diaphragmatic eventration infants - thorax.bmj.com · term and weighed 3 0 kg. delivery was normal,...

Thorax (1973), 28, 66.

Diaphragmatic eventration in infantsF. PARIS, E. BLASCO, A. CANTO, V. TARAZONA,

and M. CASILLAS

Thoracic Surgery Service, Department of Surgery, Centro Hospitalario 'La Fe', Valencia 9, Spaint

Nine cases of diaphragmatic eventration are presented in infants, seven of whom requiredsurgery. All were seen during the first 19 months of life and seven were less than 1 year old.Although differing from other studies, our cases show a predominance of right-sided lesions.Respiratory symptoms were present in most cases, and digestive symptoms appeared in a lessernumber of cases. The radiological changes were pronounced in seven of the nine cases, thedome of the diaphragm reaching to the fourth to fifth intercostal spaces. Regarding surgicaltechnique, the authors favour diaphragmatic plication through the thorax. This gave excellentclinical and radiological results and was without mortality or complications.

The first description of this anomaly was Petit's(1790) necropsy finding. He described it as aspecial form of thoracic hernia 'without anyseparation of the muscular and tendinous fibresin the diaphragm'. The condition was calleddiaphragmatic eventration by Beclard and Cruveil-hier (1829) and this term became generally accep-ted instead of such names as relaxation, phrenicinsufficiency, or hypertrophied diaphragm.The difference between eventration and hernia

is recorded by Bisgard (1947), who describes even-tration as 'an abnormally high, or elevated posi-tion of one leaf of the diaphragm as a result ofparalysis, aplasia, or atrophy of varying degreeof the muscle fibres. Its unbroken continuitydifferentiates it from diaphragmatic hernia'.

Until recently eventration in infants was rarelydiagnosed and treated, and descriptions of thecondition were mainly from necropsies (Bayne-Jones, 1916; Reed and Borden, 1935). Nowadaysthis condition is encountered more frequently inpaediatric practice. Proof of this is the work ofRichard et al. (1959), who collected 80 cases fromthe literature and added 10 of their own, and therecent work of Monereo, Cortes, and Dominguez(1970) who report 15 cases.

CASE REPORTS

CASE I D.T., a 5-month-old girl, was born at fullterm and weighed 2 8 kg. Delivery was difficult witha breech presentation and artificial respiration wasrequired. The mother stated that the child had had apersistent cough from the first few days after birthuntil the time of our examination, and that recentlyher condition had deteriorated with dyspnoea and poor

appetite. Her weight was 5 3 kg (2 7 standard devia-tions below the normal mean weight). Examinationdisclosed asymmetry of the chest wall with bulgingof the lower right hemithorax and a scaphoidabdomen. Auscultation revealed wheezing and a pecu-liar flapping sound in the mid line.The chest radiograph showed an elevated right

diaphragmatic dome, reaching the level of the fifth ribposteriorly, and a marked shift of the mediastinumto the left. On fluoroscopy the movement of the rightdiaphragm was paradoxical, and the mediastinummoved to the left on inspiration.

Conservative treatment was tried without success.the cough, dyspnoea and auscultatory findings persist-ing. In view of this, and as there was concern regard-ing the infant's development, surgery was recom-mended. A diaphragmatic plication was performedthrough a thoracic approach and the dome of thediaphragm was lowered to the level of the tenth ribposteriorly. Convalescence was satisfactory and allrespiratory symptoms disappeared. After six years thechild's development is normal, the mediastinum central.and the diaphragmatic movement normal.

CASE 2 J.LL., a 51-month-old boy, was born at fLllterm and weighed 3 0 kg. Delivery was normal, withcephalic presentation, but respiratory assistance wasrequired. The parents stated that the infant hadsuffered from repeated bronchopneumonic attackssince birth, accompanied by fever, dyspnoea, andcyanosis.On clinical examination the infant was under-

nourished, showing prominence of the lower rightchest wall and diminished breath sounds at the rightbase. His weight was 5 kg (3 4 standard deviationsbelow the normal mean weight). The chest film showedelevation of the right diaphragm, the vertex reachingthe fourth intercostal space. On fluoroscopy the move-ment of the affected side had 'the dicrotic-like pattern'

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Diaphragmatic eventration in infants

described by Chin and Lynn (1956), with the laxportion moving initially in a paradoxical manner andafterwards normal contraction of the periphery. Thisproduced mediastinal displacement on respiration,which at first sight appeared as authentic paradoxicalmovement of the right diaphragm.A diagnosis of right diaphragmatic eventration was

made. Plication was performed, lowering the muscleto the ninth rib. Postoperative recovery was satis-factory and after three years the child is progressingwell. The position and movement of the diaphragmare normal. There has been no recurrence of thepneumonic episodes.CASE 3 J.O., a 10 monlh-old boy, was born bynormal delivery with cephalic presentation andweighed 3-2 kg. The child attended hospital becauseof a chest deformity which was similar to that ofcases 1 and 2. At that time his weight was normal(8-5 kg). The films showed the right diaphragm to beelevated to the level of the sixth rib with a media-stinal shift to the left. On screening there was a doublephase movement of the diaphragm. A plication wasperformed and three years later the child is well andthe chest film is normal.CASE 4 V.F., an 11-month-old boy. was born wiih-out difficulty and weighed 3-5 kg. Presentation wascephalic. Three months after birth he suffered a rightbronchopneumonia which produced serious dyspnoea.After that he had repeated acute pneumonic attacks.His weight was 8-0 kg. Physical examination discloseda bulging of the right lower thoracic cage and ascaphoid abdomen. On fluoroscopy the movement ofthe diaphragm appeared as in the previous cases, witha slight contraction of the periphery and a relaxationof the middle portion of the right diaphragm. A plica-tion was performed via the thorax with satisfactoryresults. After one year the patient is in good condition

and movement of the diaphragm is normal. There hasbeen no recurrence of the acute bronchopneumonicattacks.CASE 5 M.D., a 19-month-old girl, was born at fullterm with cephalic presentation and weighed 3-5 kg.The mother knew of no respiratory symptoms, butsince the age of 3 months the girl had suffered fromanorexia and vomiting and normal development wasretarded. At the time of examination her weight wasa normal 10 kg. Examination revealed the same chestwall asymmetry as in the previous cases and the radio-graph showed the vertex of the dome reaching thelevel of the fourth rib. Fluoroscopy revealed sluggishmovement of the diaphragm, but it lacked the doublephase mobility. In the lateral view, an opaque wedge-shaped zone appeared in front of the dome, which weinterpreted as pulmonary atelectasis by compression(Fig. 1).

In addition to the apparent redundancy of thediaphragm, another operative finding was an intra-lobar sequestration in the anterior segment of theright lower lobe, with a blood supply from infradia-phragmatic vessels. Because of this it was extremelydifficult to separate the lung from the diaphragm. Inthis case the muscle was more atrophic than in therest of the series, particularly in the anterior andcentral portions where the peritoneum and the pleuraappeared to meet. A right lower lobectomy was per-formed follovwed by a plication, which because of thelung resection was not as extensive as in the othercases. Eighteen months after operation the girl is inexcellent condition. The position of the diaphragm,although somewhat more elevated than in the rest ofthe series, conserves good mobility.CASE 6 R.O., a 12-month-old boy, had a normalbirth with cephalic presentation and weighed 3-6 kg.The mother noticed a marked lack of appetite during

FIG. 1. Anteroposterior and lateral chest films of case 5. The lateral projection reveals an opaque zone infront of the relaxed dome of the diaphragm.

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F. Paris, E. Blasco, A. Canto, V. Tarazona, and M. Casillas

artificial feeding (Eledon), the child consuming onlyhalf or one-third of the normal portion. In additionhe suffered from sporadic vomiting and constant con-stipation. At the age of 5 months he was ill with acutebronchopneumonia and was treated with various anti-biotics and corticosteroids. Physical examinationrevealed gross maldevelopment with a weight of 6-4kg (3 standard deviations below the normal meanweight). There was marked pigeon's breast and a leftHarrington's groove, and prominence of the lowercostal margin below. The abdomen was scaphoid.Radiographs revealed elevation of the left dome tothe fourth rib space. Both stomach and colon, coveredby the relaxed diaphragm, were displaced upwards.On fluoroscopy the dicrotic-like movement of thediaphragm, as previously described, was observed. Aplication was performed and the diaphragm waslowered to the eighth intercostal space. The post-operative period was complicated by paralytic ileuswith abdominal distension, which resolved in 48 hours.One year after surgery the scaphoid abdomen haddisappeared and the weight curve is normal. Radio-graphy shows a central mediastinum and normalmovement of the diaphragm.

CASE 7 C.T., a 7-month-old boy, was born at fullterm with cephalic presentation and weighed 40 kg.The mother stated that nine days after birth he hadpneumonia with cyanosis. The fever reached 39' C(102-2' F) and required antibiotics during a 15-dayperiod. He then had repeated colds with coughand mucoid sputum and low-grade fever from375' to 380 C (99-5' to 1004' F). The findings onphysical examination were similar to those in theprevious cases and his weight (7 5 kg) was withinnormal limits. Radiographs revealed the dome reach-ing the fourth intercostal space. On screening, thediaphragm had the characteristic dynamics of eventra-tion. A very hypoplastic lower right lobe was dis-covered during the operation. A plication wasperformed.

A small muscular wedge of diaphragm removedfor examination revealed a degenerative myopathy,demonstrating dissociation of the striated muscularfibres included within dense fibroconnective tissue. Theinvoluted nature of the muscle fibres was shown bythe loss of striation, granular transformation of thesarcoplasm, and a marked proliferation of the nucleus.The boy made a good recovery and the hypoplastic

lobe readily expanded. Nine months after operationpulmonary development is normal, and the previoushypoplasia of the lobe is not noticeable.CASE 8 R.M., a 10-month-old boy, had a normalbirth with cephalic presentation and weighed 4 3 kg.At the age of 3 months the mother noticed that theboy's lips had become somewhat cyanotic withoutaccompanying cough or fever. Radiographs revealeda shadow in the base of the right lung and becauseof this we were consulted. A symmetrical thoraciccage was observed and in this he differed from theother patients. His weight was a normal 9 kg. In thefilms the diaphragm was seen to be markedly relaxed,although on fluoroscopy it contracted well and therewas no sign of mediastinal herniation. In view of thisit was not felt that surgery was indicated, and treat-ment was conservative.CASE 9 A.M., a 15-month-old girl, was born pre-maturely at 8 months; she weighed 2-0 kg and requiredincubation for one and half months until a 3 0 kgweight was reached. Presentation was cephalic. Therewas no history of respiratory or digestive symptomsand weight development was normal. The mother wasconcerned mainly about the deformity of the thoraxand for this reason we were consulted. A bulge in theposterior aspect of the base of the right hemithoraxand an anterolateral depression at the level of thesixth and seventh ribs were found on clinical examina-tion. Chest films revealed an elevated dome, and therelaxation was more noticeable posteriorly. On screen-ing there was no sign of mediastinal movement andmovement of the diaphragm was normal. Surgery was

TABLECLINICAL DATA OF THE NINE CASES

Delivery' andCase Birth Weight

(kg)

2

31

4

5

6

7

8

9

Podalic (2 8)

Cephalic (3 0)

Cephalic (3 2)

Cephalic (3 5)

Cephalic (3-5)

Cephalic (3 6)

Cephalic (4 0)

Cephalic (4-3)

Cephalic (2-0)

Age atOperation

(mth)

5

5

10

11

19

12

7

10

15

RespiratorySex Symptoms

F Severe

M Severe

M

M Severe

F

M

M

M

F

Severe

Severe

None of the cases required forceps delivery

Gastro-intestinalSymptoms

Localization andResult of Fluoroscopy

IMR rIaMild IRight. Paradoxical

movement

- Right. Chin sign

Mild

Mild

Right. Chin sign

Right. Chin sign

Right. Sluggish motility

Left. Chin sign

Right. Chin sign

Right. Normal motility

Right. Normal motility

Treatment

I Plication

Plication

Plication

Plication

Plication + lobectomyfor lung sequestrationPlication

Plication

- ~l -1- 1- ~l ~-1-

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not considered necessary and observation of thepatient was advised.

DISCUSSION

Diaphragmatic eventration in infants is not aclinical rarity. Beck and Motsay (1952) found thatin 2,500 cases of newborn infants 4% had minorrelaxations and 1-2% had serious eventrations. Wehave had the opportunity of studying nine casesover a six-year period; all of them were broughtto us before the age of 19 months and seven ofthem were less than 1 year old. Two-thirds of thecases were male.

In the Table the most important clinical dataare summarized, and in Fig. 2 the pre- and post-operative radiographs of the seven surgical casesare reproduced. The sample indicates a higher fre-quency of eventration on the right side, which iscontrary to the findings in other studies. In thereview of Richard et al. (1959), 57 cases of even-tration were left-sided, 21 right-sided, and 2 werebilateral. Six of his own cases belonged to thefirst group and four to the second. In the seriesof Monereo et al. (1970) there is a slight predomi-nance of left-sided lesions.An explanation for the higher frequency of

right eventration in our series may be the factthat we are thoracic surgeons. The viscus whichenters the thorax in right eventration and pushesagainst the relaxed diaphragm is the liver, andbeing such a bulky organ it produces greaterrespiratory difficulty. Also, on occasions we areconsulted regarding the possible diagnosis of chesttumours or hydatid cysts which may present diag-nostic difficulties in infancy. On the other hand,the stomach and colon are the organs involvedon the left side and therefore the diagnosis ismade with less difficulty. For this reason, left-sided cases are referred more often to paediatricsurgeons.The relaxation of the diaphragm in all cases

requiring surgery was complete, and it was pos-sible to follow the diaphragmatic contour; thusthere were no diagnostic problems. In partial even-tration confusion is possible. Bonham-Carter,Waterston, and Aberdeen (1962) gave little con-sideration to the difference between hernia andeventration. Christensen (1959) points out the diffi-culty of preoperative diagnosis of both conditions.Histological examination of the muscular fibresshould give definitive proof.

In practice it is necessary to differentiate be-tween complete and partial eventration. The firstis a clinical entity different from hernia and isgenerally associated with obvious respiratory

symptoms. The second, as Richard et al. (1959)point out, acts as a hernial sac regardless of itshistology.

According to its aetiology, the diaphragmaticeventration can be either congenital or acquired.The first may be present in the infantile or adultform and the second arises from a temporary orpermanent disorder.The anomaly is based on a defect of penetra-

tion of the cervical myotomes in the elementswhich make up the diaphragm, the septum trans-versum, the pleuroperitoneal folds, the commonmesentery, and the lateral wall of the coelom.Beck and Motsay (1952) consider this disease tobe a form of muscular aplasia. They base theirargument favouring the congenital theory on thelack of inflammation or degeneration of thephrenic nerve. Bayne-Jones (1916) supports thecongenital theory. In our series we have, in twocases, found an associated congenital lesion, apronounced lobar hypoplasia in one case, anda pulmonary sequestration in another.We have classified the acquired forms of even-

tration as those produced by (1) lesions of thephrenic nerve, (2) central neurological disease,(3) myopathies, and (4) changes in diaphragmaticlevels caused by neighbouring lesions (pleuraleffusion, subphrenic abscess, and space-fillingabdominal lesions).

In the infantile form, it is necessary to callattention to the lesion of the phrenic nerve causedduring delivery, a mechanism that acquired specialsignificance in the work of Bingham (1954),Richard et al. (1957), and Jaubert de Beaujeu andMollard (1965). The diaphragmatic lesion isusually found to be accompanied by an Erbbrachial paralysis, although it can also appearalone. It is necessary to consider stretching ofthe nerve roots which may occur during the ex-pulsion period of delivery. Breech presentationand forceps delivery favour nerve damage.We described one of our cases (case 1) as a

post-delivery paralytic eventration withoutbrachial plexus palsy. The reasons which ledus to consider it thus were (1) a difficult deliverywith breech presentation, (2) the appearanceduring the first few minutes of life of symptomsrequiring respiratory resuscitation, and (3) thediscovery, during thoracotomy, of an elevated butapparently normal muscular diaphragm. Weshould say, however, that we lack the evidenceto confirm post-delivery nerve damage. Theprincipal argument against this aetiology is therapid postoperative recovery of the mobility ofthe diaphragm, which would be unlikely if it wassecondary to phrenic nerve damage.

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FIG. 3. Diagram showing our procedure: the diaphragm is pulled taut in a radial direction towards themediastinum, held in place with a few stitches, and followed by multiple invaginating sutures.

Of the seven cases requiring surgery, threeshowed only respiratory symptoms consisting ofrepeated attacks of acute bronchopneumonia ac-companied by dyspnoea and cyanosis. In onecase there was anorexia and sporadic vomiting,and in two both respiratory and abdominal signswere present. In one case the child attended hos-pital only because of the chest wall deformity.The low incidence of digestive symptoms is due,in our opinion, to the predominance of right-sided lesions as well as to the difficulty of de-tecting subjective symptomatology such as post-prandial heaviness, palpitations after feeding, orheartburn in infants. A marked underdevelopmentwas evident in the weight of cases 1, 2, and 6,and in a lesser degree in case 4.

In eight cases there was bulging of the righthemithorax and a scaphoid abdomen, and in oneof these the relaxation was more pronounced inthe posterior portion, forming a posterolateralprotuberance with an anterolateral depression ofthe chest.The radiological picture was pronounced in

seven of the nine infants, the dome reaching tothe fourth to fifth intercostal spaces. The eventra-tion was complete in all except case 9 in whomonly the posterior part of the diaphragm was in-volved. Mediastinal movement on fluoroscopyappeared in six cases, and in five of these theeventrated diaphragm had the characteristicpattern of movement described by Chin and Lynn(1956).As regards technique, we are in favour of a

diaphragmatic plication performed by an inter-costal approach (Fig. 3). To achieve this the

diaphragm is stretched in a radial direction to-wards the mediastinum (Fig. 4) and a few mattresssutures of 1-0 Terylene are taken to hold thediaphragm in place. The suture line must alwaysbe parallel to the distribution of the phrenicnerve. We have never firmly fixed the plicateddiaphragm to the wall of the thorax, as has beendone by Quenu and Herlemont (1953) throughthe thorax, and by Butsch and Leahy (1950)through the abdomen, for if it is left firmly tiedit is only at the expense of mobility. For thisreason we prefer to invaginate the plication linewith multiple sutures, taking the number of layersnecessary in order to leave the diaphragm tautand lowered one or two intercostal spaces beyondthe level that we ultimately wish to achieve.

__. Vrw &_MFIG. 4. Operative view. The muscle is tensed beforebeginning the plication.

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FIG. 5. Minimal posterolateral thoracotomy. Detailof the cutaneous incision.

Other techniques are those of incision fol-lowed by an overlapping imbrication suture orexcision of the relaxed tissue (Bingham, 1954;Chin and Lynn, 1956; Bishop and Koop, 1958).These methods expose the patient to rupture ofthe suture line and secondary hernia.We believe that surgery should be performed

through the thorax, as it is easier to work overthe vertex of a convexity than in the hollow of a

concavity. However, it should not be forgottenthat authors such as Butsch and Leahy (1950)and State (1949) have operated using the sub-costal abdominal approach. Notwithstandingthese methods, our results incline us towards thethoracic approach because this leads to fewerpostoperative complications and an average hos-pital stay of five days. All operations were per-formed through a small thoracotomy (Fig. 5).

REFERENCESBayne-Jones, S. (1916). Eventration of the diaphragm, with

report of a case of right-sided eventration. Arch. intern.Med., 17, 221.

Beck, W. C., and Motsay, D. S. (1952). Eventration of thediaphragm. Arch. Surg., 65, 557.

Bingham, J. A. W. (1954). Two cases of unilateral paralysisof the diaphragm in the newborn treated surgically.Thorax, 9, 248.

Bisgard, J. D. (1947). Congenital eventration of the dia-phragm. J. thorac. Surg., 16, 484.

Bishop, H. C., and Koop, C. E. (1958). Acquired eventrationof the diaphragm in infancy. Pediatrics, 22, 1088.

Bonham-Carter, R. E., Waterston, D. J., and Aberdeen, E.(1962). Hernia and eventration of the diaphragm inchildhood. Lancet, 1, 656.

Butsch, W. L., and Leahy, L. J. (1950). A technique for thesurgical treatment of congenital eventration of thediaphragm in infancy. J. thorac. Surg., 20, 968.

Cruveilhier, J. (1829-35). Anatomie Pathologique dlu CorpsHumain, vol. 1, book 17, p. 2. Bailliere, Paris.

Chin, E. F., and Lynn, R. B. (1956). Surgery of eventrationof the diaphragm. J. thorac. Surg., 32, 6.

Christensen, P. (1959). Eventration of the diaphragm.Thorax, 14, 311.

Dor, J., Richelme, H., Aubert, J., and Boyer, R. (1969).1'Eventration diaphragmatique. J. Chir. (Paris), 97, 399.

Jaubert de Beaujeu, M., and Mollard, P. (1965). Paralysieobstetricale du diaphragme chez le nourrison. Indica-tions et modalites du traitement chirurgical. Ann. Chir.thorac. Cardiovasc., 4, 469.(Ann. Chir.. 19, 973).

McNamara, J. J., Paulson, D. L., Urschel, H. C., andRazzuk, M. A. (1968). Eventration of the diaphragm.Surgery, 64, 1013.

Monereo, J., Cortes, L., and Dominguez, J. (1970). Herniasdiafragmaticas del nino con analisis de las no hiatalesy su mortalidad. Cirug. Espaiola, 24, 135.

Petit, J. L. (1790). Traite des Maladies Chirurgicales, et desOperations qui Leur conviennent, vol. 2, p. 233.Mequignon, Paris.

Quenu, J., and Herlemont, P. (1953). Du traitement chirurgicalde 1'eventration diaphragmatique. J. Chir. (Paris), 69,101.

Reed, J. A., and Borden, D. L. (1935). Eventration of thediaphragm; with a report of two cases. Arch. Surg., 31,30.

Richard, J., Chevalier, V., Capelle, R., Cavrot, E., Content,J., and Delforge, J. (1957). La paralysie diaphrag-matique obstetricale. Arch. franc. Pediat., 14, 563.

Henry, C., Pelc, S., and Content, J. (1959).L'eventration diaphragmatique chez 1'enfant. Arch.fran9. Pediat., 16, 300.

State, D. (1949). The surgical correction of congenitaleventration of the diaphragm in infancy. Surgery, 25,461.

Thomas, T. V. (1970). Congenital eventration of the dia-phragm. Ann. thorac. Surg., 10, 180.

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