Intrapericardial Diaphragmatic Hernia in Adults

RONALD L. MENG, M.D., ALBERT STRAUS, M.D., PH.D., FRANK MILLOY, M.D.,C. FREDERICK KITTLE, M.D., HIRAM LANGSTON, M.D.

Intrapericardial diaphragmatic hernia (IDH) is the rarest typeof adult diaphragmatic hernia. Only 28 cases have been re-ported. Indirect blunt trauma has been implicated in mostcases, but one resulted from a stab wound to the anterior chest.Patients presented immediately or up to 20 years followingtrauma with symptoms of intermittent bowel obstruction,including one with strangulation, or cardiac dysfunction, in-cluding dyspnea, palpitations, and two patients with cardiactamponade. Physical findings included bowel sounds in thechest, decreased heart and lung sounds, and an absent point ofmaximal cardiac impulse. Chest roentgenography usually re-vealed supradiaphragmatic gas shadows suggestive of bowel inthe chest. Thorough examination of both anteroposterior andlateral chest roentgenograms and barium gastrointestinal seriesmay provide positive diagnosis of anterior diaphragmatichernia, and fluoroscopy after induced pneumoperitoneum mayestablish its pericardial involvement. Celiotomy is the preferredapproach to surgical repair of IDH. Since the symptoms ref-erable to adult IDH can be incapacitating or life threatening,herniorrhaphy should be performed promptly upon diagnosis,with expectation of an uneventful recovery and negligible re-currence rate.

N ONHIATAL DIAPHRAGMATIC hernia in adults isalmost always secondary to trauma. Its incidence

is increasing with highspeed transportation accidentsand civilian warfare and is currently 3-7% in traumavictims.32 Many series of diaphragmatic hernia afterblunt and penetrating trauma have mentioned asso-ciated central tendon rupture and pericardial lacera-tion, but none has reported intrapericardial diaphrag-matic herniation of abdominal viscera (IDH).Only 26 cases of adult IDH have been reported in

the English literature (Table 1). It is not a variety ofMorgagni's hernia,4' but is instead a sacless herniadirectly through a rent in the pericardial portion of thediaphragm's central tendon. Because complications ofIDH may be life-threatening, surgical repair is desir-able. Since the defect is anterior in location, coveredsuperiorly by the heart, and usually unassociated withpleural involvement, repair is best performed trans-abdominally. Thus, it is important to secure the cor-

Reprint requests: Dr. Ronald L. Meng, Rush-Presbyterian-St.Luke's Medical Center, 1753 West Congress Parkway, Chicago,Illinois 60612.

Submitted for publication: May 10, 1978.

From the Departments of General Surgery andCardiovascular-Thoracic Surgery, Rush Medical

College and Rush-Presbyterian-St. Luke'sMedical Center, Chicago, Illinois

rect diagnosis preoperatively. This can be accom-plished by appropriate awareness of its possibility andproper roentgenographic examination. In order tofacilitate future diagnosis and treatment of this increas-ingly more frequent injury, we present two additionalcases of adult IDH supplemented by a review of thosepreviously reported.

Case Reports

Case 27. An 85-year-old man presented in the emergency room withsymptoms and physical findings suggestive of acute cholecystitis.Incidental findings included bowel sounds and diminished breathsounds in the anterior chest. Chest roentgenography revealedmultiple gas shadows overlying the cardiac silhouette on both antero-posterior and lateral views (Fig. 1). The tentative diagnosis ofIDH was confirmed at fluoroscopy, where ingestion of Gastro-grafin revealed that the fundus of the stomach was supradiaphrag-matic, well anterior to the esophageal hiatus, and within the peri-cardial cavity (Fig. 2). The patient underwent surgery for suspectedcholecystitis and coincidental IDH.

After midline celiotomy the inflamed gallbladder was removed.Dissection of mild adhesions to the parietal pericardium allowedwithdrawal of the body of the stomach, the omentum, the entiretransverse colon, and loops of jejunum from the pericardial cavitythrough a 10 cm transverse rent in the anterior portion of the peri-cardial diaphragm. The heart was directly exposed after reductionof the hernia contents; there was no peritoneal sac. The defect hadthickened, fibrotic edges and was limited solely to the pericardialdiaphragm, without pleural involvement. It was primarily closedwith interrupted Tevdec® sutures.

Postoperative serial chest films revealed slow resolution ofpneumopericardium such that by the sixth postoperative day thecardiac silhouette was normal. The postoperative course was un-eventful and there was no recurrence during a five year follow-up.After subsequent questioning the patient revealed that approximately20 years earlier he had sustained multiple injuries in a severe fall.

Case 28. Serial chest roentgenograms of a 41-year-old man whohad been treated in a sanitarium for pulmonary tuberculosis demon-strated markedly improved lung fields but also a variable cardiac sil-houette (Fig. 3). In all films, the abnormality was limited to the heartshadow (Fig. 4), and subsequent barium upper gastrointestinal studydemonstrated the stomach within the pericardial cavity.

0003-4932/79/0300/0359 $00.90 © J. B. Lippincott Company

359

MENG AND OTHERS Ann. Surg. March 1979

TABLE 1. Twenty Eight Cases ofAdult Intrapericardial Diaphragmatic Hernia

AnatomyClinical Data

Pa- Loca- Surgerytient Pertinent ationNum- Age Pertinent Physical History (Fig. Preoperativeber Author (Years) Sex Symptoms Abnormalities of Trauma Roentgenography Contents 5) Diagnosis Incision

I DeCardenal, 58 M Vomiting ? None None Omentum ? ? ?et al.'4 1903

2 Martland251909

70 M Dyspnea Absent PMI,I HT

None

Colon(strangu-

lated)

OmentumColonStomach

None C Autopsy

3 Keith24 1910 50 M

4 47 M

5 O'Brien31 1939 63 M

6 Astrup, et al.:'1951

50 F PainDyspnea

Bowel sounds over Noneprecordium,

Absent PMI,HT

?.) Omentum ? Autopsy

? Omentum ? Autopsy

? OmentumLiver

PAL: intrapericardial Omentumgas shadows Colon

LGI: IDH

J Autopsy

C IDH Abdomen

7 Crawshaw"21952

8 Brookes" 1965

9 Stein, et al.)81953

10 Rodgers,et al.36 1957

11 Smith, et al.371958

48 M Vomiting,Retrosternal gurgle,Weight loss,Dyspnea,Palpitations

48 M Vomiting,Retrosternal gurgle,Dyspnea,Palpitations

45 M Pain,Dyspnea,Palpitations

Absent PMI,HT

None

HT

53 F Pain,Left chest "Flopping'

34 M Vomiting None

Blow to right chest PAL: left chest gas Omentum1 year prior shadows Colon

UGI: DH StomachJejunum

Hit by bus 1 year PAL: left chest gas Omentumprior, multiple shadows Stomachtrauma

Auto accident PAL: enlarged heart, Omentum3 years prior, left chest gas Colonmultiple trauma shadows

UGI, LGI: DH

Auto accident I year PAL: left chest gas Omentumprior, multiple shadows Colontrauma UGI: DH Stomach

Liver

Auto accident 7years prior,multiple trauma

PAL: left chest gas Omentumshadows Colon

LiverJejunum

G DH Left chest

E DH Left chest

G DH Left chest

C DH Left chest

D DH Left chest

12 Moore29 1959 66 M Pain,Vomiting,Dyspnea,

13 Robb35 1963 72 M Pain,Retrosternal gurgle,Weight loss,Palpitations

BSleft chest

None

Auto accident 1 year PAL: left chest gas Omentumprior, multiple shadows Stomachtrauma Liver

Jejunum

Two severe falls 22 PAL: left chest gas Omentumyears prior, shadows Colonmultiple trauma UGI: DH Stomach

DH Left chest

G DH Left andrightchest

14 Wren, et al.481963

15 Herman,et al. 1965

56 M Pain, Bowel soundsVomiting, & BSDyspnea left chest

38 M Pain

16 Nelson30 1966 61 F Pain

17 Davis'3 1968 17 M PainDyspnea

Struck by falling PAL: left chest gas Omentumtree, acute, shadows, Colonmultiple trauma UGI: DH

BS left chest Auto accident, acute PAL: elevated left Omentummultiple trauma hemidiaphragm Colon

UGI: DH StomachPneumonperitoneum: LiverIDH

? Auto accident 1 year PAL: intrapericardial Omentumprior, multiple gas shadows Stomachtrauma UGI: IDH

? Hit by auto, acute, PAL: left chest gas Omentummultiple trauma shadows Colon

UGI: IDH

B DH Abdomen

B IDH Abdomen

i IDH Left chest

C IDH Abdomenand leftchest

360

?)

?)

?

?

'?

?

?

?

INTRAPERICARDIAL HERNIA

TABLE 1. (Continued)

AnatomyClinical Data

Pa- Loca- Surgerytient Pertinent ationNum- Age Pertinent Physical History (Fig. Preoperativeber Author (Years) Sex Symptoms Abnormalities of Trauma Roentgenography Contents 5) Diagnosis Incision

18 Beddingfield5 25 M Pain, Bowel sounds over Stab wound PAL: normal Omentum B Cardiac Left chest,

19 Wetrich,et al.46 1969

52 M Pain,Dyspnea

precordium, rigidabdomen, cardiactamponade

anterior midchest,acute

Bowel sounds over Auto accident,precordium acute, multiple

trauma

Jejunum

PAL: enlarged heart OmentumUGI: anterior DH Stomach

tamponade, abdomenperforatedabdominalviscus

F DH Left chest

18 M Vomiting Chest wall Auto accident, PAL: left chest gas

ecchymosis acute, multiple shadowstrauma UGI: IDH

OmentumStomach

E IDH Abdomen

21 Bank, et al.41971

22 Coats, et al."1972

23 Haider, et al.2"1973

24 Borrie, et al.'1974

25

62 M Pain,Vomiting,Dyspnea

41 F Dyspnea

28 M Pain,Weight loss

42 M Pain,Dyspnea

37 M Comatose

I HT, bowel sounds Auto accident I year PAL: intrapericardial Omentumover precordium prior, multiple gas shadows Colon

trauma UGI, LGI: IDH Stomach

None Hit by car, acute, PAL: left chest gas

multiple trauma shadows

Absent PMI None

Shock, rigid Struck by fallingabdomen crane, acute,

multiple trauma

i BS left chest, Auto accident,rigid abdomen acute, multiple

trauma

PAL: enlarged heartangiocardiography-mass withinpericardium

PAL: left chest gas

shadows

PAL: left chest gas

shadows

OmentumColonStomachLi verSpleen

Omentum

OmentumStomach

OmentumStomach

H IDH Left chest

A DH Left chest

i Pericardial Left chestmass and ab-

domen

H DH Left chest

H DH Left chest

26 Melzig, et al.221976

27 Meng, et al.1978

28

10 M Comatose

85 M None

41 M None

BS left chest Hit by car, acute, PAL: elevated leftmultiple trauma hemidiaphragm,

gas shadows &Levine tube in leftchest

Bowel sounds over Severe fall 20 years PAL: intrapericardialprecordium prior, multiple gas shadows

trauma UGI: IDH

Bowel sounds over Hit by auto 10 years

precordium prior, multipletrauma

PAL: variable cardiacsilhouette, intra-pericardial gas

shadowsUGI: IDH

Omentum H DH Abdomen

OmentumColonStomachJejunum

OmentumColonStomachLiver

C IDH Abdomen

C IDH Abdomen

Abbreviations: M = male: F = female; PMI = point of maximal cardiac impulse;HT = decreased heart tones; IDH = intrapericardial diphragmatic hernia; DH = dia-

phragmatic hernia; PAL = chest roentgenogram; UGI,intestinal series; I BS = decreased breath sounds.

LGI = upper and lower gastro-

Midline celiotomy disclosed a large anterior diaphragmatic defectwhich was traversed by the body of the stomach, the omentum, thetransverse colon, loops of jejunum, and the left lobe of the liver.Upon reduction of these organs it was clear that the hernia com-

municated solely and directly with the pericardial cavity. There was

no sac, but massive adhesions. The defect was closed by interruptedsilk suture.The postoperative course was uncomplicated and no recurrence

developed during a six year follow-up. The patient subsequently dis-closed that after being hit by a car approximately 10 years earlier hehad been hospitalized for coma and multiple fractures.

Review

Although Guthrie'9 noted a traumatic peritoneoperi-cardial communication in a soldier who died in the Bat-tle of Waterloo, DeCardenal, Grenier, and Bour-deron14 were the first to report and fully describe adultIDH. The patient was operated for a bowel obstruc-tion and underwent removal of strangulated colon fromthe pericardium. In 1909, Martland25 reported the first

Vol. 189 * No. 3

1968

361

Dyspnea

20

Ann. Surg. * March 1979MENG AND OTHERS

FIG. 1. The anteroposteriorand lateral plain chest roent-genograms reveal multiplegas shadows overlying thecardiac silhouette (patient27).

of four autopsy cases revealing IDH. He proposedfaulty development of the septum transversum as theetiology, and the discovery of several animal exam-

FIG. 2. The lateral Gastrografin upper gastrointestinal roentgeno-gram reveals the supradiaphragmatic portion of the stomach anteriorto the esophageal hiatus and overlying the cardiac silhouette (pa-tient 27).

ples3' supported this theory. In 1951, however, Astrupand Zeisler,3 reporting the first case preoperativelydiagnosed and successfully repaired, assumed traumaas the etiology despite lack of compatible patienthistory. The following year Crawshaw12 reported thefirst case of adult IDH after documented trauma, andsince then, all but one of the 19 reported IDH have beenassociated with prior trauma. The addition of our twocases brings the total number of reported adult IDHto 28.

Etiology and Pathophysiology

Trauma is the major and perhaps only cause of adultIDH. Twenty of the 28 patients had suffered blunttrauma: deceleration injuries during transportation ac-cidents in 15, severe blows to the chest in three, andsevere falls in the remaining two. An additional patienthad sustained an anterior chest stab wound. The re-maining seven cases included three autopsy studieswithout medical histories and one other autopsy studyand three successfully treated patients in whom nohistory of prior trauma could be elicited.Traumatic rupture of the diaphragm depends on

diaphragmatic anatomy and type of injury. The dia-phragm is covered inferiorly by peritoneum except atthe bare area of liver and superiorly by pleura except atthe pericardium. The right and left leaves are composedof muscle, but the central tendon, which is variable insize and shape, is composed of strong decussatingfibers.The force of blunt abdominal trauma is transmitted

through the viscera to the diaphragm.2 Most diaphrag-matic hernias occur on the left because the stomach,spleen and left kidney offer less protection than the

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Vol. 189 . No. 3

FIG. 3. The cardiac sil-houette varies in contourand content on serialanteroposterior chest films(patient 28).

INTRAPERICARDIAL HERNIA

liver and right kidney. IDH is even less frequent thanright diaphragmatic hernia because of the additionalforce absorption by the heart and the extra toughnessof the pericardial diaphragm.32 Anteroposterior orlateral compression of the chest may cause shearing ofthe diaphragm at the left parapericardial area39 since theinsertion of diaphragmatic muscle onto central tendonis comparatively weak.IDH after blunt trauma may occur as part of massive

bilateral diaphragmatic disruption, as extension of atear of either hemidiaphragm, or as an isolated injury(Fig. 5). The consistent involvement of the left centralportion of the pericardial diaphragm suggests a com-mon site of potential relative weakness. Shifting of theabdominal viscera may in some way increase the sus-ceptibility of this area to injury. Certainly powerful

FIG. 4. The anteroposteriorand lateral plain chest roent-genograms reveal multiplegas shadows and homoge-nous densities overlyingthe cardiac silhouette (pa-tient 28).

forces must be applied to rupture the pericardial dia-phragm; 19 of the 20 bluntly traumatized patients sus-tained multiple severe injuries.The mechanism of IDH following penetrating

trauma is easier to explain. Laceration of the peri-cardial diaphragm may result after any stab or gun-shot wound of the anterior chest or epigastrium. How-ever, 95% of patients sustaining pericardial lacerationsuffer cardiac injury as well,6 and many expire. Fur-thermore, many abdominal stab wounds which involvethe pericardium are extraperitoneal.23'43 These factorsaccount for the rarity of IDH secondary to penetratingtrauma.

In the four adults without history of trauma the IDHwere considered congenital. Coexistent pericardial anddiaphragmatic defects comprise one class of upper

363

Ann. Surg. * March 1979MENG AND OTHERS

FIG. 5. This schematic diagram of the diaphragm as seen from theperitoneum reveals the locations of the reported IDH (Table 1).There is common involvement of the left-central portion of the peri-cardial diaphragm.

celosomias, an entire spectrum of anomalies resultingfrom improper development of the cephalic foldsomatopleure.16 An early embryologic accident pro-

duces Cantrell's pentalogy,34 consisting of sternal,abdominal wall, pericardial, diaphragmatic and cardiacmalformations, and later embryologic errors produceomphalocele and pleuropericardial and peritoneoperi-cardial defects in any combination.16'17'27

Several neonates10"15'24'42'47 and a five-year-old18 withIDH have been reported, and all of these patientswere highly symptomatic very early in life. It seems

unlikely that the IDH in the four patients withouthistories of trauma could be asymptomatic for 28-70years, and it has been suggested that these historiesmight be incomplete or that the responsible traumaticevents might have been so mild as to be forgotten.Rarely, diaphragmatic hernias have been reported aftersuch mild mishaps as stumbling without falling.32 How-ever, Bochdalek hernias have been reported in adultswho had been asymptomatic for many years,32 and our

85-year-old patient (case 27) had been asymptomaticfor 20 years after his injury. Thus, the congenitaletiology of IDH in adults must be accepted; the lack ofa history of trauma should not rule out IDH.20

Regardless of the cause of the defect, peritardialdiaphragmatic rupture allows free transmission of thenegative pleural pressure to the peritoneum. The result-ing gradient, though only 7-20 cm of water at rest, in-creases greatly during vigorous respiratory effort andencourages herniation.2'39 Although small tears mayheal, larger tears are usually filled initially with omen-

tum, which prevents healing and initiates stretching ofthe defect and the pericardial sac. This enlargementallows further herniation and further enlargement. Pa-

tients with isolated IDH who were immediately diag-nosed had half as many organs herniated as did thosewho were diagnosed after one year. Huge tears, how-ever, allowed immediate multiple organ herniation.Omentum was present in all IDH and was the only

content in four patients. Transverse colon and stomachwere each herniated in halfthe cases, jejunum in a third,and left lobe of liver in a fifth. In a single patient thespleen was herniated. Since the pericardial diaphragmis a tightly-fused single layer, laceration involves its fullthickness and allows sacless herniation. Adhesionswere encountered in most chronic hernias.

Clinical Manifestations

The clinical manifestations of adult IDH depend onherniation of abdominal viscera. In the acute phase(from injury through apparent recovery) other more ob-vious injuries usually take priority, and the IDH maybe overlooked. Only ten of the 21 adult IDH secondaryto documented trauma were discovered during thisphase. The related symptoms in eight patients werenonspecific, including chest or abdominal pain (75%),dyspnea (75%), or vomiting (25%), and the remaining twopatients were comatose. Physical examination wasunhelpful in two patients, but bowel sounds in the chestand decreased breath sounds provided diagnosis ofdiaphragmatic hernia in the others. One patient raisedsuspicion by his markedly scaphoid abdomen. Anotherpatient (case 18) exhibited cardiac tamponade andbowel sounds in the chest after an anterior chest stabwound. Diaphragmatic hernia and hemopericardiumwere diagnosed, but pericardiocentesis failed to obtainfluid or relieve the tamponade. During subsequentinduction of general anesthesia, the patient sustained acardiac arrest which promptly responded to externalcardiac massage. After removal ofjejunum and omen-tum from the IDH, the failure of pericardiocentesisand the success of sternal compression in the reductionof the tamponading hernia became obvious.

Certainly early misdiagnosis can occur despite a highindex of suspicion and thorough investigation. A pa-tient with a tiny IDH containing only omentum, forexample, could be free of symptoms, physical findings,and perhaps even roentgenographic abnormality.Thus, certain patients enter the interval phase of IDH,which is characterized by relatively stable hernia con-tent and variable intermittent symptomatology. Inthese 11 patients the onset of symptoms varied fromone to 20 years after injury. According to the size andcontent of the hernia, symptoms were occasionally ab-sent (two patients), but usually were incapacitating oreven life-threatening. Gastrointestinal complaints in-cluded epigastric pain (55%), intermittent vomiting

364

Vol. 189 * No. 3 INTRAPERICARDIAL HERNIA 365with or without distension (45%), "flopping" or gur-gling in the chest (36%), and weight loss (18%). Thepains were often aggravated by eating and alleviated bybelching or vomiting because of visceral distensionfrom transient obstruction with subsequent improve-ment. Cardiac compression resulted in palpitations anddyspnea (45%), which were aggravated and alleviatedin a similar pattern. Physical examination was un-revealing in three patients, but in the remainder re-vealed decreased breath sounds, bowel sounds withinthe chest, muffled heart tones, and absent point ofmaximal cardiac impulse. During an obstructiveepisode, the physical findings varied according to thelevel of obstruction: distension was absent if thestomach or proximal jejunum was obstructed, but quitemarked if the transverse colon was involved.

Diagnosis

Patients with IDH have often been misdiagnosedwith unfortunate results. One patient (case #9) be-came a cardiac cripple because of the misdiagnosis ofischemic heart disease. Another (case 2) expired be-cause of cardiac tamponade misdiagnosed as severeorganic heart disease. Finally, one patient (case 1) ex-pired despite removal of strangulated colon from theIDH.

Suspicion of IDH based on history and physicalexamination demands investigation. Subsequent diag-nostic studies must take an appropriate place withdiagnosis and treatment of coexistent injuries, but thecomplications associated with IDH make its diagnosismandatory.Although IDH was preoperatively diagnosed in only

eight of the 22 patients, roentgenographic evidence ofIDH had been present retrospectively in nearly all.Since IDH is simply a variant of and may indeed beassociated with intrapleural diaphragmatic hernia, thecriteria for roentgenographic diagnosis are similar.9Extraneous gas bubbles or homogenous densitieswithin the heart shadow on anteroposterior and lateralplain films may cause apparent pneumopericardium orvariable cardiac silhouette. The tip of a nasogastrictube may rest within a supradiaphragmatic portion ofthe stomach overlying the cardiac silhouette, andbarium upper and lower gastrointestinal fluoroscopymay demonstrate opacification of intrapericardialorgans. Despite these procedures, however, the intra-pericardial nature of a diaphragmatic hernia may re-main unclear if only omentum is herniated, for exam-ple. In this instance, induced pneumoperitoneum maysuccessfully reveal peritoneopericardial communica-tion by causing pneumopericardium.22 This technique,however, carries the risk of pneumothorax, since asso-

ciated pleural tears are not uncommon, and is rarelynecessary.

Pericardiocentesis might obtain gastrointestinal con-tents or no fluid at all.5'20 If IDH is considered, peri-cardiocentesis is contraindicated, and if done for reliefof tamponade, its failure should suggest IDH. Otherprocedures such as cardiac angiography, catheteriza-tion, echography, and phonography have failed to aid indiagnosis of IDH.20The main differential diagnosis is Morgagni's her-

nia,41 a true hernia of the anterior mediastinum withoutpericardial involvement. It can be ruled out by its lackof conformity to the pericardium or by lack of pneumo-pericardium after pneumoperitoneum. Other rare,similarly-appearing conditions include esophago-,gastro-, and enteropericardial fistulas,45 tracheoperi-cardial fistulas,26 subphrenic abscess33 ruptured intopericardium, pericarditis 44 pericardial cyst,40 absentpericardium,17 and pericardiopleural laceration. 17'21

Treatment

Whereas there is debate regarding the most idealapproach for repair of acute and chronic intrapleuraldiaphragmatic hernias, certainly the best approach forrepair of IDH is transabdominal. The transthoracicapproach for IDH repair necessitates cardiac displace-ment and incision of the lateral pericardium and hemi-diaphragm. Furthermore, IDH has been completelyoverlooked at thoracotomy.7'35 The transabdominal ap-proach provides direct exposure and thorough exam-ination of the abdominal viscera. Its only disadvan-tage is the difficult exposure of the right hemidia-phragm should it require repair, in which case rightthoracotomy may be required. This preferred approachmakes preoperative distinction between chronic intra-pleural and intrapericardial diaphragmatic hernias veryimportant.

Repair is effected by closure of the defect with non-absorbable suture. Bolsters of Teflong,11 pericardium,8or fascia lata12'36 have been occasionally used, but thepericardial diaphragm can be satisfactorily suturedprimarily. No patient other than case 1 died, and therewere no recurrences.The ease of repair, low complication rate, and lack

of recurrence certainly justify correction of the defect,which otherwise could cause severe and possibly fatalconsequences.

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366 MENG AND OTHERS Ann. Surg. * March 1979

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