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Hiatal hernia and gastroesophageal reflux in infants and children
Hiatal hernia and gastroesophageal reflux in infants and children John R. Lilly, M.D.* (by invitation), and Judson G. Randolph, M.D.,** Washington, D. C.
JU/sophageal hiatus hernia is a common congenital defect in infants and children. The symptoms and ultimate morbidity of this condition in the young are primarily re lated to gastroesophageal reflux, not to esophagitis. In infants, reflux of gastric con tent is rarely associated with gastric hyper acidity; instead, a clinical picture evolves which may merge with other ill-defined feeding problems of this age group. If un recognized, these symptoms, which are sub tle yet commonplace, can lead to deva stating malnutrition and suppression of growth in the early years of life. This report outlines the clinical characteristics and ther apeutic management of 52 consecutive cases of hiatal hernia in children seen on the Sur gical Service of the Children's Hospital, Washington, D. C , from January, 1957, through December, 1965.
definite projection of the stomach above the diaphragm on barium studies of the upper gastrointestinal tract. All hernias were of the sliding type. In 4 patients, marked gastroesophageal reflux was demon strated during barium study; but a hjatal hernia could not be visualized despite several radiographie attempts. The majority (47) of the patients were examined by cinefluoroscopy and the image intensifier. A gastrointestinal series was obtained after treatment in 40 of the 52 patients; all of the surgical patients were so studied. The fol low-up period ranged from 1 to 5 years. Forty-three patients were under 2 years of age, and 25 were less than 3 months old (Fig. 1). Six of the children were 10 years of age or older. Only 3 children between the ages of 2 and 10 years were found to have hiatal hernia.
Patient material
Symptoms
In 48 of 52 children, the diagnosis of hiatal hernia was confirmed by observing a
The admitting diagnoses in hospitalized patients who were subsequently found to have a hiatal hernia reveal the protean manifestations of this condition (Table I ) . This list emphasizes the wide spectrum of diagnostic possibilities from which these pa tients were culled. All were subsequently proved to have a hiatal hernia as the pri mary disease underlying the symptoms sug gesting other diagnoses. The largest number of infants and chil dren were admitted to the hospital because
From the Surgical Service of the Children's Hospital, Wash ington, D. C. 20009, and the Department of Surgery, George Washington University School of Medicine. Read at the Forty-seventh Annual Meeting of The American Association for Thoracic Surgery, New York, N. Y-, April 17-19, 1967. ♦Associate Surgeon, the Children's Hospital, Washington, D. C. Instructor in Surgery, George Washington Univer sity School of Medicine. **Surgeon-in-Chief, the Children's Hospital, Washington, D. C. Associate Professor of Surgery, George Washing ton University School of Medicine.
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<2yrs.
I0-I6yis.
Fig. 1. Age at diagnosis in 52 infants and chil dren with hiatal hernia.
Table I. Hospital admitting diagnosis in 52 infants and children subsequently found to have hiatal hernias as their diagnosis* Feeding problem Pneumonia Hypertrophie pyloric stenosis Anemia of undetermined etiology Peptic ulcer Hiatal hernia Milk allergy Sepsis Other
13 7 6 6 6 3 2 2 7
*The 7 patients classified as "other" included lead intoxi cation, vascular ring, hypothyroidism, asthma, and congen ital heart disease.
of vomiting. This symptom usually resulted in an admitting diagnosis of "feeding prob lem" or hyperthophic pyloric stenosis. A significant number of children were admitted after respiratory complications had oc curred, and their admission diagnoses in cluded pneumonia, asthma, vascular ring, tracheoesophageal fistula (H type), and congenital heart disease. Even more striking were the patients seen later in the course of their disease. Those patients admitted with diagnoses of sepsis, hypothyroidism, lead in toxication, and chronic brain syndrome, for example, were desperately ill due to inani tion and debilitating complications of a long-standing, unrecognized, symptomatic
hiatal hernia. Malnutrition and bleeding from esophagitis brought about anemia prompting admission in 6 patients. No ad missions were based on frank upper gastro intestinal hemorrhage. Older children with hiatal hernia are recognized by the more conventional com plaints of the entity as seen in adult patients. In infants, however, there are four main symptoms (Fig. 2 ) , and the diagnosis should be considered in all babies with: (1) unexplained vomiting, (2) failure of proper growth and weight gain, (3) re peated respiratory complications, and (4) anemia of undetermined etiology. 1. Vomiting. Vomiting is a major symp tom of hiatal hernia in infants and children and was present to some degree in 92 per cent of the patients studied. In 60 per cent of the cases, régurgitation of feedings began in the first week of life. Vomiting with hiatal hernia has often been characterized as an effortless régurgitation of undigested food occurring in recumbency. There were only 4 children who vomited in this distinctive pat tern. Almost half of the patients in the infant age group vomited in a projectile manner at some time, eliciting a tentative diagnosis of hypertrophie pyloric stenosis in 7 of the group. Régurgitation,in 13 patients was described by their parents as only a slight "spitting" after feeding. Of especial importance is the fact that vomiting was so unimpressive as to be overlooked in infants who had other more prominent symptoms, such as pneumonia or failure to gain and grow. Although only 2 patients had frank hematemesis, 8 had "coffee-stained" vomitus which was found to contain occult blood. Bile staining was not observed in the vomitus of any patient. 2. Failure to thrive. "Failure to thrive," admittedly an ill-defined term, has been ap plied rather generally in pédiatrie literature to infants in whom weight gain, growth, and general development have been precariously low. All too often, after a thorough work-up (including a gastrointestinal series), the physician finds no specific disease entity.
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Fig. 2. Major symptoms of hiatal hernia in children.
Weight gain and over-all physical develop ment may be subnormal for years (Fig. 3). Other less fortunate children ultimately die, and no specific cause of death is found. A postmortem examination in such cases dis closes only general debility, iron deficiency anemia, and bronchopneumonia. Analysis of this series of patients demonstrates that some babies have a hiatal hernia as the basis for the failure to thrive. All 20 of the patients in this series classi fied as "failure to thrive" were below the third percentile in weight and growth (Fig. 4 ) . Régurgitation of feedings was present in varying degrees in most of these patients but was often not a prominent symptom. Included in this group were those babies admitted with presumptive diagnoses of chronic brain syndrome, hypothyroidism, and lead intoxication. The appearance of these starving and listless infants suggested such initial diagnoses. CASE 1. A 9-month-old male was admitted to the Children's Hospital, Washington, D. C. on Aug. 11, 1965, because of failure to thrive. He had begun vomiting shortly after birth, but this was considered inconsequential until he was 3 months of age, at which time he vomited at least half of each feeding. By 5 months of age, his weight gain had ceased. Four different formulas were tried over the next 3 months with little success. The baby was constantly hungry and would immediately re-feed after vomiting. Anti-
cholinergics and sedatives were given without benefit. By the time of admission, the baby was vomiting everything he ate or drank. On examina tion, the patient was an emaciated, listless 9month-old baby weighing 9 pounds. There were no other abnormal physical findings. Except for a hemoglobin value of 9.8 grams, laboratory studies were normal. On gastrointestinal series, a small sliding hiatal hernia was demonstrated (Fig. 5). He was placed in the upright position and started on small amounts of oral feedings. He was im proving until the fourth hospital day when he abruptly developed periods of apnea and bradycardia. It was thought that aspiration of vomitus had occurred. Despite extensive resuscitative ef forts, the child died. Postmortem examination dis closed extensive aspirated material in the tracheobronchial tree. The presence of an esophageal hia tal hernia was confirmed, and was the only other significant abnormality.
3. Recurrent aspiration pneumonitis. Re spiratory complications of hiatal hernia in this series were particularly notable and, on occasion, devastating. Repeated régurgita tion led to aspiration and pneumonia in 15 infants, comprising 30 per cent of the cases studied. Seven were admitted to the hospital with pneumonia, and in 4 there had been repeated hospitalizations for recurrent pneu monia. Cyanosis, secondary to aspiration, occurred in 1 patient who was admitted to the hospital with a diagnosis of congenital heart disease. Wheezing resulting from re peated trachea! aspiration prompted a diag nosis of asthma in another baby. Vascular
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fore esophagram was repeated. A definite small sliding esophageal hiatal hernia was demonstrated on this second study with marked gastroesopha geal reflux (Fig. 6, A). After another 15 days of intensive conservative treatment without improve ment, hiatal herniorrhaphy was carried out. The patient never vomited again. At discharge, only 14 days after surgery, she had gained a pound in weight. Subsequent growth and weight gain have been striking, and she has had no further pneu monia.
Fig. 3. A, Preoperative photograph of a 3-yearold boy with failure to thrive, who weighed only 13 pounds on admission to Children's Hospital. He was unable to stand without assistance. B, Six weeks after surgical repair of the hiatal hernia he had gained 4 pounds and could walk for the first time in his life.
ring and tracheoesophageal fistula without esophageal atresia (H-type) were suggested in 2 other infants. Often the vomiting that preceded the complication was overlooked or minimized until the more serious and even life-threatening respiratory problem supervened. CASE 2. A 10-month-old female was admitted Jan. 15, 1965, to the Children's Hospital, Wash ington, D. C , with a provisional diagnosis of failure to thrive and bronchopneumonia. The pa tient had begun vomiting on the tenth day of life. Weight gain had been negligible, and she weighed only 1 pound more than her weight at birth. Suspected aspiration pneumonia prompted admission. On examination, she was an immature, listless infant with obvious growth retardation. Weight was 8 pounds 15 ounces. Physical findings were consistent with right upper lobe pneumonia. Hemoglobin was 9.2 grams. Gastrointestinal x-ray series was reported as normal. The patient was treated with antibiotics for pneumonia. Attempts at feeding resulted immediately in vomiting. Ga vage feedings were equally ineffective. A hiatal hernia was strongly suspected clinically, and there
4. Bleeding. Anemia associated with hi atal hernia can result from chronic blood loss secondary to peptic esophagitis. 7 ' 22 Un questionably, the poor nutrition so often seen with hiatal hernia in this age plays at least a complimentary role in iron deficiency anemia.22 Frank hematemesis occurred in 2 patients, and, in 11, occult blood was found either in the vomi tus or in the stool. Six of the patients had hemoglobin values below 6 grams and required blood transfusion. Especially in this group of patients, the complications of the hiatal hernia tended to obscure the underlying disease. Only after radiographie demonstration of the hiatal hernia was the etiology of the anemia un masked and was able to be subsequendy cured by appropriate therapy of the her nia. CASE 3. A 4-week-old infant girl was admitted to Children's Hospital, Washington, D. C , on Aug. 4, 1964, because of vomiting. The patient was emaciated and poorly developed. The hemo globin was 10.6 grams. She was started on sugar water feedings, but continued to vomit. On the second hospital day, the vomitus became frankly bloody and she passed tarry stools. The hemo globin dropped to 7.5 grams. An upper gas trointestinal series showed a small but definite sliding hiatal hernia (Fig. 7). Subsequent esophagoscopy revealed distal esophagitis, but no stric ture formation. She was propped in a sitting position continuously and within 24 hours all vomiting ceased. Diet was started with small, fre quent, thickened feedings, and weight gain promptly occurred. The hemoglobin value was well maintained and there was no further bleeding from the gastrointestinal tract. The erect position was constantly maintained and then discontinued with no further symptoms. Gastric secretory studies. Gastric secre tion is normal in healthy infants immediately after birth. 3 Utilizing a technique developed
LBS. ,90%
34 32
90%
o » MEDICAL • ■ SURGICAL
30 28 10%
10%
26 24 22 20 18 16 14 12 10 8
0
3
6
9
12 15 18 21 24 27 MONTHS
PRE-THERAPY
3
6
9
12 15 18 MONTHS
21 24 27
POST-THERAPY
Fig. 4. Growth percentile in 20 infants classified as failure to thrive. Pre-therapy graph demon strates marked retardation of growth and weight gain at the time of diagnosis. Post-therapy graph shows excellent response to therapy in both the conservatively and surgically treated groups.
Fig. 5. Radiograph of patient (Case 1) shows small hiatal hernia. Note the absence of cardioesophageal sphincter action and the presence of gastric rugal folds above the diaphragm.
in our laboratory which takes into account the small specimen as well as the small subject, gastric analyses have been carried out in 335 newborn infants. In our studies, gastric secretion was shown to vary sig nificantly in a variety of disease states in infancy.4 For example, babies with a major loss of intestine and those with bronchopneumonia have highly significant altera tions in their gastric acid production (Table I I ) . Thus, it is of special interest that 11 infants with hiatal hernia, ranging in age from 9 days to 10 weeks, showed values for gastric volume, pH, total acid and rate of acid production which were comparable to those of normal infants. In our material, there is no evidence that hiatal hernia in infants is associated with gastric hyperacidi tyX-ray findings. An accurate esophagram is the sine qua non of diagnosis. Often con siderable patience is needed to demonstrate a small hernia. On occasion, a second radiologic examination of the cardia will be necessary. By far the most impressive find ing in infants with hiatal hernia at the time of fluoroscopy, however, is the presence of
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Fig. 6. A, Preoperative x-ray study of patient (Case 2) demonstrates small hiatal hernia which led to pronounced retardation of growth. B, Esophagram in the same patient, 10 days after operative correction.
total, unobstructed reflux of gastric content into the esophagus. An hiatal hernia was first seen in the first esophagram in 40 patients. A second study was necessary in the remainder. It should be noted that, in the majority of this latter group, initial studies were carried out by a radiologist not especially trained in pédiatrie
radiology. We have concluded that a radi ologist who is experienced in evaluating esophagogastric function in infants is of prime importance. Endoscopy. Esophagoscopy was not rou tinely carried out on all patients in this series. The criteria for esophagoscopy are: (1) patients who have definite evidence of
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»Λ Fig. 7. In Case 3, the hernia was difficult to demonstrate on fluoroscopy and was only transi ently seen. Definite esophagitis was demonstrated on subsequently esophagoscopy. Complete resolution of esophagitis and bleeding was obtained by conservative treatment.
Table II. Gastric analysis in infants: Response to specific diseases* Acid ratet
Weight adjusted rate§
Groups
pH
Total acidf
Well (38) Hiatal her nia (11) Short gut (7) Pneumonia (38)
3.2
6.2
0.15
54
3.6
6.2
0.15
45
2.41!
19.111
4.6
4.411
0.5811 0.11
15111 29[|
•Gastric secretory studies demonstrate normal gastric acid production in 11 infants with hiatal hernia. tmEq./lOO ml. gastric juice. îmEq./100 ml. gastric juice per minute. §(mEq./100 ml. gastric juice/Kg.) 10s. II Highly significant (p = < 0.01).
esophagitis in the from of x-ray changes, bleeding, or pain; (2) those showing a poor response to conservative therapy, and (3) those who were scheduled to have surgical repair of the hiatal hernia. (In this latter group, esophagoscopy was carried out at the beginning of the operative procedure.) Thirty-one patients, or approximately 60
per cent of the patients, were studied by esophagoscopy. If a hiatal hernia is found on x-ray examination and can be success fully treated by conservative means, there is rarely need for esophagoscopic evalua tion. Treatment and results Conservative management. The effect of gravity obtained by placing the patient in the upright position is the most important factor in conservative treatment.13 An angle of 60 degrees is recommended and should be maintained 24 hours a day (Fig. 8). This maneuver should be continued for 6 weeks after all symptoms have ceased. Fre quent, small, thickened feedings23 are an ad junct to conservative management. Although sometimes advised,12 anticholinergic drugs are not helpful and, indeed, theoretically would have an adverse effect in some pa tients. All 52 patients in this series were given a trial of conservative therapy. Thirty-two (62 per cent) responded satisfactorily and are considered medical successes as evi denced by complete disappearance of symp-
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Table III. Results of therapy Treatment Medical Surgical Herniorrhaphy Herniorrhaphy and pyloroplasty Herniorrhaphy, pyloro plasty, and vagotomy Herniorrhaphy, pyloro plasty later Totals
Fig. 8. The main facet of conservative therapy is the upright position. This position should be maintained 24 hours a day and continued for 6 weeks after all symptoms have ceased.
toms (Table III). Response to conservative management was unpredictable. In general, infants in the early weeks of life had a better chance of success with medical treatment than older children. There was no correla tion between successful conservative treat ment and size of the hernia, degree of esophagitis, or the presence of bleeding. The 2 patients with marked hematemesis, for example, responded dramatically to conservative therapy and have not bled or vomited in 14 and 20 months, respectively, since institution of therapy. A marked improvement was obvious af ter only 3 days of therapy in 10 of the 33 patients considered medical successes. In the other 22 patients who were ultimately cured by medical therapy, vomiting per sisted for several weeks. It is important to note, however, that weight gain was ade quate during this period, even though some régurgitation continued. If a satisfactory outcome was to result with conservative management, it was predictable within 3
Success
Failure
32
20 (1 death)
14
—
3
—
1
—
1
—
19
0
weeks. If there was no improvement either in vomiting or weight gain after 3 weeks of medical management, patients were con sidered candidates for surgical correction of their hiatal hernia. We have been disappointed in the results of conservative treatment in children be yond the age of 2 years. In this group, the symptomatology more closely resembles that of adult patients. Trials of medical management have been notably unsuccess ful, and all 9 of these patients have been brought to surgical correction with good re sults. The one medical fatality (Case 1) en tered the hospital almost moribund and died in 4 days in spite of supportive measures. Surgical therapy. Nineteen of the 52 pa tients have required surgical correction of the hernia. The surgical approach to the hernia was almost equally divided between the abdominal (10) and thoracic (9) routes. Results were satisfactory, regardless of the approach (Table III). All of the sur gical patients have had postoperative barium studies, and no recurrences have been found to date. There have been no surgical fatali ties. The response to surgical correction of the hiatus hernia has been dramatic. In 12 pa tients, there was an almost immediate cessa tion of vomiting combined with a spurt in weight and growth. In 6, prompt weight gain occurred, although some vomiting con tinued intermittently for several weeks and
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then stopped. In 1 patient, vomiting con tinued until a pyloroplasty was done. Pro longed gastric emptying was found in 3 chil dren in the preoperative gastrointestinal xray examinations, and these patients had pyloroplasty performed at the time of hiatal hernia repair (Table III). Discussion The etiology of hiatal hernia in infancy and childhood is still not understood. Some authors have believed that congenital short ening of the esophagus produces a hernia.23' 5 · " The fact that the herniated cardia of the stomach has been brought below the diaphragm easily in most surgical series7· 22 argues against this concept. Roviralta20 has coined the term "phrenicopyloric syn drome," believing the basis for congenital hiatal hernia is a functional pylorospasm. Johnston17 concurs and has advocated py loroplasty and possible vagotomy as the operation of choice for hiatal hernia in chil dren. A poorly developed right diaphrag matic crus,2·22· 14 an inadequate tethering effect of the left gastric artery,6 and lax phrenicoesophageal ligaments7 are other conceptions of the etiology of a congenital hiatal hernia. At the present time, however, the precise etiology remains obscure. It should be made clear that accepted symptomatology, diagnostic findings, and therapeutic approaches in the adult with an hiatal hernia are not transferrable to the infant. For example, repeated and often projectile vomiting present in infants is not a prominent symptom in an adult with an hiatal hernia. Although infants are unable to complain of pain, the majority act hun gry after vomiting and readily re-feed. In the adult, rarely does the radiologist see the marked esophageal reflux present in infants with this condition. Weight reduction and antacids have no place in the treatment of infants and children with hiatal hernia. Al though the semi-upright position is used in the treatment of both infants and adults, in adults, one desires symptomatic improve ment, in infants, cure.17 In most infants, a hernia proved at initial esophagogram can
not be demonstrated after prolonged relief of symptoms by conservative measures. This significant group of divergent find ings suggests a different etiology of hiatal hernia in the adult and infant. Such a possi bility is reinforced by the age incidence of hiatal hernia found in the present series (Fig. 1). The majority (43) were under 2 years of age. A significant number of chil dren were 10 years or older; this latter group more closely simulated adults with hiatal hernia. Only 3 patients were found to have hiatal hernia between 2 and 10 years of age. This age grouping would be un expected if the etiology of hiatal hernia was identical in the infant and adult. It is our feeling that hiatal hernia in infancy is a congenital defect present at birth which is capable of undergoing spontaneous im provement as is seen in umbilical hernia in children. Conversely, hiatal hernia in adults most likely represents the result of a gradual degeneration of ligaments and muscles with aging. 17 ' 18 In 1947, Berenberg and Neuhauser8 fo cused attention on infants with gastroesophageal reflux without hiatal hernia. The wellknown term "chalasia" was coined for this condition. With the increasing utilization of cinefluoroscopy, chalasia has been reported less frequently. It may well be that many cases of chalasia reported previously were, in truth, small hiatal hernias. It matters lit tle, however, whether chalasia represents a small, unrecognized hiatal hernia or a neuromuscular failure of the esophagocardiac valve mechanism. 14 · 21 As others have re ported,9· 1 0 · 1 9 we have seen definite peptic esophagitis in patients with chalasia in whom an hiatal hernia could not be seen. The treatment should be identical. Hiebert and Belsey16 have reported good results with an anti-reflux operation in patients suffering from esophageal reflux in whom no hiatal hernia could be demonstrated. Four patients are included in this review with gastroesophageal reflux without an anatomical hiatal hernia. One of these patients (Fig. 9) required surgical intervention and was there by cured where medical therapy had been
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Fig. 9. Radiographs of child with gastroesopha geal reflux (chalasia). A hiatal hernia could not be demonstrated on two upper gastrointestinal series. Marked and uncontrolled reflux was present in both series.
unsuccessful. We believe that the principle advanced by Hiebert and Belsey16 is appli cable to some infants with uncontrolled gastroesophageal reflux. The fact that two thirds of the babies with hiatal hernia were cured by medical measures, and the absence of satisfactory guidelines in predicting which patients will respond to conservative therapy, dictates a need for vigorous trial of medical manage ment in all infants with hiatal hernia. The exception is the patient who already has developed an esophageal stricture for whom prompt surgery is mandatory. The symp tomatic response to medical therapy is often prompt and gratifying. Results are unmis takable after 3 weeks of intensive medical therapy. As long as there is definite evi dence of improvement of even a modest degree, the medical regimen should be con
tinued. However, if vomiting continues and there is no weight gain after 3 weeks have elapsed, patients are considered surgical candidates. With this potentially devastating disease, further time should not be squan dered in useless experimentation with antispasmodics and formula changes while the infant languishes. The results of surgical re pair of the hernia are pleasing and should not be denied when the above criteria have been met. Because of the logical concept behind the Allison repair1 and the simplicity of its ac complishment, this surgical approach was used in all patients herein reported. There was no mortality, and complications were notably minimal. A difficult problem has been the selection of those patients requiring pyloroplasty in addition to herniorrhaphy. Those patients with partial pyloric outlet obstruction, in dicated by delayed gastric emptying, should have pyloroplasty at the time of hiatal her nia repair. 12 ' 15 Since there are no absolutes for normal emptying time, delayed gastric emptying remains a subjective impression of the radiologist. Nevertheless, at this time, the finding of delayed gastric emptying on preoperative gastrointestinal series is the most valuable clue to the need for cocomitant pyloroplasty. Pyloroplasty adds to the morbidity of the surgical procedure. Since only 4 patients of the 19 requiring surgery also needed pyloroplasty, we do not advocate routine concomitant pyloroplasty with hiatal hernia repair. Improved radiographie equipment has en hanced the diagnosis of hiatal hernia, par ticularly in babies. It should be emphasized, however, that a radiologist familiar with xray diagnosis in children is indispensable in delineating the small and inconstantly seen hiatal hernias. Of even greater importance is the need for awareness by the pediatri cian of the role of hiatal hernia in many in fants and children who eat poorly and fail to grow. At our hospital, the diagnosis of hiatal hernia was established in only 7 chil dren between 1957 and 1963. Beginning in 1963, when increasing attention was focused
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on this problem, hiatal hernia was found in 45 infants and children in a 3-year span. This does not reflect an increase in the incidence of this disease but represents the result of improved recognition on the part of pédiatrie practitioners. Conclusions 1. Hernia of the esophageal hiatus is a surprisingly common gastrointestinal ab normality in pédiatrie patients, which differs significantly in symptomatology and therapy from the disease as seen in the adult popu lation. 2. The four main symptoms of hiatal hernia in infants are: (1) vomiting, (2) failure to thrive, (3) respiratory complica tions, and (4) anemia. 3. Diagnosis requires a precise esophagram performed by a radiologist experi enced in work with children. 4. Gastric hyperacidity has not been found in infants with congenital esophageal hiatal hernia. 5. Medical therapy has been successful in almost two thirds on this group of 52 infants and children with hiatal hernia and cardioesophageal reflux. 6. Surgical correction is effective and should be carried out on those patients who are not improved by medical means or who already have an esophageal stricture when seen. 7. Pyloroplasty may be necessary in some babies who undergo surgical correction of esophageal hiatal hernia, but is not recom mended as a routine complimentary proce dure. We would like to express our appreciation to Dr. John Washington, Dr. Albert Modlin, and Dr. Morris Feitel for allowing us to extend sur gical care to their patients described in the text. In addition, we are grateful to Dr. Gordon Avery, Director of Newborn Services, for his help in the conservative management of a number of the infants. REFERENCES 1 Allison, P. R.: Reflux Esophagitis, Sliding Hiatal Hernia, and the Anatomy of Repair, Surg., Gynec. & Obst. 92: 419, 1951.
2 Allison, R.: The Diaphragm in Gibbon, John, H., Jr., editor, Surgery of the Chest, Philadel phia, 1962, W. B. Saunders Co., p. 254. 3 Avery, G. B., Randolph, J. G., and Weaver, T. H.: Gastric Acidity in the First Day of Life, Pediatrics 37: 1005, 1966. 4 Avery, G. B., Randolph, J. G., and Weaver, T. H.: Gastric Response to Specific Disease in Infants, Pediatrics 38: 874, 1966. 5 Barrett, N . R.: Chronic Peptic Ulcer of the Oesophagus and Oesophagitis, Brit. J. Surg. 38: 175, 1950. 6 Barrett, N . R.: Hiatus Hernia, Brit. J. Surg. 42: 231, 1954. 7 Belsey, R.: Surgery of the Diaphragm in Brown, James, J. M., editor, Surgery of Child ren, Baltimore, 1963, Williams & Wilkins Co., p. 762. 8 Berenberg, W., and Neuhauser, E. D.: Car dioesophageal Relax (Chalasia) As a Cause of Vomiting in Infants, Pediatrics 5: 414, 1950. 9 Bettex, M., and Stillhart, H.: Operation for Hiatus Hernia and Cardioesophageal Chalasia by Fundoplication After Nissen, Surgery 55: 451, 1964. 10 Blank, L., and Pew, W. L.: Cardioesophageal Relaxation (Chalasia) Studies on the Normal Infant, A m . J. Roentgenol. 65: 40, 1956. 11 Botha, G. S. M.: The Gastro-oesophageal Re gion in Infants, Arch. Dis. Child. 33: 78, 1958. 12 Brown, F . B., et al.: Medical and Surgical Management of Esophageal Hiatus Hernia in Children, Bol. Med. Hosp. Infant. (Mex.) 41: 17, 1963. 13 Filler, R. E., Randolph, J. G., and Gross, R. E.: Esophageal Hiatus Hernia in Infants and Children, J. THORACIC & CARDIOVAS. SURG.
47:
551, 1964. 14 Forshall, I.: The Cardio-esophageal Syndrome in Childhood, Arch. Dis. Child. 30: 46, 1955. 15 Gross, R. E.: Thoracic Surgery for Infants, J. THORACIC & CARDIOVAS. SURG. 48:
162,
1964.
16 Hiebert, C. A., and Belsey, R.: Incompetency of the Gastric Cardia Without Radiological Evidence of Hiatus Hernia, J. THORACIC & CARDIOVAS. SURG. 42:
352,
1961.
17 Johnston, J. H.: Hiatal Hernia in Childhood, Arch. Dis. Childh. 35: 61, 1960. 18 Listerud, M. B.: Details of Interest and Con troversy in the Anatomy of the Esophageal Hiatus and Hiatal Hernia, Surg. Clin. North America 44: 1211, 1964. 19 Ravitch, M.: Chalasia, Pédiatrie Surgery, Chicago, 1962, Yearbook Medical Publishers, Inc., vol. I, p. 299. 20 Roviralta, E.: Less vomissements du nourrison, Paris, 1952, Flamarion. 21 Strawczynski, H.: The Behavior of the Lower
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Esophageal Sphincter in Infants and Its Rela tionship to Gastro-esophageal Régurgitation, J. Pediat. 64: 17, 1964. 22 Waterson, D.: Hiatus Hernia, Pédiatrie Sur gery, Chicago, 1962, Yearbook Medical Pub lishers, Inc., vol. I, p. 301. 23 Wyllie, W. G., and Field, E. C : The Etiology of Intermittent Oesophageal Régurgitation and Haematemesis in Infants, Arch. Dis. Child. 21: 218, 1946.
Discussion DR. CLEMENT A. HIEBERT Portland, Me.
Dr. Randolph has just presented in his cus tomarily clear and concise manner the clinical picture of the child with a valveless cardia. Quite properly, he emphasized that the infant, like the adult, may get into serious trouble with gastro esophageal reflux even when no hernia can be demonstrated. Chronic vomiting is the classic hallmark of this entity and, as most normal children vomit from time to time, the problem is to distinguish Dr. Randolph's baby roasting his esophagus with corrosive ferments from Shakespeare's infant harmlessly "mewling and puking in his mother's arms." Which vomiters will improve and which will not? Another problem is to persuade pediatricians to allow surgery before the irreversible damage to esophagus, lungs, or growth pattern has oc curred. Infatuation with a "normal" upper GI series has resulted in many a child having spinal taps, electrolyte studies, and abdominal films galore before vomiting of a nonobstructive nature is even thought of. The surgeon himself may be to blame. [Slide] This slide depicts an infant operated upon in the mistaken belief he had pyloric stenosis. A Ramstedt operation was none theless done but the child continued to vomit and became metabolically depleted. Here he lies, hun grily sucking his fingers moments after vomiting. The picture is the counterpart of the nausea-free vomiting in the adult with an incompetent and patulous cardia. I esophagoscoped this youngster and he had raging esophagitis; but suitable repair of the car dia stopped the vomiting and the esophagus looked normal by esophagoscopy a week later. Pyloroplasty alone is not the answer. In contrast with the authors I believe that the adult hiatal hernia frequently is progression of the infantile condition. Granted, there may be a long symptom-free interval, but if you quiz pa tients carefully, after a successful operation, many will acknowledge no previous appreciation ever of what it was to swallow and digest normally. Others will recall parents having said they were
terrible feeding problems in infancy. A celebrated patient of ours had vomited every day of his life for as long as he could remember. The only way he survived was to ruminate. As a child he earned understandable notoriety, when he would stand on a bridge above the train tracks and, at the command of his associates, eject the contents of his stomach into the stack of any designated pas sing train! An operation on his cardia when he was 35 years of age cleared matters up, trains included. I congratulate the authors. Actually, there is no real need to praise them, for their sterling presentation speaks for itself. I predict this paper will find its way into the classic literature on the subject. DR. I O H N J.
CINCOTTI
Sepulveda, Calif.
I thoroughly enjoyed Dr. Randolph's excellent paper, and Dr. Hiebert's delightful discussion. I would like to comment on the Allison repair, as used by the authors. Mr. Allison was impressed with my 1961 modification of the crural aspect of his repair and agreed that it might be uni versally used. In the late 1940's, Dr. Harken and I were pleased to be guided through an Allison proce dure by Mr. Philip Allison. My review of 111 cases in 1960 revealed a 10 per cent recurrence rate. In 27 of this group, I used an earlier modi fication, that is, compressed Ivalon (later, Teflon mesh) stents for crural buttressing, with the ex pectation of subsequent fiber ingrowth forming an aponeurosis-like reinforcement of the crural hi atus. However, the recurrence rate was not af fected by this change. In 1961 I began a series in which the original Allison repair was modified by including firm bites of the posterior gastric aspect of the esophagogastric junction, within the most caudally placed, yet untied, crural limb approximating sutures, and/or approximating the origins of the crura to the posterior gastric junction. Posterior junctional fixation sutures are more easily placed using the thoracic approach to the hiatus. We use identifying clips for the serial study of the di ameter of the reconstructed hiatus and the fixed junction. The Samuels' hemostatic clip (Week) is ideal for this purpose for, with slight closing pressure, only the tips of the clip approximate firmly. No tissue is crushed, while the permanently fixed clips permit easy anatomical identification. [Slide] Operative technique. [Slide] Clip rela tionship. I believe that, with this newer modification, hiatal widening will occur to roughly the same extent that the unmodified repair permits recur rences. If the posterier junctional anchoring su tures hold, however, recurrences are unlikely.
Journal of
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Lilly and Randolph
In closing, our pédiatrie and medical colleagues have been reluctant to advise early repair of symptomatic hiatal hernias because of the un acceptable recurrence rate. If we can offer a pro cedure that has a low rate of failure, we may see a significant decline in the incidence of pa tients with the damaged or destroyed sphincter or, what is all too often, an iatrogenously supervised esophageal stricture. D R . O T T O C. Baltimore,
BRANTIGAN
Md.
I rise to comment on an important aspect of this problem. I am certain the essayists are well aware of it. I would like to point out that gastroesophageal reflux is not necessarily esophageal hiatal diaphragmatic hernia, nor is esophagitis necessarily esophageal hiatal hernia. One can have gastroesophageal reflux without hernia, and one can have marked esophagitis without hiatal hernia. If treatment is to be rational and simplified, there
Thoracic and Cardiovascular Surgery
must be made a careful distinction between esophageal hiatal diaphragmatic hernia and esophagitis since the conditions may occur to gether or independently of each other. DR. R A N D O L P H (Closing) I would like to thank Dr. Brantigan for em phasizing that reflux may be troublesome without definite evidence of a hiatal hernia. We appreci ate Dr. Cincotti's comments on improvements in technique. I wish to thank Dr. Hiebert for his remarks. The question about which of the many infants who vomit should be studied for possible hiatal hernia is very much to the point. I would say that any baby who vomits to any degree, and who also shows any of the other three major symptoms, namely, failure to thrive, or pneumonitis, or anemia should be investigated. In closing, I must concede that we have had no child among our patients who could vomit down a train's smokestack.
JU/sophageal hiatus hernia is a common congenital defect in infants and children. The symptoms and ultimate morbidity of this condition in the young are primarily re lated to gastroesophageal reflux, not to esophagitis. In infants, reflux of gastric con tent is rarely associated with gastric hyper acidity; instead, a clinical picture evolves which may merge with other ill-defined feeding problems of this age group. If un recognized, these symptoms, which are sub tle yet commonplace, can lead to deva stating malnutrition and suppression of growth in the early years of life. This report outlines the clinical characteristics and ther apeutic management of 52 consecutive cases of hiatal hernia in children seen on the Sur gical Service of the Children's Hospital, Washington, D. C , from January, 1957, through December, 1965.
definite projection of the stomach above the diaphragm on barium studies of the upper gastrointestinal tract. All hernias were of the sliding type. In 4 patients, marked gastroesophageal reflux was demon strated during barium study; but a hjatal hernia could not be visualized despite several radiographie attempts. The majority (47) of the patients were examined by cinefluoroscopy and the image intensifier. A gastrointestinal series was obtained after treatment in 40 of the 52 patients; all of the surgical patients were so studied. The fol low-up period ranged from 1 to 5 years. Forty-three patients were under 2 years of age, and 25 were less than 3 months old (Fig. 1). Six of the children were 10 years of age or older. Only 3 children between the ages of 2 and 10 years were found to have hiatal hernia.
Patient material
Symptoms
In 48 of 52 children, the diagnosis of hiatal hernia was confirmed by observing a
The admitting diagnoses in hospitalized patients who were subsequently found to have a hiatal hernia reveal the protean manifestations of this condition (Table I ) . This list emphasizes the wide spectrum of diagnostic possibilities from which these pa tients were culled. All were subsequently proved to have a hiatal hernia as the pri mary disease underlying the symptoms sug gesting other diagnoses. The largest number of infants and chil dren were admitted to the hospital because
From the Surgical Service of the Children's Hospital, Wash ington, D. C. 20009, and the Department of Surgery, George Washington University School of Medicine. Read at the Forty-seventh Annual Meeting of The American Association for Thoracic Surgery, New York, N. Y-, April 17-19, 1967. ♦Associate Surgeon, the Children's Hospital, Washington, D. C. Instructor in Surgery, George Washington Univer sity School of Medicine. **Surgeon-in-Chief, the Children's Hospital, Washington, D. C. Associate Professor of Surgery, George Washing ton University School of Medicine.
42
Volume 55
Hiatal hernia and gastroesophageal reflux
Number 1
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January, 1968
<2yrs.
I0-I6yis.
Fig. 1. Age at diagnosis in 52 infants and chil dren with hiatal hernia.
Table I. Hospital admitting diagnosis in 52 infants and children subsequently found to have hiatal hernias as their diagnosis* Feeding problem Pneumonia Hypertrophie pyloric stenosis Anemia of undetermined etiology Peptic ulcer Hiatal hernia Milk allergy Sepsis Other
13 7 6 6 6 3 2 2 7
*The 7 patients classified as "other" included lead intoxi cation, vascular ring, hypothyroidism, asthma, and congen ital heart disease.
of vomiting. This symptom usually resulted in an admitting diagnosis of "feeding prob lem" or hyperthophic pyloric stenosis. A significant number of children were admitted after respiratory complications had oc curred, and their admission diagnoses in cluded pneumonia, asthma, vascular ring, tracheoesophageal fistula (H type), and congenital heart disease. Even more striking were the patients seen later in the course of their disease. Those patients admitted with diagnoses of sepsis, hypothyroidism, lead in toxication, and chronic brain syndrome, for example, were desperately ill due to inani tion and debilitating complications of a long-standing, unrecognized, symptomatic
hiatal hernia. Malnutrition and bleeding from esophagitis brought about anemia prompting admission in 6 patients. No ad missions were based on frank upper gastro intestinal hemorrhage. Older children with hiatal hernia are recognized by the more conventional com plaints of the entity as seen in adult patients. In infants, however, there are four main symptoms (Fig. 2 ) , and the diagnosis should be considered in all babies with: (1) unexplained vomiting, (2) failure of proper growth and weight gain, (3) re peated respiratory complications, and (4) anemia of undetermined etiology. 1. Vomiting. Vomiting is a major symp tom of hiatal hernia in infants and children and was present to some degree in 92 per cent of the patients studied. In 60 per cent of the cases, régurgitation of feedings began in the first week of life. Vomiting with hiatal hernia has often been characterized as an effortless régurgitation of undigested food occurring in recumbency. There were only 4 children who vomited in this distinctive pat tern. Almost half of the patients in the infant age group vomited in a projectile manner at some time, eliciting a tentative diagnosis of hypertrophie pyloric stenosis in 7 of the group. Régurgitation,in 13 patients was described by their parents as only a slight "spitting" after feeding. Of especial importance is the fact that vomiting was so unimpressive as to be overlooked in infants who had other more prominent symptoms, such as pneumonia or failure to gain and grow. Although only 2 patients had frank hematemesis, 8 had "coffee-stained" vomitus which was found to contain occult blood. Bile staining was not observed in the vomitus of any patient. 2. Failure to thrive. "Failure to thrive," admittedly an ill-defined term, has been ap plied rather generally in pédiatrie literature to infants in whom weight gain, growth, and general development have been precariously low. All too often, after a thorough work-up (including a gastrointestinal series), the physician finds no specific disease entity.
44
Journal of
Lilly and Randolph
Thoracic and Cardiovascular Surgery
Fig. 2. Major symptoms of hiatal hernia in children.
Weight gain and over-all physical develop ment may be subnormal for years (Fig. 3). Other less fortunate children ultimately die, and no specific cause of death is found. A postmortem examination in such cases dis closes only general debility, iron deficiency anemia, and bronchopneumonia. Analysis of this series of patients demonstrates that some babies have a hiatal hernia as the basis for the failure to thrive. All 20 of the patients in this series classi fied as "failure to thrive" were below the third percentile in weight and growth (Fig. 4 ) . Régurgitation of feedings was present in varying degrees in most of these patients but was often not a prominent symptom. Included in this group were those babies admitted with presumptive diagnoses of chronic brain syndrome, hypothyroidism, and lead intoxication. The appearance of these starving and listless infants suggested such initial diagnoses. CASE 1. A 9-month-old male was admitted to the Children's Hospital, Washington, D. C. on Aug. 11, 1965, because of failure to thrive. He had begun vomiting shortly after birth, but this was considered inconsequential until he was 3 months of age, at which time he vomited at least half of each feeding. By 5 months of age, his weight gain had ceased. Four different formulas were tried over the next 3 months with little success. The baby was constantly hungry and would immediately re-feed after vomiting. Anti-
cholinergics and sedatives were given without benefit. By the time of admission, the baby was vomiting everything he ate or drank. On examina tion, the patient was an emaciated, listless 9month-old baby weighing 9 pounds. There were no other abnormal physical findings. Except for a hemoglobin value of 9.8 grams, laboratory studies were normal. On gastrointestinal series, a small sliding hiatal hernia was demonstrated (Fig. 5). He was placed in the upright position and started on small amounts of oral feedings. He was im proving until the fourth hospital day when he abruptly developed periods of apnea and bradycardia. It was thought that aspiration of vomitus had occurred. Despite extensive resuscitative ef forts, the child died. Postmortem examination dis closed extensive aspirated material in the tracheobronchial tree. The presence of an esophageal hia tal hernia was confirmed, and was the only other significant abnormality.
3. Recurrent aspiration pneumonitis. Re spiratory complications of hiatal hernia in this series were particularly notable and, on occasion, devastating. Repeated régurgita tion led to aspiration and pneumonia in 15 infants, comprising 30 per cent of the cases studied. Seven were admitted to the hospital with pneumonia, and in 4 there had been repeated hospitalizations for recurrent pneu monia. Cyanosis, secondary to aspiration, occurred in 1 patient who was admitted to the hospital with a diagnosis of congenital heart disease. Wheezing resulting from re peated trachea! aspiration prompted a diag nosis of asthma in another baby. Vascular
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Hiatal hernia and gastroesophageal reflux
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January, 1968
fore esophagram was repeated. A definite small sliding esophageal hiatal hernia was demonstrated on this second study with marked gastroesopha geal reflux (Fig. 6, A). After another 15 days of intensive conservative treatment without improve ment, hiatal herniorrhaphy was carried out. The patient never vomited again. At discharge, only 14 days after surgery, she had gained a pound in weight. Subsequent growth and weight gain have been striking, and she has had no further pneu monia.
Fig. 3. A, Preoperative photograph of a 3-yearold boy with failure to thrive, who weighed only 13 pounds on admission to Children's Hospital. He was unable to stand without assistance. B, Six weeks after surgical repair of the hiatal hernia he had gained 4 pounds and could walk for the first time in his life.
ring and tracheoesophageal fistula without esophageal atresia (H-type) were suggested in 2 other infants. Often the vomiting that preceded the complication was overlooked or minimized until the more serious and even life-threatening respiratory problem supervened. CASE 2. A 10-month-old female was admitted Jan. 15, 1965, to the Children's Hospital, Wash ington, D. C , with a provisional diagnosis of failure to thrive and bronchopneumonia. The pa tient had begun vomiting on the tenth day of life. Weight gain had been negligible, and she weighed only 1 pound more than her weight at birth. Suspected aspiration pneumonia prompted admission. On examination, she was an immature, listless infant with obvious growth retardation. Weight was 8 pounds 15 ounces. Physical findings were consistent with right upper lobe pneumonia. Hemoglobin was 9.2 grams. Gastrointestinal x-ray series was reported as normal. The patient was treated with antibiotics for pneumonia. Attempts at feeding resulted immediately in vomiting. Ga vage feedings were equally ineffective. A hiatal hernia was strongly suspected clinically, and there
4. Bleeding. Anemia associated with hi atal hernia can result from chronic blood loss secondary to peptic esophagitis. 7 ' 22 Un questionably, the poor nutrition so often seen with hiatal hernia in this age plays at least a complimentary role in iron deficiency anemia.22 Frank hematemesis occurred in 2 patients, and, in 11, occult blood was found either in the vomi tus or in the stool. Six of the patients had hemoglobin values below 6 grams and required blood transfusion. Especially in this group of patients, the complications of the hiatal hernia tended to obscure the underlying disease. Only after radiographie demonstration of the hiatal hernia was the etiology of the anemia un masked and was able to be subsequendy cured by appropriate therapy of the her nia. CASE 3. A 4-week-old infant girl was admitted to Children's Hospital, Washington, D. C , on Aug. 4, 1964, because of vomiting. The patient was emaciated and poorly developed. The hemo globin was 10.6 grams. She was started on sugar water feedings, but continued to vomit. On the second hospital day, the vomitus became frankly bloody and she passed tarry stools. The hemo globin dropped to 7.5 grams. An upper gas trointestinal series showed a small but definite sliding hiatal hernia (Fig. 7). Subsequent esophagoscopy revealed distal esophagitis, but no stric ture formation. She was propped in a sitting position continuously and within 24 hours all vomiting ceased. Diet was started with small, fre quent, thickened feedings, and weight gain promptly occurred. The hemoglobin value was well maintained and there was no further bleeding from the gastrointestinal tract. The erect position was constantly maintained and then discontinued with no further symptoms. Gastric secretory studies. Gastric secre tion is normal in healthy infants immediately after birth. 3 Utilizing a technique developed
LBS. ,90%
34 32
90%
o » MEDICAL • ■ SURGICAL
30 28 10%
10%
26 24 22 20 18 16 14 12 10 8
0
3
6
9
12 15 18 21 24 27 MONTHS
PRE-THERAPY
3
6
9
12 15 18 MONTHS
21 24 27
POST-THERAPY
Fig. 4. Growth percentile in 20 infants classified as failure to thrive. Pre-therapy graph demon strates marked retardation of growth and weight gain at the time of diagnosis. Post-therapy graph shows excellent response to therapy in both the conservatively and surgically treated groups.
Fig. 5. Radiograph of patient (Case 1) shows small hiatal hernia. Note the absence of cardioesophageal sphincter action and the presence of gastric rugal folds above the diaphragm.
in our laboratory which takes into account the small specimen as well as the small subject, gastric analyses have been carried out in 335 newborn infants. In our studies, gastric secretion was shown to vary sig nificantly in a variety of disease states in infancy.4 For example, babies with a major loss of intestine and those with bronchopneumonia have highly significant altera tions in their gastric acid production (Table I I ) . Thus, it is of special interest that 11 infants with hiatal hernia, ranging in age from 9 days to 10 weeks, showed values for gastric volume, pH, total acid and rate of acid production which were comparable to those of normal infants. In our material, there is no evidence that hiatal hernia in infants is associated with gastric hyperacidi tyX-ray findings. An accurate esophagram is the sine qua non of diagnosis. Often con siderable patience is needed to demonstrate a small hernia. On occasion, a second radiologic examination of the cardia will be necessary. By far the most impressive find ing in infants with hiatal hernia at the time of fluoroscopy, however, is the presence of
Volume 55 Number 1
Hiatal hernia and gastroesophageal reflux
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January, 1968
Fig. 6. A, Preoperative x-ray study of patient (Case 2) demonstrates small hiatal hernia which led to pronounced retardation of growth. B, Esophagram in the same patient, 10 days after operative correction.
total, unobstructed reflux of gastric content into the esophagus. An hiatal hernia was first seen in the first esophagram in 40 patients. A second study was necessary in the remainder. It should be noted that, in the majority of this latter group, initial studies were carried out by a radiologist not especially trained in pédiatrie
radiology. We have concluded that a radi ologist who is experienced in evaluating esophagogastric function in infants is of prime importance. Endoscopy. Esophagoscopy was not rou tinely carried out on all patients in this series. The criteria for esophagoscopy are: (1) patients who have definite evidence of
Journal of
48
Thoracic and Cardiovascular
Lilly and Randolph
Surgery
»Λ Fig. 7. In Case 3, the hernia was difficult to demonstrate on fluoroscopy and was only transi ently seen. Definite esophagitis was demonstrated on subsequently esophagoscopy. Complete resolution of esophagitis and bleeding was obtained by conservative treatment.
Table II. Gastric analysis in infants: Response to specific diseases* Acid ratet
Weight adjusted rate§
Groups
pH
Total acidf
Well (38) Hiatal her nia (11) Short gut (7) Pneumonia (38)
3.2
6.2
0.15
54
3.6
6.2
0.15
45
2.41!
19.111
4.6
4.411
0.5811 0.11
15111 29[|
•Gastric secretory studies demonstrate normal gastric acid production in 11 infants with hiatal hernia. tmEq./lOO ml. gastric juice. îmEq./100 ml. gastric juice per minute. §(mEq./100 ml. gastric juice/Kg.) 10s. II Highly significant (p = < 0.01).
esophagitis in the from of x-ray changes, bleeding, or pain; (2) those showing a poor response to conservative therapy, and (3) those who were scheduled to have surgical repair of the hiatal hernia. (In this latter group, esophagoscopy was carried out at the beginning of the operative procedure.) Thirty-one patients, or approximately 60
per cent of the patients, were studied by esophagoscopy. If a hiatal hernia is found on x-ray examination and can be success fully treated by conservative means, there is rarely need for esophagoscopic evalua tion. Treatment and results Conservative management. The effect of gravity obtained by placing the patient in the upright position is the most important factor in conservative treatment.13 An angle of 60 degrees is recommended and should be maintained 24 hours a day (Fig. 8). This maneuver should be continued for 6 weeks after all symptoms have ceased. Fre quent, small, thickened feedings23 are an ad junct to conservative management. Although sometimes advised,12 anticholinergic drugs are not helpful and, indeed, theoretically would have an adverse effect in some pa tients. All 52 patients in this series were given a trial of conservative therapy. Thirty-two (62 per cent) responded satisfactorily and are considered medical successes as evi denced by complete disappearance of symp-
Volume 55 Number 1
Hiatal hernia and gastroesophageal reflux
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January, 19o8
Table III. Results of therapy Treatment Medical Surgical Herniorrhaphy Herniorrhaphy and pyloroplasty Herniorrhaphy, pyloro plasty, and vagotomy Herniorrhaphy, pyloro plasty later Totals
Fig. 8. The main facet of conservative therapy is the upright position. This position should be maintained 24 hours a day and continued for 6 weeks after all symptoms have ceased.
toms (Table III). Response to conservative management was unpredictable. In general, infants in the early weeks of life had a better chance of success with medical treatment than older children. There was no correla tion between successful conservative treat ment and size of the hernia, degree of esophagitis, or the presence of bleeding. The 2 patients with marked hematemesis, for example, responded dramatically to conservative therapy and have not bled or vomited in 14 and 20 months, respectively, since institution of therapy. A marked improvement was obvious af ter only 3 days of therapy in 10 of the 33 patients considered medical successes. In the other 22 patients who were ultimately cured by medical therapy, vomiting per sisted for several weeks. It is important to note, however, that weight gain was ade quate during this period, even though some régurgitation continued. If a satisfactory outcome was to result with conservative management, it was predictable within 3
Success
Failure
32
20 (1 death)
14
—
3
—
1
—
1
—
19
0
weeks. If there was no improvement either in vomiting or weight gain after 3 weeks of medical management, patients were con sidered candidates for surgical correction of their hiatal hernia. We have been disappointed in the results of conservative treatment in children be yond the age of 2 years. In this group, the symptomatology more closely resembles that of adult patients. Trials of medical management have been notably unsuccess ful, and all 9 of these patients have been brought to surgical correction with good re sults. The one medical fatality (Case 1) en tered the hospital almost moribund and died in 4 days in spite of supportive measures. Surgical therapy. Nineteen of the 52 pa tients have required surgical correction of the hernia. The surgical approach to the hernia was almost equally divided between the abdominal (10) and thoracic (9) routes. Results were satisfactory, regardless of the approach (Table III). All of the sur gical patients have had postoperative barium studies, and no recurrences have been found to date. There have been no surgical fatali ties. The response to surgical correction of the hiatus hernia has been dramatic. In 12 pa tients, there was an almost immediate cessa tion of vomiting combined with a spurt in weight and growth. In 6, prompt weight gain occurred, although some vomiting con tinued intermittently for several weeks and
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Lilly and Randolph
Thoracic and Cardiovascular Surgery
then stopped. In 1 patient, vomiting con tinued until a pyloroplasty was done. Pro longed gastric emptying was found in 3 chil dren in the preoperative gastrointestinal xray examinations, and these patients had pyloroplasty performed at the time of hiatal hernia repair (Table III). Discussion The etiology of hiatal hernia in infancy and childhood is still not understood. Some authors have believed that congenital short ening of the esophagus produces a hernia.23' 5 · " The fact that the herniated cardia of the stomach has been brought below the diaphragm easily in most surgical series7· 22 argues against this concept. Roviralta20 has coined the term "phrenicopyloric syn drome," believing the basis for congenital hiatal hernia is a functional pylorospasm. Johnston17 concurs and has advocated py loroplasty and possible vagotomy as the operation of choice for hiatal hernia in chil dren. A poorly developed right diaphrag matic crus,2·22· 14 an inadequate tethering effect of the left gastric artery,6 and lax phrenicoesophageal ligaments7 are other conceptions of the etiology of a congenital hiatal hernia. At the present time, however, the precise etiology remains obscure. It should be made clear that accepted symptomatology, diagnostic findings, and therapeutic approaches in the adult with an hiatal hernia are not transferrable to the infant. For example, repeated and often projectile vomiting present in infants is not a prominent symptom in an adult with an hiatal hernia. Although infants are unable to complain of pain, the majority act hun gry after vomiting and readily re-feed. In the adult, rarely does the radiologist see the marked esophageal reflux present in infants with this condition. Weight reduction and antacids have no place in the treatment of infants and children with hiatal hernia. Al though the semi-upright position is used in the treatment of both infants and adults, in adults, one desires symptomatic improve ment, in infants, cure.17 In most infants, a hernia proved at initial esophagogram can
not be demonstrated after prolonged relief of symptoms by conservative measures. This significant group of divergent find ings suggests a different etiology of hiatal hernia in the adult and infant. Such a possi bility is reinforced by the age incidence of hiatal hernia found in the present series (Fig. 1). The majority (43) were under 2 years of age. A significant number of chil dren were 10 years or older; this latter group more closely simulated adults with hiatal hernia. Only 3 patients were found to have hiatal hernia between 2 and 10 years of age. This age grouping would be un expected if the etiology of hiatal hernia was identical in the infant and adult. It is our feeling that hiatal hernia in infancy is a congenital defect present at birth which is capable of undergoing spontaneous im provement as is seen in umbilical hernia in children. Conversely, hiatal hernia in adults most likely represents the result of a gradual degeneration of ligaments and muscles with aging. 17 ' 18 In 1947, Berenberg and Neuhauser8 fo cused attention on infants with gastroesophageal reflux without hiatal hernia. The wellknown term "chalasia" was coined for this condition. With the increasing utilization of cinefluoroscopy, chalasia has been reported less frequently. It may well be that many cases of chalasia reported previously were, in truth, small hiatal hernias. It matters lit tle, however, whether chalasia represents a small, unrecognized hiatal hernia or a neuromuscular failure of the esophagocardiac valve mechanism. 14 · 21 As others have re ported,9· 1 0 · 1 9 we have seen definite peptic esophagitis in patients with chalasia in whom an hiatal hernia could not be seen. The treatment should be identical. Hiebert and Belsey16 have reported good results with an anti-reflux operation in patients suffering from esophageal reflux in whom no hiatal hernia could be demonstrated. Four patients are included in this review with gastroesophageal reflux without an anatomical hiatal hernia. One of these patients (Fig. 9) required surgical intervention and was there by cured where medical therapy had been
Volume 55
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Hiatal hernia and gastroesophageal reflux
51
January, 1968
Fig. 9. Radiographs of child with gastroesopha geal reflux (chalasia). A hiatal hernia could not be demonstrated on two upper gastrointestinal series. Marked and uncontrolled reflux was present in both series.
unsuccessful. We believe that the principle advanced by Hiebert and Belsey16 is appli cable to some infants with uncontrolled gastroesophageal reflux. The fact that two thirds of the babies with hiatal hernia were cured by medical measures, and the absence of satisfactory guidelines in predicting which patients will respond to conservative therapy, dictates a need for vigorous trial of medical manage ment in all infants with hiatal hernia. The exception is the patient who already has developed an esophageal stricture for whom prompt surgery is mandatory. The symp tomatic response to medical therapy is often prompt and gratifying. Results are unmis takable after 3 weeks of intensive medical therapy. As long as there is definite evi dence of improvement of even a modest degree, the medical regimen should be con
tinued. However, if vomiting continues and there is no weight gain after 3 weeks have elapsed, patients are considered surgical candidates. With this potentially devastating disease, further time should not be squan dered in useless experimentation with antispasmodics and formula changes while the infant languishes. The results of surgical re pair of the hernia are pleasing and should not be denied when the above criteria have been met. Because of the logical concept behind the Allison repair1 and the simplicity of its ac complishment, this surgical approach was used in all patients herein reported. There was no mortality, and complications were notably minimal. A difficult problem has been the selection of those patients requiring pyloroplasty in addition to herniorrhaphy. Those patients with partial pyloric outlet obstruction, in dicated by delayed gastric emptying, should have pyloroplasty at the time of hiatal her nia repair. 12 ' 15 Since there are no absolutes for normal emptying time, delayed gastric emptying remains a subjective impression of the radiologist. Nevertheless, at this time, the finding of delayed gastric emptying on preoperative gastrointestinal series is the most valuable clue to the need for cocomitant pyloroplasty. Pyloroplasty adds to the morbidity of the surgical procedure. Since only 4 patients of the 19 requiring surgery also needed pyloroplasty, we do not advocate routine concomitant pyloroplasty with hiatal hernia repair. Improved radiographie equipment has en hanced the diagnosis of hiatal hernia, par ticularly in babies. It should be emphasized, however, that a radiologist familiar with xray diagnosis in children is indispensable in delineating the small and inconstantly seen hiatal hernias. Of even greater importance is the need for awareness by the pediatri cian of the role of hiatal hernia in many in fants and children who eat poorly and fail to grow. At our hospital, the diagnosis of hiatal hernia was established in only 7 chil dren between 1957 and 1963. Beginning in 1963, when increasing attention was focused
Journal of
52
Lilly and Randolph
Thoracic and Cardiovascular Surgery
on this problem, hiatal hernia was found in 45 infants and children in a 3-year span. This does not reflect an increase in the incidence of this disease but represents the result of improved recognition on the part of pédiatrie practitioners. Conclusions 1. Hernia of the esophageal hiatus is a surprisingly common gastrointestinal ab normality in pédiatrie patients, which differs significantly in symptomatology and therapy from the disease as seen in the adult popu lation. 2. The four main symptoms of hiatal hernia in infants are: (1) vomiting, (2) failure to thrive, (3) respiratory complica tions, and (4) anemia. 3. Diagnosis requires a precise esophagram performed by a radiologist experi enced in work with children. 4. Gastric hyperacidity has not been found in infants with congenital esophageal hiatal hernia. 5. Medical therapy has been successful in almost two thirds on this group of 52 infants and children with hiatal hernia and cardioesophageal reflux. 6. Surgical correction is effective and should be carried out on those patients who are not improved by medical means or who already have an esophageal stricture when seen. 7. Pyloroplasty may be necessary in some babies who undergo surgical correction of esophageal hiatal hernia, but is not recom mended as a routine complimentary proce dure. We would like to express our appreciation to Dr. John Washington, Dr. Albert Modlin, and Dr. Morris Feitel for allowing us to extend sur gical care to their patients described in the text. In addition, we are grateful to Dr. Gordon Avery, Director of Newborn Services, for his help in the conservative management of a number of the infants. REFERENCES 1 Allison, P. R.: Reflux Esophagitis, Sliding Hiatal Hernia, and the Anatomy of Repair, Surg., Gynec. & Obst. 92: 419, 1951.
2 Allison, R.: The Diaphragm in Gibbon, John, H., Jr., editor, Surgery of the Chest, Philadel phia, 1962, W. B. Saunders Co., p. 254. 3 Avery, G. B., Randolph, J. G., and Weaver, T. H.: Gastric Acidity in the First Day of Life, Pediatrics 37: 1005, 1966. 4 Avery, G. B., Randolph, J. G., and Weaver, T. H.: Gastric Response to Specific Disease in Infants, Pediatrics 38: 874, 1966. 5 Barrett, N . R.: Chronic Peptic Ulcer of the Oesophagus and Oesophagitis, Brit. J. Surg. 38: 175, 1950. 6 Barrett, N . R.: Hiatus Hernia, Brit. J. Surg. 42: 231, 1954. 7 Belsey, R.: Surgery of the Diaphragm in Brown, James, J. M., editor, Surgery of Child ren, Baltimore, 1963, Williams & Wilkins Co., p. 762. 8 Berenberg, W., and Neuhauser, E. D.: Car dioesophageal Relax (Chalasia) As a Cause of Vomiting in Infants, Pediatrics 5: 414, 1950. 9 Bettex, M., and Stillhart, H.: Operation for Hiatus Hernia and Cardioesophageal Chalasia by Fundoplication After Nissen, Surgery 55: 451, 1964. 10 Blank, L., and Pew, W. L.: Cardioesophageal Relaxation (Chalasia) Studies on the Normal Infant, A m . J. Roentgenol. 65: 40, 1956. 11 Botha, G. S. M.: The Gastro-oesophageal Re gion in Infants, Arch. Dis. Child. 33: 78, 1958. 12 Brown, F . B., et al.: Medical and Surgical Management of Esophageal Hiatus Hernia in Children, Bol. Med. Hosp. Infant. (Mex.) 41: 17, 1963. 13 Filler, R. E., Randolph, J. G., and Gross, R. E.: Esophageal Hiatus Hernia in Infants and Children, J. THORACIC & CARDIOVAS. SURG.
47:
551, 1964. 14 Forshall, I.: The Cardio-esophageal Syndrome in Childhood, Arch. Dis. Child. 30: 46, 1955. 15 Gross, R. E.: Thoracic Surgery for Infants, J. THORACIC & CARDIOVAS. SURG. 48:
162,
1964.
16 Hiebert, C. A., and Belsey, R.: Incompetency of the Gastric Cardia Without Radiological Evidence of Hiatus Hernia, J. THORACIC & CARDIOVAS. SURG. 42:
352,
1961.
17 Johnston, J. H.: Hiatal Hernia in Childhood, Arch. Dis. Childh. 35: 61, 1960. 18 Listerud, M. B.: Details of Interest and Con troversy in the Anatomy of the Esophageal Hiatus and Hiatal Hernia, Surg. Clin. North America 44: 1211, 1964. 19 Ravitch, M.: Chalasia, Pédiatrie Surgery, Chicago, 1962, Yearbook Medical Publishers, Inc., vol. I, p. 299. 20 Roviralta, E.: Less vomissements du nourrison, Paris, 1952, Flamarion. 21 Strawczynski, H.: The Behavior of the Lower
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Hiatal hernia and gastroesophageal reflux
53
January, 1968
Esophageal Sphincter in Infants and Its Rela tionship to Gastro-esophageal Régurgitation, J. Pediat. 64: 17, 1964. 22 Waterson, D.: Hiatus Hernia, Pédiatrie Sur gery, Chicago, 1962, Yearbook Medical Pub lishers, Inc., vol. I, p. 301. 23 Wyllie, W. G., and Field, E. C : The Etiology of Intermittent Oesophageal Régurgitation and Haematemesis in Infants, Arch. Dis. Child. 21: 218, 1946.
Discussion DR. CLEMENT A. HIEBERT Portland, Me.
Dr. Randolph has just presented in his cus tomarily clear and concise manner the clinical picture of the child with a valveless cardia. Quite properly, he emphasized that the infant, like the adult, may get into serious trouble with gastro esophageal reflux even when no hernia can be demonstrated. Chronic vomiting is the classic hallmark of this entity and, as most normal children vomit from time to time, the problem is to distinguish Dr. Randolph's baby roasting his esophagus with corrosive ferments from Shakespeare's infant harmlessly "mewling and puking in his mother's arms." Which vomiters will improve and which will not? Another problem is to persuade pediatricians to allow surgery before the irreversible damage to esophagus, lungs, or growth pattern has oc curred. Infatuation with a "normal" upper GI series has resulted in many a child having spinal taps, electrolyte studies, and abdominal films galore before vomiting of a nonobstructive nature is even thought of. The surgeon himself may be to blame. [Slide] This slide depicts an infant operated upon in the mistaken belief he had pyloric stenosis. A Ramstedt operation was none theless done but the child continued to vomit and became metabolically depleted. Here he lies, hun grily sucking his fingers moments after vomiting. The picture is the counterpart of the nausea-free vomiting in the adult with an incompetent and patulous cardia. I esophagoscoped this youngster and he had raging esophagitis; but suitable repair of the car dia stopped the vomiting and the esophagus looked normal by esophagoscopy a week later. Pyloroplasty alone is not the answer. In contrast with the authors I believe that the adult hiatal hernia frequently is progression of the infantile condition. Granted, there may be a long symptom-free interval, but if you quiz pa tients carefully, after a successful operation, many will acknowledge no previous appreciation ever of what it was to swallow and digest normally. Others will recall parents having said they were
terrible feeding problems in infancy. A celebrated patient of ours had vomited every day of his life for as long as he could remember. The only way he survived was to ruminate. As a child he earned understandable notoriety, when he would stand on a bridge above the train tracks and, at the command of his associates, eject the contents of his stomach into the stack of any designated pas sing train! An operation on his cardia when he was 35 years of age cleared matters up, trains included. I congratulate the authors. Actually, there is no real need to praise them, for their sterling presentation speaks for itself. I predict this paper will find its way into the classic literature on the subject. DR. I O H N J.
CINCOTTI
Sepulveda, Calif.
I thoroughly enjoyed Dr. Randolph's excellent paper, and Dr. Hiebert's delightful discussion. I would like to comment on the Allison repair, as used by the authors. Mr. Allison was impressed with my 1961 modification of the crural aspect of his repair and agreed that it might be uni versally used. In the late 1940's, Dr. Harken and I were pleased to be guided through an Allison proce dure by Mr. Philip Allison. My review of 111 cases in 1960 revealed a 10 per cent recurrence rate. In 27 of this group, I used an earlier modi fication, that is, compressed Ivalon (later, Teflon mesh) stents for crural buttressing, with the ex pectation of subsequent fiber ingrowth forming an aponeurosis-like reinforcement of the crural hi atus. However, the recurrence rate was not af fected by this change. In 1961 I began a series in which the original Allison repair was modified by including firm bites of the posterior gastric aspect of the esophagogastric junction, within the most caudally placed, yet untied, crural limb approximating sutures, and/or approximating the origins of the crura to the posterior gastric junction. Posterior junctional fixation sutures are more easily placed using the thoracic approach to the hiatus. We use identifying clips for the serial study of the di ameter of the reconstructed hiatus and the fixed junction. The Samuels' hemostatic clip (Week) is ideal for this purpose for, with slight closing pressure, only the tips of the clip approximate firmly. No tissue is crushed, while the permanently fixed clips permit easy anatomical identification. [Slide] Operative technique. [Slide] Clip rela tionship. I believe that, with this newer modification, hiatal widening will occur to roughly the same extent that the unmodified repair permits recur rences. If the posterier junctional anchoring su tures hold, however, recurrences are unlikely.
Journal of
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Lilly and Randolph
In closing, our pédiatrie and medical colleagues have been reluctant to advise early repair of symptomatic hiatal hernias because of the un acceptable recurrence rate. If we can offer a pro cedure that has a low rate of failure, we may see a significant decline in the incidence of pa tients with the damaged or destroyed sphincter or, what is all too often, an iatrogenously supervised esophageal stricture. D R . O T T O C. Baltimore,
BRANTIGAN
Md.
I rise to comment on an important aspect of this problem. I am certain the essayists are well aware of it. I would like to point out that gastroesophageal reflux is not necessarily esophageal hiatal diaphragmatic hernia, nor is esophagitis necessarily esophageal hiatal hernia. One can have gastroesophageal reflux without hernia, and one can have marked esophagitis without hiatal hernia. If treatment is to be rational and simplified, there
Thoracic and Cardiovascular Surgery
must be made a careful distinction between esophageal hiatal diaphragmatic hernia and esophagitis since the conditions may occur to gether or independently of each other. DR. R A N D O L P H (Closing) I would like to thank Dr. Brantigan for em phasizing that reflux may be troublesome without definite evidence of a hiatal hernia. We appreci ate Dr. Cincotti's comments on improvements in technique. I wish to thank Dr. Hiebert for his remarks. The question about which of the many infants who vomit should be studied for possible hiatal hernia is very much to the point. I would say that any baby who vomits to any degree, and who also shows any of the other three major symptoms, namely, failure to thrive, or pneumonitis, or anemia should be investigated. In closing, I must concede that we have had no child among our patients who could vomit down a train's smokestack.