An Unusual Presentation of Diaphragmatic Hernia

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1 January 2007 An Unusual Presentation of Diaphragmatic Hernia Daniel B. Horton Harvard Medical School Year III

2 Patient LG: Clinical Presentation, Nov 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

3 Patient LG: Chest Radiograph, 11/26/04 Shifted mediastinum Crowded vessels? Atelectasis? Elevated hemidiaphragm Bowel gas Bowel gas PACS, BIDMC

4 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

5 In fact, after a recent colonoscopy was limited by a redundant colon, patient LG underwent virtual CT colonoscopy which revealed her diagnosis

6 Patient LG: Virtual Colonoscopy Coronal CT, 8/3/04 SCOUT VIEW Herniated bowel PACS, BIDMC

7 Patient LG: Virtual Colonoscopy Axial CT, 8/3/04 Main Pulm Art SOFT TISSUE WINDOW Asc Aorta Colon Fat Atelectasis LUNG WINDOW Courtesy of Dr. Khasgiwala

8 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.

9 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

10 Patient LG: CT-Guided Abscess Drainage, 1/1/05 NON-CONTRAST AXIAL CT Pigtail Catheter PACS, BIDMC

11 Patient LG: Axial Contrast CT, 1/3/05 CT, 5 months ago Abscess w/ catheter Empyema Kidney Courtesy of Dr. Khasgiwala

12 Patient LG: Coronal and Sagittal Contrast CT, 1/3/05 CORONAL SAGITTAL Abscess w/ catheter Colon Empyema Colon Kidney Diaphragm Courtesy of Dr. Khasgiwala

13 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

14 Let s put our patient s unusual presentation into a broader context

15 Diaphragmatic Hernias (DH): Classification Congenital Bochdalek Morgagni Hiatus Idiopathic Acquired Traumatic Iatrogenic

16 Diaphragmatic Hernias (DH): Classification Congenital Bochdalek Morgagni Hiatus Idiopathic Acquired Traumatic Iatrogenic

17 Development of Diaphragm GESTATIONAL AGE Week 7 Week 9 Septum Transversum Week 14 Body wall muscle ingrowth Anterior defect leads to Morgagni hernia IVC Es Ao Dorsal mesentery of esophagus Pleuroperitoneal Pleuroperitoneal fold canal Defect leads to Bochdalek hernia Pleuroperitoneal membrane Defect leads to hiatus hernia Adapted from Sadler, Langman s Medical Embryology, 9th Ed, 218.

18 Mature Diaphragm Diagram INFERIOR SURFACE Morgagni foramina R Esophageal Hiatus L IVC Aorta Bochdalek foramina Moore and Agur, Essential Clinical Anatomy, 2 nd Ed, 188.

19 Congenital DH: Bochdalek Posterolateral defect through Bochdalek foramen Most common congenital diaphragmatic hernia (1:2200 live births) 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 Moore and Agur, Essential Clinical Anatomy, 2 nd Ed, 188. Sadler, Langman s Medical Embryology, 9th Ed, 220.

20 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, 2 nd Ed, 188.

21 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 Type I Sliding Type II Paraesophageal Type III Mixed Moore and Agur, Essential Clinical Anatomy, 2 nd Ed,

22 Imaging Pediatric DH Prenatal ultrasound (US) Neonatal radiographs Contrast studies may help

23 Companion Patient #1 Prenatal US: Congenital DH SAGITTAL HIGH-RESOLUTION FETAL ULTRASOUND Head Diaphragm Head Diaphragm Liver Stomach Hedrick & Adzick, UpToDate, Findings suggestive of DH: Abdominal organs in thorax Contralateral mediastinal shift Small abdominal circumference

24 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

25 Companion Patient #2 Neonatal Radiograph: Congenital Bochdalek DH AP Compressed Lung Shifted mediastinum ECG leads ET Tube Bowel DDx: Bochdalek diaphragmatic hernia Congenital cystic adenomatoid malformation Cystic pulmonary interstitial emphysema Courtesy of Dr. Khasgiwala

26 Companion Patient #3 Neonatal Radiograph: Congenital Morgagni DH AP Lateral Bowel Mediastinum Compressed Lung Bowel Courtesy of Dr. Khasgiwala

27 Companion Patient #4 Neonatal Radiograph: Congenital Hiatus DH AP Lateral Cystic mass in posterior mediastinum Karpelowsky, Wieselthaler, Rode, J Pediatr Surg :

28 Companion Patient #5 Neonatal Barium Study: Congenital Hiatus DH Gastric fundus Esophageal hiatus Esophagus 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 :

29 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

30 And now let s turn to other presentations of diaphragmatic hernias in adults, which sometimes recapitulate (if not represent) congenital phenotypes

31 Diaphragmatic Hernias (DH): Classification Congenital Bochdalek Morgagni Hiatus Idiopathic Acquired Traumatic Iatrogenic

32 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

33 Idiopathic DH: Hiatus Hernias Most common diaphragmatic hernia overall, usually of unclear etiology 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 Type I Sliding Type II Paraesophageal Type III Mixed

34 Kahrilas, Pandolfino. GI Motility online 2006 doi: /gimo48 Daniel Horton, HMS III Companion Patient #6 Barium Study: Type I Hiatus Hernia Esophagus A ring Gastric rugal folds Esophageal hiatus

35 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: Mullins et al., AJR 2001;177:

36 Companion Patient #7 Radiographs and CT: Morgagni DH in Asymptomatic 64yo Female PA RADIOGRAPH PARASAGITTAL CONTRAST CT Lateral Asterisk=herniated mesenteric fat Arrowheads=anteromedial diaphragmatic defect Arrow=R anterior cardiophrenic mass Schubert H and Haage P. N Engl J Med 2004;351:e12

37 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)

38 Companion Patient #8 Radiograph & Axial CT: Traumatic DH in 47yo Male s/p MVA PA CHEST RADIOGRAPH (CONED DOWN) AXIAL CONTRAST CT Effusion Rib fractures Diaphragm defect Fat Bowel Bowel Hemothorax Rib fracture Eren, Kantarcı, Okur. Clinical Radiology 2006; 61:

39 Acquired DH: Iatrogenesis Thoraco-abdominal surgeries, esp. esophagogastrectomy (e.g., for esophageal cancer), may lead to acquired DH Similar presentation and complications to traumatic hernias

40 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

41 So what finally happened with our patient?

42 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)

43 PRE-OP 7/6/05 Daniel Horton, HMS III Patient LG: Chest Radiograph Before and After Diaphragm Repair PA Lateral POST-OP 9/8/05 Linear atelectasis Key findings: Repaired diaphragm Normal mediastinum Minor post-op changes Pleural effusion PACS, BIDMC

44 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

45 Acknowledgments Vaibhav Khasgiwala, MD David Roberts, MD Molly Collins Alex Herrera Pamela Lepkowski Larry Barbaras, webmaster

46 References Eren S, Kantarcı M, and Okur A. Imaging of diaphragmatic rupture after trauma. Clinical Radiology 2006; 61: Hedrick HL and Adzick NS. Congenital diaphragmatic hernia: Prenatal diagnosis and management. UpToDate 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 Kahrilas PJ and Pandolfino JE. Hiatus hernia. GI Motility online 2006; doi: /gimo48. Karpelowsky JS, Wieselthaler N, and Rode H. Primary paraesophageal hernia in children. J Pediatr Surg 2006;41: Mei-Zahav M, Solomon M, Trachsel D, and Langer JC. Bochdalek diaphragmatic hernia: not only a neonatal disease. Arch Dis Child 2003;88: 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: Moore KL and Agur AMR. Essential Clinical Anatomy, 2nd Ed 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: Sadler TW. Langman s Medical Embryology, 9th Ed Lippincott Williams & Wilkins, Philadelphia. Schubert H and Haage P. Images in clinical medicine. Morgagni's hernia. NEJM 2004;351:e12.

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