CONGENITAL RIGHT-SIDED DIAPHRAGMATIC HERNIASOME DIFFICULTIES IN DIFFERENTIAL DIAGNOSIS AND

OPERATIVE REPAIRBY

RONALD BELSEY, M.S., F.R.C.S., and JOHN APLEY, M.D., M.R.C.P.(From the Thoracic Unit, Frenchay Park Hospital, Bristol, and the Bristol Children's Hospital)

Right-sided congenital diaphragmatic hernia hasin the past been considered to be incompatible withlife. Even as recently as 1925 Hedblom statedthat 75 per cent. of all cases of congenital diaphrag-matic hernia die before the age of one month.With modem advances in technique death is nowin most cases avoidable, and must be blamed onfailure to diagnose correctly. Mis-diagnosis is somuch the rule that in adult life diaphragmatichernia has been called 'the masquerader of theupper abdomen,' and it has been calculated thaton an average three incorrect diagnoses are madebefore the correct one is established (Harrington,1945). In infants and young children, in whomcardio-respiratory symptoms often predominate,the diagnosis is even more likely to be missed.

In this paper two cases with right-sided congenitaldiaphragmatic hernia are described; in neither wasthe diagnosis established until several other tentativediagnoses had been discarded.

Frequency of OccurrenceWhilst it is certain that herniation on the right

side is considerably less common than on the left,the comparative incidence of occurrence has notbeen accurately assessed. In the Frenchay thoracicunit approximately 3 per cent. of all admissions arecases of diaphragmatic hernia, and it is significantthat the apparent incidence has risen appreciablysince the adoption of the routine head-low proneposition during radiological examination withbarium.On the right side herniation most commonly

occurs through either the pleuro-peritoneal hiatus(Bochdalek) or the oesophageal hiatus; through thepara-sternal hiatus (Morgagni) it is less common.It is important to recognize that apparently right-sided herniation may, in fact, arise from theoesophageal hiatus (figs. I and 2).

SymptomatologyThe symptomatology is not appreciably affected

by the site of herniation and varies between wideextremes. Cases may be discovered fortuitouslv (asin the two described below) or, in severe examples,death may occur within a few hours of birth.

It is said that symptoms are more severe in thecongenital than in the acquired types of hernia, butin our experience the contrary is true: the congenitaltype frequently remains silent, whereas the acquiredtype often becomes dramatically obvious.

Cardio-resprtory symptoms. With congenitalherniation cardio-respiratory symptoms tend topredominate. In severe cases dyspnoea and cyanosismay be evident soon after birth. They may persistor may recur paroxysmally, perhaps in relation tomeals. Later in life palpitations or precordial painmay be described in milder cases.Alientary system. Disturbances of the alimen-

tary system may occur with the above or alone.They arise in the main from variable degrees ofobstruction either in the stomach or lower downin the intestines, and their manifestations areconfusingly variable. If obstruction becomescomplete, as not infrequently occurs, symptomstypical of acute obstruction develop and the outlookis gloomy indeed. In the absence of completeobstruction a common symptom is vomiting, thoughin mild cases there may be no more than eructationand a feeling of distension after meals. Occasionallyhaematemesis or melaena occurs from engorgedmucosal vessels, and if bleeding is persistent thevague symptoms of anaemia may develop. InCase 2, described below, the gall-bladder wassituated in the thoracic cavity, and in another recentcase the intestines were imperfectly rotated and theappendix lay in the thorax; inflammation arisingin any of the misplaced abdominal organs canobviously produce obscure symptoms whose inter-pretation may be perplexing.

General symptoms. Of the general symptoms, ithas already been mentioned that some may beattributable to anaemia. In infants and youngchildren there may be failure to thrive, probably dueto a combination of cardio-respiratory and ali-mentary disturbances. In a recent case, at tenmonths of age an infant weighed little more thantwo pounds over its birth-weight; both the generalcondition and weight improved dramatically afteroperation.

DiagnosisHistory. By the history alone the diagnosis can

be only suspected. Excluding the most severe cases,129

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the common story (as in Case 2) is that of so-calledrecurrent pneumoma. This erroneous diagnosis isusually based on the evidence of dyspnoea andabnormal physical signs obtained when the child isexamined because of some unrelated upperrespiratory infection. Apparently inexplicablevomiting which recurs over a considerable periodshould also suggest the diagnosis.

Clinical emination This may, on occasion,provide an unequivocal diagnosis. The essentialfeature is the detection of bowel sounds in the chest.To elicit this pathognomonic sign it is advisable toauscultate soon after the patient has taken a meal,and to continue auscultation for at least ten minutes.In both the cases described with these precautionsthe characteristic bowel sounds were invariablyheard; without them they were frequently notdetected. It has long been realized that even inthe presence of a known diaphragmatic herniaabnormal signs in the chest may be absent; possiblythe observance of the above precautions wouldreduce the number of such failures.

In addition, the variability of the physical signsfrom time to time in the same patient is itselfsuggestive of the diagnosis. Impairment of expan-sion on the affected side, dullness to percussion,and suppression of breath sounds, constitute theusual findings in the chest, and the mediastinummay be displaced. Occasionally the signs simulatethose of pneumothorax or hydro-pneumothorax.T'he liver may lie in the thoracic cavity, and thephysical signs which it produces are very commonlyattributed to pleural effusion. If the duodenum isobstructed and the stomach consequently dilated,a characteristic splash may be elicited. When largeportions of the bowel are contained in the chest theabdomen may fail to develop proportionately andmay present a scaphoid appearance.

Rad1oocal AppearanesIn every suspected case radiological studies are

essential, especially in view of the danger inherentin diagnostic aspiration where portions of the bowelare contained in the chest.

Only conditions occurring on the right side whichare commonly confused will be considered here,though in theory similar appearances might beproduced by 'gas' infections of the pleura,eventration of the right diaphragm with upwardprotrusion of part of the liver, multiple tuberculouscavities, congenital cystic disease of the lungs,hydatid cysts that have ruptured into the bronchialtree, and diverticula of the oesophagus.The radiological appearances are suggestive

because of their very oddity. There may be shadow-ing at the base which is typical of nothmg.' Theconsistency of the shadow is not completely uniform,with a rare exception: if omentum alone hasherniated the opacity may appear homogeneous.An appearance of 'tenting is not unusual (fig. 3).If lateral v-iews of the chest are studied in conjunctionwith antero-posterior views there should be little

difficulty in distinguishing herniation from freepleural effusion.The commonly accepted statement that radio-

logical diagnosis can be made easily by threefeatures, the detection of gas bubbles, collapsedlung, and mediastinal displacement, misleads byover-simplification.

In most cases diagnosis is indeed suggested by thepresence of gas shadows and the characteristicpattern caused by the valvulae conniventes of thesmall intestines and the haustration of the largebut this appearance may be simulated by (1) lungabscesses or saccular bronchiectasis (figs. 4 and 5);(2) loculated or encapsulated effusions; (3) loculatedsub-diaphragmatic abscess; (4) interposition of colonbetween liver and diaphragm (fig. 6) or, (5) con-genital abnormalities of the oesophagus. Collapseof lung and mediastinal displacenent occur invarying degree, but are only subsidiary in diagnosis.

Certainty of diagnosis can be achieved by meansof barium follow-through studies (figs. 7, 8, 12, 13).In no case should the precaution be omitted oftilting the patient in the prone position with thehead low during the examination.

Lateral views with barium in the intestine help inlocalizing the position of the diaphragmatic defect,but to assess the size of the defect from radiologicalappearances is usually impossible.

TreatmentThe treatment of the condition is always surgical.

It should be undertaken as early as possible to avoidfirst, the technical difficulties caused by failure ofexpansion of the abdomen, which becomes pro-gressively and relatively more marked the longerthe abdominal contents remain in the thorax;second, the general maldevelopment which accom-panies long standing disturbances of intestinalfunction; and third, the occurrence of acuteintestinal obstruction. If obstruction does occur,surgical treatment becomes a matter of extremeurgency.Two routes are available, the abdominal and the

thoracic. The thoracic approach permits a sounderrepair of large diaphragmatic defects, and anincrease in the size of the abdominal cavity byraising the attachments of the diaphragm to a higherlevel in the thorax. Following replacement of theabdominal viscera the lung can be expanded withoutdifficulty by the anaesthetist, and any structuralchange in the lung following the prolonged com-pression by displaced abdominal viscera is unusual.

Inboththecasesreportedthe defect was so large thatthe usualrepairwith an overlap was out of the question.Various methods of overcoming such a difficultyhave been devised. Harrmngton's (1948) techniqueof performing a basal thoracoplasty to free thediaphragn has the disadvantage of being a deform-ing operation which interferes permanently with thefunction of the affected side of the chest. A newprocedure was evolved, and proved successful inboth cases. The chest was entered through the bed

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CONGENITAL RIGHT DIAPHRAGMATIC HERNIA 131

of the ninth rib. After replacement ofthe abdominalviscera, the remnants of the diaphragm were freedfrom their attachments to the costal margin allround, and left with only their mediastinal originand corresponding blood and nerve supply intact.The diaphragmatic defect was then closed by tworows of interrupted sutures of stainless steel wireand an overlap. The repaired diaphragm, muchreduced in size, was reattached to the thoracic wallat a level two interspaces higher than normal withinterrupted sutures of the same material, nodifficulty being encountered in bridging the lesserdistance horizontally across the thoracic cavity,after eliminating the costo-phrenic sulcus. Thephrenic nerve was not crushed in either case, andthe functional result has proved excellent in bothpatients.

Case ReportsCase 1. A girl aged 7 years was found on routine

chest examination by a school medical officer to haveabnormal physical signs in the right chest. Shewas sent to the local tuberculosis dispensary wherea radiograph of the chest was taken. A diagnosisof right-sided pleural effusion was made, and thechild was admitted as an emergency to a children'ssanatorium with instructions to the resident medicalofficer to ' aspirate the chest.' The resident medicalofficer elicited from the mother a history of recurringbouts of abdominal pain and vomiting. The attackshad been irregular and bore little relationship tomeals. There were no symptoms suggestive ofpulmonary disease. Examination revealed anunder-nourished and under-developed child; thelower half of the right chest was dull to percussionand breath sounds were replaced by bowel sounds,but this sign was elicited only on prolonged ausculta-tion. The abdomen was scaphoid in appearance.A radiograph revealed a dense shadow in the lowerhalf of the right chest with a few gas-containingareas showing typical intestinal haustration towardsits peaked upper margin (fig. 3). A barium follow-through examination revealed the presence of halfthe duodenum, which was partially obstructed, thewhole of the small intestine, and the right half ofthe colon within the right pleural cavity. Hadthoracentesis been performed before an accuratediagnosis had been made, disaster would almostsurely have followed.The child was transferred to the Frenchay

thoracic unit and, after a preliminary course ofbreathing exercises lasting four weeks, the herniawas repaired through the thoracic approach. Nohernial sac was present. Replacement of the viscerawas difficult owing to the contraction ofthe abdomen.The defect in the diaphragm, a patent pleuro-peritoneal canal, was so large that the plastic repairwith transposition to a higher level, as alreadydescribed, was necessary. The lung was inflatedwithout difficulty and the chest closed withoutdrainage. Convalescence was uneventful. Thechild has remained free from symptoms, andexamination five years later showed normal

development. X-ray examination shows that theright diaphragm moves normally, the right lung isfully expanded, and there is no impairment ofthoracic function (fig. 9).

Case L A girl aged 4 years was admitted tohospital with a diagnosis ofright basal ' pneumonia. *The mother reported two similar attacks of pneu-monia, also on the right side, during the previousyear. On admission the child was pale, looked ill,and had an intermittent cough; the temperaturewas 101 F., and the pulse rate 130, and respirations40 per minute. Examination revealed poor move-ment, impaired percussion note, diminished breathsounds, and poor vocal fremitus at the right base.A diagnosis of pneumonic consolidation with apleural effusion was made. Penicillin therapy wascommenced. Eleven days after admission thedullness at the right base was less marked and bowelsounds in the chest were detected for the first time.Review of the radiographs suggested the presenceof intestines, containing mostly fluid but also a littlegas, within the right pleural cavity (figs. 10 and 1 1).

Further x-ray examination showed appearancesmore typical of a diaphragmatic hernia, and abarium follow-through revealed that the whole ofthe small bowel and the right half of the colon werein the right pleural cavity (figs. 12 and 13). Thetempeiature, pulse rate, and respiration rate quicklydropped to normal, and penicillin was discontinuedafter twelve days. Breathing exercises were started.Four weeks after admission a right thoracotomy

was performed through the bed of the ninth rib.No hernial sac was present. Part of the duodenum,the whole of the small bowel, the right half of thecolon, and the liver lay free in the pleural cavity.The right lung was collapsed. The gall bladder wasin contact with the axillary part of the thoracic wall.Any attempt to needle the chest might have pro-duced bizarre and dramatic consequences. Follow-ing replacement of the abdominal viscera the defectwas again found to be too large for a simple repair,and a similar operation to that performed in theprevious case was necessary. Convalescence wasuneventful. The child has remained free fromsymptoms since the operation, her general conditionis excellent, and on x-ray and screening the recon-structed right diaphragm appears to functionnormally (figs. 14 and 15).

Summary1. The symptomatology, diagnosis, and differ-

ential diagnosis of right-sided congenital diaphrag-matic hernia are ieviewed.

2. A new technique for the plastic repair of largediaphragmatic defects is described.

3. Two cases successfully treated surgically arereported.

REFEREN'CESHedblom, C. A. (1925). Jour. Amer. med. Ass., 85, 947.Harrington. S. W. (1945). Ann. Surg., 122, 546.

(1948). Surg. Gynec. Obstet.. 86, 735.

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FIGs. 10 and I I.-(Case2.) Antero-posterior andlateral films of chest show-ing 'gas bubbles' and'tenting' of the opacityat the right base.

FIGs. 12 and 13.-(Case2.) Barium meal in pronehead-low pition; bothsmaHl and large intestinelying free in right pleuralcavity.

FIGs. 14 and 15.-(Case2.) Post-operative film ofchest following repair;right diaphragm function-ing normally.

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