The Differential Diagnosis of Alimentary Tract Obstructions in the Newborn J. NORTON NICHOLS,
M.D.,
BSTRUCTIONS
CAUSES
OF
OBSTRUCTION
Atresia is a deveIopmenta1 defect, intrinsic in type, and by definition impIies compIete imperforation. It may occur in any of the body’s hoIIow tubes, and as appIied to the alimentary tract it may be found at any IeveI. It is the only type of obstruction found above the duodenum, or beIow the sigmoid. For completeness, it may: be added that stenosis, in the form of a tiny opening through an otherwise imperforate diaphragm, differs from atresia onIy in degree; it is less common, and for practica1 purposes is considered under the same heading. American
Journal
of Surgery,
Volume
90. August,
,935
California
Mid-gut voIvuIus consists of a voIvuIus c entire smaI1 intestine. The afferent loop ;I twist is the first part of the jejunum, whiI# exit loop is some part of the colon. It is I possibIe through non-rotation of the embr: mid-gut, this in turn producing Iack of post mesenteric fixation. The symptoms are the high jejunat obstruction, and this is the IeveI at which this entity occurs. Diaphragmatic hernia may incIude ant struct any portion of the alimentary below the diaphragm. This condition, u the term “upside down stomach,” received publicity in the past. Within the first ten the symptoms are more IikeIy to be ca respiratory than intestina1. Intermittent ods of cohapse with dyspnea, cyanosis extremeIy rapid respirations are sugge: symptoms. The defect is usuaIIy in the diaphragm and auscultation may be misIeac A roentgenogram of the chest is the most \ abIe diagnostic measure. In meconium ileus the obstruction is in louver iIeum and is caused by a colunr thickened, sticky meconium which is dessic and contracted in its lowest portion. 1 caused by pancreatic insufficiency ant earIy evidence of cystic fibrosis of the panel The infant may be born with a promi abdomen which becomes more distended a is swaIIowed. The first meconium is absent the recta1 sphincter is smaI1 and tight. X findings wiI1 be discussed later. NeuroIogic obstruction due to myeni plexus deficiency, or earIy Hirschsprung’s ease, occurs onIy in the coIon-usuaIIy Ieft. Distention and vomiting may be ~107 deveIoping. The First meconium may brownish and thin, rather than sticky and ta black. Recta1 examination usuaIIy shows a f resistant and tubular peIvic sigrnoid. A si roentgenogram, following the administratio a barium enema under low pressure, furni. the necessary information. It wiII demonst a somewhat enIarged Ieft coIon above a I
of the alimentary tract furnish more than 80 per cent of the grave surgicaI emergencies seen in the newborn. So deadIy have they been in the past that in I932 but nine survivors of atresia of the intestine couId be counted in all the literature. The Hurstsuccessfu1 repair of an atresia of the esophagus was accompIished in 194 I. TotaI mid-gut volvulus was poorly understood and the distorted anatomy too often became cIear onIy at examination; whde . meconium postmortem iIeus and neurogenic obstruction were, and stiI1 are, attended by an extremeIy high death rate. Today al1 of the nation’s Iarger centers report increasing numbers of survivors, due Iargely to earIier diagnosis, prompt surgical intervention and improved pediatric care. This paper is concerned with early recognition; and whiIe the aII-inclusive diagnosis of “obstruction” is of first importance, a more concise differentiation offers many advantages in pIanning, preparation and surgica1 approach. Such a differentiation can usually be made quite accurately. The causes of obstruction are reIativeIy few, and several of these are seen onIy at a single, but definite, location. Conversely, there are certain anatomic IeveIs at which but one type of lesion can occur. These two reIated statements \viII be discussed separately.
0
Los Angeles,
262
DifferentiaI
Diagnosis
of Alimentary
tivcly smaller low-er segment. This distention does not have to be great to be significant in the newborn, for there is no other type ofcongenital obstruction which produces an enlarged, or even a normal, left colon. Imperforate anus is 01 course an exception, but in these a barium rnema is obviously impossible. These five subheads will account for the causative factor in nearly all alimentary tract obstructions within the first ten days of life. Returning now to the various anatomic levels, consideration will be given to the symptoms from obstruction at each. ANATOMIC
LOCATION
OF OBSTRUCTION
The highest point is the nasopharyngea1 and the obstruction is a choanal airway, atresia. It is not within the realm of the average general surgeon but is mentioned because it might well be called the roof of the alimentary tract since digestive mucous membrane is found on its lower, or posterior surface. It is a partition, either bony, fibrous or membranous, across the nasopharynx and btocks the nasal air\vay. The normal newborn is instinctively a nose breather and obstruction produces serious distress. He pulls for air against closed lips; he cannot ciose his mouth during sleep, or over a nipple. The one thing he can, and does do, is cry; this aerates him, but he cannot rest. The diagnosis is usualI? made with a probe, follo\ved b_v the instllIation of a few drops of opaque orl, and a roentgenogram. Treatment in our hospital is carried out by a nose and throat specialist. Seventeen patients have been seen and treated, and it is interesting indeed to note that one of these was born with both choanal atresia and an imperforatc anus. This is really going to extremes. The next lower Ieve is the upper third of the esophagus-the site of atresia of the esophagus, with or without trachea-esophagea1 fistula. No diagnosis is more easily made. The symptoms arc a wet mouth, frothy mucus, and dyspnea and coughing on any attempt to swallow. A catheter passed down the throat meets an obstruction at about I 2 cm., and the instilIation of I to z cc. of opaque oil folIowed by a single x-ray film in the upright position makes the diagnosis certain. In cases of fIstuIa, the roentgenogram will also show a proportionateI) larger amount of air in the stomach than in the intestines. In cases of atresia without fistula, the entire abdomen is opaque. Continuing downward the next Ievel is the 263
Tract
Obstructions
duodenum, tither above or below the ampulla. Onl,v atresia can be the causatil-c factor here, and vomiting is the one earl,v symptom. This is an important anatomic location, for bile furnishes the color for the lirst meconium. If diverted uplvard by an obstruction below the ampulla, the vomitus is green and the stools are colorless. The reverse is true \vhen the point of obstruction is abo\-e the ampulla. The x-ray flat film, in either instance, sho\vs a dilated stomach and no air in the intestine and duodenum, below. A few inches further down, at the first portion of the jejunum, either of two causatil-e factors may produce obstruction, namely, atresia of the jejunum or mid-gut voIvulus. They are easily differentiated. Both produce green \.omitus, never brown. Atresia allo\vs only grayish mucus per rectum, while in volvulus some mcconium has usually accumulated fleforc the twist occurs. The great aid here is again the roentgenogram. In atresia, dilatation down to the obstruction is seen, then no other air; in volvulus, some IittIe air gets through the twist and enters the closed loop, appearing as small arc-Iike shadows without haustrations. In any case of malrotation a barium enema will usual]? show the cecum in the right upper abdominal quadrant, or otherwise displaced from its usual location. A notable exception to the usual rule regarding green emesis may be mentioned here. Certain cerebral birth injuries, either of>stetric or spontaneous, may produce a similar picture for the first few- days, and be characterizecl only fly lassitude and bright green ~omitus. They are readily differentiated from atresia but are more diffIcuIt to separate from malrotation with volvulus. As a rule cases of cerebral injury show hut little distention of the stomach and duodenum on the plain roentgenogram, while gas in the intestine and coIon, although scanty, tends to be normaIIy distributed. The remainder of the jejunum and upper iIcum are obstructed only by atresia. Vomiting, grayish mucous stools, and x-ray evidence of distended Ioops dohvn to the obstructed point lead to the diagnosis. In the low ileum the problem may be difficult. Atrcsia and mcconium ilcus produce quite similar clinical pictures. Vomiting, distention and absence of mcconium are common to each. Barium enema shows a tiny left colon in both instances. On palpation, however, a dough) feel in the louver abdomen may bc elicited in
Nichols The infant born with a distended abdomen which is ffat to percussion may within a few hours deveIop further distention, IocaI tympany overlying the flatness and a tense shiny skin. In such cases a diagnosis of prenatal obstruction with perforation, and a superimposed pneumoperitoneum, will seldom be wrong. The infant with slow progressive abdominal distention, foIIowed by vomiting and inadequate stools, and on whom a colon roentgenogram shows an enIargement of the Ieft colon, has early Hirschsprung’s disease. The baby with imperforate anus has a ready made diagnosis for those who care to look. In males, if the imperforation is we11 above the skin level, a fistuIous communication with the urinary tract, usually the posterior urethra, will co-exist more than 70 per cent of the time. Meconium in the urine makes this diagnosis certain; in any event it wiI1 be found and corrected during the abdominoperineal procedure. From the perineal approach it is often missed. The size of the recta1 sphincter is of importance, provided it has not been distorted by the first rude examining finger. It is abvays small and undeveIoped in cases in which a congenital obstruction exists above it. Vomiting is the most important symptom of a11 obstructions below the diaphragm; but its greatest value in differentiation depends upon whether it is clear, green or brown. X-rav evidence, while our most valuable and concIu&e diagnostic aid, can often be anticipated and at times cIarified by bedside observation and deduction. The application of these principles has been of proved vaIue at the Childrens Hospital of Los AngeIes, where the records for the past eighteen years show that 454 patients with alimentary tract obstruction have been operated upon, with an over-all survival rate of 53 per cent. In conclusion, this wish for the unborn babies of the future is expressed; that some day, in every well equipped hospital, a plain roentgenogram of each baby wiI1 be taken at the end of twenty-four hours, and become a routine part of the chart. A portabIe x-ray unit connected with the nursery could make this possibIe with very IittIe inconvenience. Obstetric fractures, skeletal abnormalities, atelectasis, transposition of viscera, diaphragmatic hernia, intestina1 and esophagea1 atresias are just some of the abnormalities which could be diagnosed days in advance of the symptoms.
meconium ileus, and the packed Ioops may even be palpated. The x-ray film shows many distended co& in both; but in meconium ileus the meconium itself may occasionally appear as a hneIy granular shadow and small scattered air shadows, much smaher than the distended loops, may be seen. These are fairly diagnostic if found, and probabIy represent air bubbtes which have been forced downward into the sticky mass without compIetely IiIIing the lumen. Atresia of the colon is extremely rare but gives a dramatic x-ray picture when it occurs. The colon distends immensely with air, sometimes filling half of the abdomen, and may be confused with pneumoperitoneum. This picture, combined with tota absence of meconium, makes the diagnosis cIearIy apparent. The left coIon is the Iocation for an interesting diagnostic problem which is overIooked more often than it should be, nameIy, the neurogenie obstruction of early Hirschsprung’s disease. It is due to myenteric plexus deficiency, an d microscopically it is evidenced by absence of ganglionic celIs between the muscle layers. Its characteristic symptoms were outlined previously but it may again be stated that, provided the rectum is patent and in continuity with the gut above, there is no other congenita1 obstruction in early infancy which shows an enlarged left colon to barium enema and roentgenogram. SUMMARY
AND
CONCLUSIONS
In view of the foregoing statements it becomes apparent that certain physica findings are of such diagnostic inference that a correct impression may often be reached at the bedside. In summary they may be stated thusly. The baby who cannot breathe with cIosed lips probabIy has a choanal atresia. The baby with a wet mouth and frothy mucus, who cannot swahow without coughing or becoming cyanosed, probabIy has atresia of the esophagus. The child born with a smaI1 scaphoid abdomen which remains hat to percussion after six hours can only have atresia of the esophagus without fistula. The baby with attacks of dyspnea and an exceedingIy high respiratory rate may we11 have a diaphragmatic hernia. The infant who persistentIy vomits green materia1 probabIy has an atresia just beyond the ampuha, or a mid-gut voIvuIus. 264
Differential
Diagnosis
of Alimentary
‘I‘oda~ no patient is surrounded with more c-uqui&te care than is the newborn, and to give him this extra protection would seem only his just due. Up to now, however, few if any hospitals have done so. DISCUSSION
OK\ ILI.E F. GRIMES (San Francisco, Calif.) : It is true that of all the surgical emergencies .~ occurring in the newborn, congenital malformations of the gastrointestina1 tract are by far the most common. Of these, the trachea-esophageal fistula, the imocrforate anus and the meconium ileus are the main offenders. When such alimentary tract malformations occur, about 25 per cent of these individuals will have serious abnormalities elsewhere. such as congenital heart disease. If t’hc defect is‘an imperforate anus, the perccntage of additional malformations will be even higher since there may very well be associated genitourinary abnormalities. Before the detinitive repair of an imperforate anus is attempted, an evaluation of the genitourinary system might well be made. Whenever reasonable doubt exists regarding the advisability of an immcdiatc definitive rcpai r of an imperforate anus, a simple decomprcssing procedure should be accomplished. The differential diagnosis of alimentarv tract obstruction in the newborn is of great importance since early diagnosis and prompt surgical intervention may prevent rupture of the proximal bowel which mav occur earlvi in the course of the obstruction. The distention proximal to the obstruction leads to ischemic necrosis of the bowel wall and subsequent perforation. Peritonitis in this situation is invariably fatal. The practica1 factors which Dr. Nichols mentions arc of great vaIue in the diagnosis of these conditions. IIowcver. althoueh the necessitv for surgery is evident by the evaIuation of physical and x-ray findings, an accurate diagnosis often cannot be established until surgery is accompIished. B. P. MULLEN (Seattle. Washington): I would like to emphasize the anomalies of malrotation of the mid-gut Ioop producing obstruction. A brief discussion of the embryology will help bring to mind the develooment of these anomalies. As the mid-gut loop glows it is extruded through the umbilica1 ring into the umbiIica1 cord due to the fact that there is not enough space in the cavity to contain it as the liver is very Iarge at this time. It continues to grow as a physiologic umbiIica1 hernia until the abdominal cavity has enlarged to the point where it can contain a11 of the loop. The small intestine during this stage elongates and the cecum becomes thickened and the enlarged cecum hangs up in the sac until the last. The small intestine is reduced, first producing normal rotation of the mid-gut loop which is in a counter-clockwise
certainly
CI
i
d
265
Tract
Obstructions
direction. IIowcver, when t tic umbilical ring is quite large, sometimes the entire mid-gut loop reduces simultaneously and instead of a counterclockwise rotation a clockwise rotation results. Two types of obstruction may occur as a result. First, the entire mid-gut loop suspended frotn the narrow duodenal coIic isthmus may become completely rotated one or more times producing a volvuIus. None of the normal lixations of the intcstine occur in this condition. Strangulation may occur or it may not actually strangulate, and the patient may survive with this condition until aduIt life. The other anomaly which I am most interested in is known as rctroposition of the transverse colon. In this condition there is also a clockwise rotation but only of ninety degrees. The cccum and the transverse colon rotate behind the duodenum. The eecum may become wholIy or partly attached in its normal position but the transverse colon becomes located behind the duodenum. .4s the root of the small intestine becomes adherent to the posterior abdominat wall in an oblique manner from the origin of the superior mescntcry vessels to the cecum, it forms a tunnel about the transverse colon. The remainder of the large bowel attachments are normal. The obstruction is produced by a narrowing of this canal or tunne1, or by a \;olvuIus of the cecum. 1 have two such cases I intend to report which were found incidentally at the time of a gastric resection. This condition is extremely rare. To date I have found only four-teen eases reported in the litcratur-c and these two cases will be the liftcenth and sixteenth. To the differential diagnosis of the closed Ioop obstruction as a result of a competent iliocecal valve plus complete obstruction distally due to a tumor or voIvuIus must be added the anomaly of rctroposition of the transverse colon. Neither of my t\vo cases had obstruction. ,I believe that a resection of the right colon with anastomosis of the iIeum to the transv-ersc colon end-to-end beyond the tunnel and removing the cecum and extr-acting the part within the tunnel would be the procedure of choice. However, in cases of volvulus in which the attachment has not occurred, the procedure wouId be to simply unwind the volvuIus with or without attaching the cecum in its normal position. This has been done successfully. J. NORTON NICI~OLS (closing): Dr. Grimes is, of course, correct in his statement that many of the abdomina1 conditions cannot be accurately differentiated until Iaparotomy is performed. A goodly number wil1 stilI remain in which the true state of affairs can be suspected, and the observer alerted to the possibilities and dangers. This in itself makes carefu1 observation worth while, for there is no preventable factor which increases mortality more than a delayed diagnosis. 1 wish to express my thanks to those who have taken part in the discussion.