Radiology 2006; 61:467-477. • Hedrick HL and Adzick NS. Congenital diaphragmatic hernia: Prenatal diagnosis and management. UpToDate 2006. • Juhl JH. Ch. 31 Diseases of the pleura, mediastinum, and diaphragm. Essentials of Radiologic Imaging, 6th Ed. Juhl JH, Crummy AB, Eds. 1993; Lippincott Company, Philadelphia. • Kahrilas PJ. Hiatus Hernia. UpToDate 2005. • Kahrilas PJ and Pandolfino JE. Hiatus hernia. GI Motility online 2006; doi:10.1038/gimo48. • Karpelowsky JS, Wieselthaler N, and Rode H. Primary paraesophageal hernia in children. J Pediatr Surg 2006;41:1588-93. • Mei-Zahav M, Solomon M, Trachsel D, and Langer JC. Bochdalek diaphragmatic hernia: not only a neonatal disease. Arch Dis Child 2003;88:532-5. • Megremis SD, Segkos NI, Gavridakis GP, Mattheakis MG, Kehayas EG, Triantafyllou LB, Sfakianaki EE, and Chalkiadakis GE. Sonographic appearance of a late-diagnosed left bochdalek hernia in a middle-aged woman: case report and review of the literature. J Clin Ultrasound 2005;33:412-7. • Moore KL and Agur AMR. Essential Clinical Anatomy, 2nd Ed. 2002. Lippincott Williams and Wilkins, Philadelphia. • Mullins ME, Stein J, Saini SS, and Mueller PR. Prevalence of incidental bochdalek's hernia in a large adult population. AJR 2001;177:363-366. • Sadler TW. Langman’s Medical Embryology, 9th Ed. 2004. Lippincott Williams & Wilkins, Philadelphia. • Schubert H and Haage P. Images in clinical medicine. Morgagni's hernia. NEJM 2004;351:e12. An Unusual Presentation of Diaphragmatic Hernia Patient LG: Clinical Presentation, Nov. 2004 Patient LG: Chest Radiograph, 11/26/04 DDx: Elevated Hemidiaphragm In fact, after a recent colonoscopy was limited by a redundant colon, patient LG underwent virtual CT colonoscopy which revealed her diagnosis… Patient LG: Virtual Colonoscopy Coronal CT, 8/3/04 Patient LG: Virtual Colonoscopy�Axial CT, 8/3/04 Slide Number 8 Patient LG: Hospital Admission�12/28/04-1/7/05 Patient LG: CT-Guided Abscess�Drainage, 1/1/05 Patient LG: Axial Contrast CT, 1/3/05 Patient LG: Coronal and Sagittal Contrast CT, 1/3/05 Patient LG: Hospital Admission�12/28/04-1/7/05 Let’s put our patient’s unusual presentation into a broader context Diaphragmatic Hernias (DH): Classification Diaphragmatic Hernias (DH): Classification Development of Diaphragm Mature Diaphragm Diagram Congenital DH: Bochdalek Congenital DH: Morgagni Congenital DH: Hiatus Imaging Pediatric DH Companion Patient #1 Prenatal US: Congenital DH Prenatal US: Congenital DH Companion Patient #2 Neonatal Radiograph: Congenital Bochdalek DH Companion Patient #3 Neonatal Radiograph: Congenital Morgagni DH Slide Number 27 Companion Patient #5 Neonatal Barium Study: Congenital Hiatus DH Neonatal Radiograph: Congenital DH And now let’s turn to other presentations of diaphragmatic hernias in adults, which sometimes recapitulate (if not represent) congenital phenotypes Diaphragmatic Hernias (DH): Classification Imaging Adult DH Idiopathic DH: Hiatus Hernias Slide Number 34 Idiopathic DH: Other Types Slide Number 36 Acquired DH: Trauma Companion Patient #8 Radiograph & Axial CT: Traumatic DH in 47yo Male s/p MVA Acquired DH: Iatrogenesis Diaphragmatic Hernia Repair So what finally happened with our patient? Patient LG: Clinical Course Patient LG: Chest Radiograph Before and After Diaphragm Repair Summary Acknowledgments References