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Gastrointestinal surgical emergencies in the newborn infant
FETAL
AND
M ED ICIN E
NEONATAL RichardE. Behrman, Editor
Gastrointestinal surgical emergencies in the newborn infant James L. Talbert, M.D., Alvin H. Felman, M.D., and Franklin L. DeBusk, M.D. GAINESVILLE, FLA.
R E C E N T A D V A N C E S in pediatric surgery are reflected in all aspects of surgical care of the newborn infant including preand postoperative support. Gastrointestinal emergencies in the neonate, as they are at all ages, are characterized by obstruction, perforation, or bleeding. There are, however, significant differences in the etiology, diagnosis, and treatment of these conditions in the newborn infant. INTESTINAL OBSTRUCTION Diagnosis. Intestinal obstruction is the most frequent gastrointestinal emergency which requires surgery in the neonatal period. Early recognition is essential for success; the optimum time for sm'gicat intervention is usually within the first day of life, and a significant delay may increase the possibility of fatal outcome. Five major signs suggest the possible existence of intestinal obstruction: maternal hydramnios, excessive gastric aspirate, bilious vomitus, abdominal distension, and obstipation. M a t e r n a l hydramnios. I-Iydramnios is frequently associated with obstruction of the proximal gastrointestinal tract in the fetus. From the Departments of Surgery, Pediatrics, and Radiology of the University of Florida College of Medicine. Supported by United State Public Health Service Grant No. HD 03297 from the National Institute oJ Child Health and Human Development, Bethesda, Md.
Normally amniotic fluid is continually ingested and absorbed from the small intestine; some of this fluid is then transferred to the maternal circulation via the placenta and partially excreted in the urine of the mother. 1 Obstruction of the upper gastrointestinal tract interrupts this cycle and results in the accumulation of an excess of amniotie fluid. Hydramnios provides an early warning which, if correctly interpreted, may significantly decrease the delay of su@caI intervention after birth. Specific conditions which are associated with hydramnios include those producing obstruction of the esophagus, stomach, duodenum, or proximal small bowel. Obstruction of the distal small bowel or colon does not ordinarily produce hydramnios since an adequate surface for absorption remains in the proximal gastrointestinal tract. Excessive gastric aspirate. Gastric intubation should always be performed when there is maternal hydramnios; a gastric aspirate exceeding 15 to 20 c.c. of fluid, particularly if bilious, is suggestive evidence of intestinal obstruction. When there is obstruction of the upper intestinal tract, an abdominal roentgenogram obtained 4 to 6 hours after birth usually reveals distension of the intestine by accumulated gas above this obstruction. Bilious vomitus. Since obstruction proximal to the ampulla of Vater is rare (except Vol. 76, No. S, pp. 783-797
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Fig. 1. Small bowel atresia. The amount of air-filled small bowel may suggest the level of obstruction. A, duodenal atresia, B, jejunal atresia, C, ileal atresia.
by esophageal atresia), the color of the vomitus (clear or bilious) helps to distinguish the usual postprandial regurgitation of infancy from pathologic vomiting. Healthy newborn infants may spit up, but normal full-term infants do not vomit bile. In premature infants bilious vomiting may occasionally result from an immature pyloric sphincter. Abdominal distension. T h e newborn infant with intestinal obstruction or paralytic ileus develops abdominal distension owing to an accumulation of ingested air; this sign is not apparent at birth. The configuration of the distended abdomen may suggest the level of obstruction. Protuberance of the epigastrium indicates obstruction of the stomach or the duodenum, whereas generalized abdominal distension, usually apparent by 12 to 24 hours, suggests obstruction of the distal in-
testinal tract. The frequent association of abdominal distension (as a result of paralytic ileus) with peritonitis or sepsis necessitates the consideration of these entities in the differential diagnosis. Obstipation. Failure to expel meconium is a late sign of intestinal obstruction. A normal newborn infant may not have a meconium stool until 24 to 72 hours after birth. By this time other signs of intestinal obstruction are usually apparent. Occasionally small inspissated meconial stools are passed even in the presence of an obstructive lesion in the proximal gastrointestinal tract. Radiographic studies. Abdominal roentgenograms usually provide confirmatory evidence of intestinal obstruction. In congenital esophageal atresia a radiopaque catheter will not pass beyond the esophageal obstruction;
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air in the stomach indicates a distal tracheoesophageal fistula. The lungs of these infants are often overaerated and contain radiating a n d / o r confluent densities which reflect aspiration of oropharyngeal secretions and/or regurgitation of gastric contents. In gastric obstruction from a pyloric web, the stomach is distended and air is absent in the distal intestinal tract. In duodenal obstruction a characteristic "double bubble of accumulated gas" results from dual pockets of air distending the duodenum and stomach (Fig. 1, A). Complete midintestinal obstruction leads to distended, air-filled loops of jejunum and the absence of gas in the distal boweI (Fig:.. 1, B). Distal obstructions are more difficult to localize at this age because distended large and small bowel often cannot be distinguished on plain roentgenograms of the abdomen (Fig. 1, C). Diffuse distension of the bowel may be the result of a variety of obstructive lesions such as ileal or colonic atresia, aganglionic megacolon, meconium plug syndrome, intussusception, volvulus, and imperforate anus. In meconium ileus the roentgenogram may demonstrate minute bubbles of gas intermixed with meconium throughout the intestinal lumen, producing a granular "ground glass" appearance. 2 Peritoneal and scrotal calcifications and/or metaphyseal bands present at birth suggest intrauterine perforation and meconium peritonitisY, ~ An upper gastrointestinal radiographic study is needed to diagnose newborn intestinal obstruction only in those instances in which the obstruction appears incomplete or the exact site is in doubt. A barium enema may be useful in distinguishing the various types of lower bowel obstruction. Associated anomalies. The presence of intestinal obstruction in a mongoloid infant is presumptive evidence of duodenal atresia; 30 per cent of patients with duodenal atresia have mongolism. Atresias of the proximal gastrointestinal tract are often accompanied by premature birth. All forms of intestinal atresia are associated with an increased incidence of other congenital anomalies, particularly those involving the heart, spine,
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and urinary tract. Jejunal atresia may be associated with meconium ileus, intestinal malrotation, and volvulus. Malrotation of the intestine may also accompany omphaloeele, diaphragmatic hernia, or asplenia. The diagnosis of cystic fibrosis can usually be confirmed by analysis of sweat electrolytes in infants with meconium ileus; abnormalities of electrolyte a n d / o r protein content of hair, nails, and meconium have also been identified in the affected infants. Causes of intestinal obstruction. Preampullary obstruction. Repetitive nonbilious vomiting suggests obstruction proximal to the point of entrance of the common bile duct into the duodenum at the ampulla of Vater. ESOPr~AG~AL ATaESlA. Esophageal atresia is usually heralded by maternal hydramnios and after a few hours by respiratory distress in the newborn infant. With or without these signs the first feeding almost invariably produces severe choking and cyanosis owing to regurgitation and tracheal aspiration. In the rare instances of H-type tracheoesophageal fistula, the anomaly may escape detection for some days and present later with recurrent episodes of pneumonia and respiratory distress during feedings. T h e diagnosis of esophageal atresia is ordinarily confirmed by passing a No. 8 or 10 French radiopaque catheter through the nasopharynx into the proximal esophagus until obstruction is encountered approximately 10 cm. distal to the nares (at about the level of T2 vertebral body). A chest roentgenogram will then define the level of esophageal obstruction and indicate by the presence or absence of gas in the stomach whether there is an associated distal tracheoesophageal fistula. The rare association of a proximal tracheoesophageal fistula may also be excluded by injecting a small amount of contrast medium into the proximal esophageal pou@. Occasionally the diagnosis of esophageal atresia is overlooked when an excessively small or flexible feeding tube is inserted through the nasopharynx; the tube may coil in the posterior nasopharynx or rarely pass by way of the larynx, trachea, and tracheoesophageal fistu-
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la into the stomach, erroneously suggesting the presence of a patent esophagus. PYLORICl ATRESlA. Pyloric atr&ia is usually caused by a congenital web at the level of the antrum or pyloric sphincter. This condition is extremely rare and is difficult to identify, particularly v/hen it occurs in association with other forms of distal obstruction. A smal ! dimple of air may be produced by ballooning of the web into the distal duodenal lumen, simulating the double bubble sign considered pathognomonic for duodenal obstruction. At the time of surgical exploration a catheter should be passed from the stomach toward or into the duodenum to establish the level of obstruction. P Y L O R I C S T E N O S I S , Hypertrophic pyloric stenosis rarely is manifest in the first few days of life. Wallgren 5 reported no evidence of pyloric stenosis in a series of 1,000 upper gastrointestinal roentgenographic examinations of newborn infants. Statistically, 6 patients would have been expected to have had this diagnosis. Subsequently, 5 infants of the 1,000 did develop typical symptoms of pyloric stenosis, thus confirming the characteristic delay in the onset of symptoms of obstruction. Sporadic cases of hypertrophic pyloric stenosis in newborn infants have been reported, but this condition is usually characterized by progressive nonbilious vomiting in a Caucasian male infant between 2 weeks and 2 months of life. The vomiting often becomes projectile and is accompanied by weight loss and dehydration. If vomiting has been severe or prolonged, the serum electrolytes suggest a pattern of hypochloremic, hypokalemic alkalosis. Inspection of~the abdomen reveals a distended epigastrium with visible gastric peristaltic waves. In over 90 per cent of the patients a characteristic pyloric "olive" can be palpated just to the right of the midline in the epigastrium. DUODENAL ATRESIA. Vomiting is an early manifestation of this condition. Although the atretic segment is frequently located near the ampulla of Vater, bile often appears in the vomitus as the result of a proximal communication between the duodenum and the hepatobiliary system2 An abdominal roent-
The ]ournal o[ Pediatrics May 1970
genogram distinguishes this condition fl'om gastric obstruction (Fig. 1, A). Postampullary obstruction. These conditions comprise the overwhelming majority of cases of intestinal obstruction in the newborn infant. Bilious vomiting is the primary manifestation of obstruction of the small intestine. INTESTINAL ATRESIA. Approximately 25 per cent of atresias of the small bowel occur in the duodenum. Atresias of the colon or stomach are rare. Duodenal atresia is associated with mongolism. Stenosis is also more common in the duodenum than in other portions of the gastrointestinal tract. Occasionally stenosis escapes notice until adult life. It is rarely necessary t o perform extensive radiographic studies prior to surgical exploration of newborn infants with intestinal obstruction from atresia (Fig. 1). A barium enema will be helpful only in cases of distal ileal obstruction in order to exclude a lesion of the colon. The etiology of intestinal atresia is still under investigationfi Initially, the hypothesis of Tandler was widely accepted--that there was arrested intestinal development at a proliferative stage owing to a failure in the final process of recanalization. 7 Recent studies have revealed that obstruction of the lumen by epithelial growth occurs only in the duodenum of the fetus. Thus this theory does not explain the more frequent atresias of the distal small intestine. An alternative hypothesis of ischemic injury has been advanced by Louw, S and investigations have demonstrated that ligation of mesenteric blood vessels in fetal puppies will produce intestinal atresia at birth. Vascular injury of the gastrointestinal tract may be associated with volvulus, strangulation, and intussusception or with vasculitis from intrauterine infectious such as rubellaY ANNULAR PANCREAS. Annular pancreas is a rare condition caused by a fusion of the anterior and posterior analogues of the pancreas around the midportion of the duodenum. Severe stenosis or atresia of the duodenum may result. If obstruction is complete the diagnosis cannot be differentiated from
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Fig. :~ Malrotation and duodenal bands. "This infant developed bilious vomiting at 2 days of age. A, supine abdominal radiograph is normal, B, in the prone position the stomach and proximal duodenum are distended, C, barium enema shows the cecum to be in the right upper quadrant (arrow) confirming the diagnosis of malrotation. (Courtesy of Dr. John A. Kirkpatrick, Jr., St. Christopher's Hospital, Philadelphia, Pa.)
other causes of duodenal atresia. A side-toside duodenojejunostomy is the preferred operation when no other abnormalities are present; a bypass procedure with division of the constricting ring of pancreatic tissue is contraindicated. MALR.OTATION. Malrotation of the intestine is frequently associated with a large omphalocele or congenital diaphragmatic hernia; in these conditions intestinal migration may also be arrested, with segments remaining within the thoracic cavity or omphalocele sac. The diagnosis of malrotation of the intestine is suggested by the ectopic position of the cecum as demonstrated by barium enema (Fig. 2). Intestinal malrotation results from a failure of normal embryonic rotation of the cecum about the superior mesenteric vessels when the intestinal tract migrates back within the abdominal cavity. Instead of a 360 degree counterclockwise rotation whereby the cecum is positioned in the right lower quadrant, the process is ordinarily arrested when the cecum reaches the epigastrium. The mesentery of the small intestine remains suspended on a narrow pedicle which includes the superior mesenteric vessels. Lateral peritoneal bands connect the malptaced epigastric cecum to the right upper quadrant
abdominal wall and the liver. These attachments overlie the distal duodenum and may compress it, producing partial or complete obstruction of the lumen (Fig. 2). Since the small intestine is also suspended from the narrow mesenteric pedicle, the potential for obstruction from a volvulus is also present. This latter complication may occur either in utero or during early infancy/Early operative intervention is essential in cases of obstruction with midgut volvulus in order to avoid irreversible ischemic damage. The syndrome of asplenia and cyanotic heart disease may also be associated with realrotation. These cyanotic infants are distinguished by a right-sided heart, nucleated red blood cells (which may be misinterpreted as white blood cells by automatic counters, producing erroneously high white cell counts), bilaterally equal liver shadows on abdominal roentgenograms, and left axis deviation on the electrocardiogram. Any infant with this condition who develops intestinal obstrtt~tion should be immediately suspected of having a midgut volvulus? ~ MECONIU2r ILEUS. Approxmately 10 per cent of patients with fibrocystic disease of the pancreas develop intestinal obstruction from abnormally thick tenacious meconium. T h e
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diagnosis is suggested by the signs and symptoms of distal small bowel obstruction; perforation or volvulus of the intestine is a frequent complication. * The abdomen may have a characteristic doughy consistency owing to the presence of the abnormal meconium within the intestinal lumen. Perforation of the intestine results in meconium peritonitis and the development of intraabdominal and scrotal calcifications.
The Journal of Pediatrics May 1970
The instillation of acetylcysteine into the proximal gastrointestinal tract is a valuable adjunct in treating this condition; dissolution of the obstructing meconium may avoid surgical intervention. 11, 12 More frequently, a proximal temporary ileostomy is required for instillation of acetylcysteine or pancreatic enzymes into the distal bowel in order to remove the obstructing meconium. The high frequency of associated serious abdominal
Fig. 3. Meconium plug syndrome. A, diffuse intestinal distension is evident in this 2-day-old infant, B, barium study reveals a core of meconium in the rectosigmoid colon (arrow), C, on postevacuation study a segment of meeonium remains in the transverse colon (arrow).
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a n d / o r respiratory complications combine to produce an over-all poor prognosis in this condition. MECONIUM P L U G S Y N D R O M E . This syndrome is characterized by tenacious plugs of inspissated meconium which occlude the distal colon and rectum and produce temporary obstruction (Fig. 3). In most instances this entity is not associated with fibrocystic disease of the pancreas. Anorectal stenosis or atresia are rare disorders which can be distinguished from this syndrome b y digital examination of the rectum. Hirschsprung's disease can usually be excluded by a barium enema, although an occasional infant with the"'meconium plug syndrome sub,sequently ig found to have aganglionosis of the distal bowel. Digital examination of the rectum or a barium enema often results in the passage of the inspissated meconium plug with the relief of obstruction? ~ These patients should be followed, however, to exclude the possibility of a serious underlying abnormality. H I R S C I r l S P R U N G ' S D I S E A S E . Congenital megacolon results from an absence of ganglion cells in the parasympathetic myenteric nerve plexus of the colon and rectum. Rarely, this
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condition may involve the entire colon or even extend into the small intestine (Fig. 4) ; the absence of intestinal peristaltic activity is responsible for functional intestinal obstruction. In early infancy, constipation or severe diarrhea may occur. Hirschsprung's disease may also present as acute intestinal obstruction in the newborn infant. A severe, fulminant enteritis and overflow type of diarrhea can be secondary to such obstruction. Roentgenographic diagnosis may be difficult in early life, prior to the distension of the proximal intestine. Disproportionate distension of the large and small bowel suggests the usual variety of Hirschsprung's disease (Fig. 5). A competent ileocecal valve allows the colon to dilate; not uncommonly the sigmoid appears roentgenographically as a bowel loop or "mass" extending into the right upper quadrant. A careful barium enema with a straight-tipped catheter inserted just beyond the rectal sphincter usually confirms the diagnosis. The aganglionic segment appears contracted and the proximal intestine distended. Follow-up roentgenograms in 24 hours usually show significant retention of barium in the uninvolved proximal bowel. A definitive diagnosis of Hirschsprung's
Fig. 4. Malrotation and total aganglionic megacolon. This neonate presented with persistent bilious vomiting. A, moderate intestinal distension is evident, B, barium enema reveals the cecum (arrow) in the upper midabdomen. The entire colon is narrowed and the walls are irregular and serrated, C, marked retention of barium is evident on the 24 hour follow-up examination. No obstructing bands were found at the time of the operation.
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Fig. 5. Hirschsprung's disease. Two-day-old infant with vomiting and abdominal distension. A, there is diffuse gasseous distension of bowel. Note air-filled loop of bowel in right upper quadrant (arrows). B and C, barium enema confirms the diagnosis of Hirschsprung's disease with a low transitional segment (arrows). The right upper quadrant bowel seen in A is the redundant sigmoid colon. (Courtesy of Dr. John A. Kirkpatrick, Jr., St. Christopher's Hospital, Philadelphia, Pa.)
disease is established by the absence of ganglion cells in the myenteric and submucosal plexus of tissue obtained by a full-thickness rectal biopsy. Although so-called "skip-involvement" of the intestine has been reported, for practical purposes the aganglionosis usually extends continuously from distal involved to proximally uninvolved intestine. The treatment of choice is to establish colostomy at the point of transition from normal to aganglionic bowel. A definitive pull-through procedure is performed between 1 and 2 years of life, prior to the usual time of toilet training. 14 I M P E R F O R A T E A N U S . Physical examination at birth ahnost always establishes the di~tgnosis of imperforate anus. The anus may be normal in external appearance in cases of anorectal atresia or stenosis. Anomalies of the urinary tract and lumbosacral spine are frequently associated with imperforate anus and must be excluded in all patients by an intravenous pyelogram and roentgenograms of the spine. Cases of "low" rectal pouch are regularly associated with ectopic perineal openings or fistulas that extend, into the distal vagina. Cases of "high" rectal pouch usually have
an associated fistula into the urethra or bladder in males or to the upper vagina in female infants. Injection of contrast media into external fistulous tracts will usually define the level of the communicating pouch. Whenever an external fistula is absent, the urine of males or the v @ n a of females should be examined for meconium. Berdon and associates 15 have suggested that the classic upside-down abdominal roentgenogram may prove unreliable ifl predicting the length of the atretic segment of rectum (Fig. 6). Such studies may prove helpful, however, if they demonstrate passage of air into the bladder through a fistula. If none of these methods prove successful in demonstrating the level of the rectal pouch, urethrocystography may define a communication between the rectum and the bladder or urethra. The differentiation between a "high" and "low" rectal pouch has important surgical implications. If the pouch is high, a temporary colostomy is indicated and a definitive abdominoperineal pull-through procedure should be deferred; a primary perineal anoplasty is usually indicated for a "low" pouch. HERNIAS. If an infant has an incarcerated inguinal hernia at birth, reduction should
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Fig. 6. High imperforate anus. A, diffuse intestinal dilation with air-fluid levels is evident, B and, C, inverted studies reveal no air ira bladder. Following a colostomy with irrigation of the distal colon, meconium appeared in the urine.
not be attempted as is frequently the practice in older infants and children. There is a significant danger of strangulation and necrosis in these cases since the period of incarceration is uncertain. Immediate exploration a n d herniorrhaphy should be performed. MISCELLANEOUS. A variety of other conditions may produce intestinal obstruction. Intestinal bands may result from inflammatory adhesions or persistent vitelline duct remnants (i.e., Meckel's diverticulum). Intussusception is rare in the newborn infant and duplications of the intestinal tract usually produce partial intestinal obstruction. ~ INTESTINAL PERFORATION IN THE N E W B O R N
Intestinal perforation may occur prior to birth as a result of intestinal obstruction in the fetus, or it may develop following birth f r o m a variety of causes. Since the fetal intestinal contents are sterile and remain so for the first few days after birth, perforation during these periods produces a reactive, chemical peritonitis. If leakage occurs after the first few days of life, bacterial peritonitis ensuesJ 7 Antepartum perforation. Intrauterine perforation usually results from distal intestinal obstruction secondary to atresia, volvulus, strangulated hernia, or meconium ileus associated with cystic fibrosis of the pancreas.
Meconium is present in the distal intestinal tract to the level of the ileocecal valve by the fourth month of gestation and may be released into the peritoneal cavity when subsequent perforation occurs. The sterile meconium induces a severe chemical and foreign body reaction, an intense fibroplastic peritonitis. The peritoneal contents are enveloped by an adherent, fibrous membrane in which calcium is rapidly deposited. Since these events frequently antedate closure of the processus vaginalis, scrotal calcifications may also result. The point of perforation is usually sealed by the reactive process, but occasional persistent leakage may lead to the formation of a large fibrous cyst which can fill the entire abdominal cavity. If intraperitoneal leakage persists after the third postpartum day, bacterial contamination occurs. Intrauterine intestinal perforation and meconium peritonitis can be diagnosed prior to birth by maternal roentgenograms demonstrating typical calcifications within the fetal abdomen. Following birth such infants characteristically develop signs and symptoms of intestinal obstruction, either because of the original bowel lesion or as a result of the dense adhesive scar which encompasses the peritoneal contents. Intra-abdominal and scrotal calcifications a n d / o r metaphyseaI bands confirm the diagnosis, z' 3 Intraperi-
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toneal air appears within 48 t o 79 hours in those patients in whom intestinal leakage persists. Whenever intestinal "obstruction or persistent peritoneal leakage is suspected, immediate surgical exploration is required. In the absence of these indications, watchful waiting and SUldportive therapy are occasionally ~ustified? s Postpartum perforation. As in the case of the fetus, the most frequent cause of postpartum perforation is an underlying intestinal obstruction from-atresia, meconium ileus, volvulus, hernia, or Hirschsprung's disease. In ~ddition to these entities, however, several unique sources of neonatal gastrointestinal perforation should be recognized. Gastric per[oration. Neonatal gastric perforation usually occurs within the first week of life and often within the first 3 days. I9 Over 20 per cent of the patients are born prematurely. The diagnosis may be suggested initially by bilious vomiting and rapidly progressive abdominal distension. The entire abdomen, including the usual area of liver dullness, becomes tympanitic to percussion. The distension is frequently severe enough to produce secondary respiratory distress and circulatory embarrassment; signs and syrup-
Fig. 7. Gastric perforation. Three-day-old premature infant with vomiting and abdominal distension. An extensive pneumoperitoneum is evident; the faleiform ligament (arrow) is visualized. (Courtesy of Dr. Jbhn A. Kirkpatrick, Jr., St. Christopher's Hospital, Philadelphia, Pa.)
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toms of shock may ensue. Plain abdominal roentgenograms are frequently pathognomonic of gastrointestinal perforation (Fig.
7). Neonatal gastric perforation may occur owing to gastric distension resulting from distal intestinal obstruction, positive pressure ventilation during resuscitation at birth, trauma to the stomach wall as a result of nasogastric intubation, or birth injury. The relatively high gastric acidity of the first few weeks of life, particularly in association with prematurity, intracranial lesions, or other sources o f neonatal stress, may also predispose to acute peptic ulceration and perforation. Finally, "idiopathic" gastric perforation in which none of the preceding factors are present has been recognized as a distinct entity in the neonatal period. The stomach wall characteristically ruptures along the greater curvature. Histologic examination of biopsy specimens taken from the site of perforation have suggested that a congenital deficiency of gastric musculature may be a primary or contributory factor. Regardless of etiology, surgical intervention is imperative in aIi cases; occasionally immediate relief of massive abdominal distension by needle or tube paracentesis and treatment of shock a n d / o r respiratory distress may be required. The situation is comparable to that encountered in tension pneumothorax, with impairment of diaphragmatic excursion, compression of the lungs, and obstructed venous return to the heart. If necessary, abdominal decompression may be maintained during transport of the child to surgery by attaching the paracentesis tube to an underwater seal. At the time of surgery, along with exploring the surface of the stomach, the surgeon must explore the intestine for the possibility of an associated distal intestinal obstruction. The site of perforation should be debrided of all devitalized tissue and closed with a double layer of inverting sutures. A gastrostomy is a useful adjunct for maintaining gastric drainage during the immediate postoperative period and subsequently for initiating intestinal alimentation. Neonatal necrotizing enterocolitis. Perfora-
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tion of the colon or terminal ileum in the newborn infant is often associated with an idiopathic necrotizing enterocolitis. 2~ T h e syndrome commonly occurs in the premature infant within the first few days of life, although it may appear in the full-term newborn infant as late as 2 months of life. The typical patient is a premature infant who has had mild respiratory distress at birth but subsequently did well until the third to fifth day of life when he developed vomiting, progressive respiratory distress, and abdominal distension. Signs of sepsis and shock with hypothermia and poor peripheral perfusion develop rapidly. Diarrhea or passage of bloody mucus per~xectum may also appear. Abdominal roentgenograms usually demonstrate free peritoneal gas, pneumatosis intestinalis, or hepatic portal venous gas. Occasionally the roentgenogram may confirm the diagnosis on the basis of these findings before the clinical syndrome becomes evident. A milder form of the disease is characterized by gross rectal bleeding; in contrast, rectal bleeding plays a relatively insignificant role in the more fulininant disease which rapidly progresses to perforation. Therapy should include intestinal decompression, parenteral fluid therapy, and antibiotics. A surgeon should be consulted immediately and should follow the patient carefully with the pediatrician for any evidence of progression of the process. Intraperitoneal perforation requires immediate exploration. If an operation is required the involved bowel should be resected and either an endto-end anastomosis or a temporary diverting colostomy or ileostomy should be performed. A survival rate of 67 per cent has been reported when these principles of management were employed. The etiology of this unusual condition remains unclear but there is a frequent association with previous cannulation of the umbilicM artery or v e i n y Histologic sections of the involved segments of bowel often suggest ischemic changes. Iatrogenic intestinal perforation. Traumatic perforation of the intestinal tract by nasogastric intubation or anorectal insertion
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of enema tubes, thermometers, or diagnostic catheters presents a particular threat to the newborn infantY 2 The distance between the anus and the sigmoid colon within the peritoneal cavity may be less than 3 cm. at this age. Therefore, insertion of any firm object into the infant's rectum involves a significant risk. This complication can be prevented by using soft rubber catheters for rectal cannulation, limiting the insertion of rectal thermometers to a distance less than 2 cm., and the careful administration of any enema in the newborn infant. Neonatal appendicitis. Appendicitis is extremely difficult to diagnose in the newborn infant. The disease is rare at this age and the symptoms are relatively nonspecific? 7 The rarity of this condition in the infant is attributed to a relatively wide appendiceal lumen with a sparsity of intestinal lymphoid tissue. Appendiceal obstruction, the initiating factor in most cases of appendicitis, is therefore less likely to occur at this age than in older children or adults. An association of Hirschsprung's disease with perforation of the appendix has been documented, and rectal biopsy should be considered in all such cases in infants. (If the diagnosis of Hirschsprung's disease is confirmed by an absence of ganglion cells in the specimen, a diverting colostomy should be performed immediately.) Treatment of intestinal perforation. The treatment of any form of intestinal perforation in the newborn infant includes nasogastric decompression, restoration of fluid balance, correction of electrolyte and acid-base disturbances, and administration of antibiotics. Judicious haste is imperative; the goals are early restoration of homeostasis and surgical exploration within 3 to 4 hours. Immediate abdominal paracentesis may be required in some patients to relieve massive abdominal distension and associated cardiorespiratory embarrassment. In assessing fluid rr ments it should be noted that the total peritoneal surface is roughly equivalent to 75 per cent of the body surface area. Accordingly, fluid losses in a patient with peritoneal inflammation may approximate those encountered in a major body surface burn. The
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monitoring of serial determinations of central venous pressure, total serum prot_eins, hematocrit, and urinary output and specific gravity is particularly helpful in maintaining an optimum balance of fluid and electrolytes. GASTROINTESTINAL
BLEEDING
The high mortality rate from gastrointestinal hemorrhage in the newborn infant emphasizes a need for prompt diagnosis and treatment. The loss-of 60 ml. of blood in the full-term infant is comparable to a loss of 600 td 700 ml. in the adult. Seemingly small amounts of blood in the stool of an infant may represent a significant loss. Extrinsic sources of blood as well as intrinsic sites of bleeding must be considered in the differential diagnosis. Sources of fieonatal bleeding. Maternal sources--swallowed blood syndrome. Swallowed maternal blood usually appears in the stools on the second to third day of life. It may be swallowed by the infant during delivery or ingested during breast-feeding if there is a fissure of the nipple. The test of Apt and Downey 23 will distinguish between maternal and infant blood on the basis of the greater resistance to alkali denaturation of red blood cells containing fetal hemogiobin. Newborn bleeding disorders. Hemorrhagic disease of the newborn infant secondary to hypoprothrombinemia may cause a bleeding disorder at this age and may require treatment by administration of vitamin K and fresh blood or plasma. Less frequently, congenital or acquired deficiencies of coagulation factors or platelets may also cause bleeding in the newborn infant. Intestinal ischemia. Intestinal ischemia and subsequent gangrene can occur when the mesenteric blood supply of the bowel is compromised. Malrotation of the intestine with associated volvulus is an example of this complication; in the newborn infant the presence of a palpable abdominal mass, intestinal obstruction, and bloody stools usually indicates a volvulus rather th~n an intussusception. Other situations associated with intestinal
The Journal of Pediatrics May 1970
ischemia, such as incarcerated hernia and neonatal necrotizing enterocolitis, are infrequent in the newborn period. Stress ulcer. Stress ulcers of the stomach and duodenum may lead to rapid exsanguination of the small infant. 24 Gastric lavage with cold saline solution or topical thrombin has been advocated as treatment, but frequently early exploration for direct suture ligation of the bleeding vessel in the base of the ulcer is necessary. An upper gastrointestinal radiographic series may be helpful in demonstrating an acute stress ulcer of the stomach or duodenum, but the presence of blood in the stomach in association with rapid hemorrhage is usually adequate indication for immediate laparotomy. Extensive gastric surgery, such as resection or vagotomy and pyloroplasty, is not required. Gastric drainage through a large gastrotomy tube is a useful adjunct to ensure gastric decompression and can then be used for postoperative feeding. SPECIAL SURGICAL TECHNIQUES
The following surgical techniques may provide palliative relief until definitive surgery is indicated. Pediatricians should be aware of file technical details of some of these procedures if they participate in postoperative management. Gastrostomy. In patients with a tracheoesophageal fistula, a gastrostomy provides a means for decompressing the gastrointestinal tract and preventing regurgitation of acidic gastric contents into the tracheobronchial tree. This technique, in combination with aspiration of the proximal blind esophageal pouch, may facilitate a delay of definitive surgery for hours or even days and provide for the infant's condition to stabilize, reducing the risk of subsequent operation. The gastrostomy may be combined with closure of the tracheoesophageal fistula when longer periods of support are required. A number of excellent techniques have been developed by pediatric surgeons to perform gastrostomies under local anesthesia in the newborn infant. 2s, 26 After intra-abdominal neonatal surgery a
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gastrostomy provides postoperative gastrointestinal decompression and avoids the potential complications of prolonged nasogastric intubation, e.g., airway obstruction by intrusion of the tube into the nasopharynx, erosion of the mucosa of the stomach or esophagus, or inadequate decompression by a small nasogastric tube. A mushroom type of catheter may be preferable to the Foley balloon catheter for gastrostomies in newborn infants; the balloon at the Foley catheter tip may be too large relative to the size of the stomach and produce partial gastric or pyloric obstruction. The Foley balloon can also pass into the duodenum and produce complete obstruction. A ivariety of techniques have beer~ advocated f0 prevent this latter complication, including speciaI double balloon catheters, stabilization with spools, etc., but a simple, adequate method is to secure the catheter at the skin level with a bolus of gauze snugly wrapped about it. Various devices have also been proposed for administering gastrostomy tube feedings and achieving simultaneous decompression while the feeding is progressing (an automatic "burper). A reliable approach is to attach a large, glass, Y tube to the end of the gastrostomy tube. If this system is then elevated, the height of the fluid column within the tube represents the maximum pressure which can be exerted within the stomach itself. Feedings may be administered through one side arm of the Y tube while simultaneous decompression is achieved through the opposite arlTl.
Enterostomy. Another technique helpful in initiating early feedings in infants after a gastrointestinal operation is the insertion of an enterostomy tube. It is either threaded through the gastrostomy into the distal small bowel or inserted into it through a separate incision. These tubes must be made of a pliable material which retains its soft consistency when exposed to the digestive juices of the intestinal tract. Silicone rubber tubing with an internal diameter of a No. 18 needle has proved optimal for this purpose. Other plastic materials frequently become rigid when inserted into the gastrointestinal tract,
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a characteristic which invites subsequent erosion and/or perforation. The enterostomy tubes are connected to constant infusion pumps for administration of feedings since the diameter is too small for a free gravity flow of formula (even when special noncurd formula mixtures are employed). Gastric aspirate from the gastrostomy tube may also be replaced into the distal gastrointestinal tract with this system. Colostomy. If the specific requirements of the small infant are not recognized, the maintenance of colostomies at this age may be associated with a variety of surgical complications2S, 2s; with proper attention t o detail, however, the postoperative management of a colostomy should be almost as easy as caring for a normal infant. The pediatrician must guard against the development of constipation or of stenosis of the colostomy orifice; it may be necessary to dilate the colostomy stoma periodically a n d / o r to add stool softeners. Irritation of the skin around the colostomy orifice is caused by excessive moisture. This complication should be treated by applying powdered cornstarch to the skin. If maceration develops, karaya gum powder should be substituted for cornstarch. When the colostomy has been performed as a palliative step, final corrective surgery should be undertaken prior to the time of initiating toilet training. 9 Monitoring techniques. A variety of monitoring techniques are necessary to manage gastrointestinal surgical emergencies successfully in the newborn infant. 27 These supportive measures often provide the critical margin for success, but they do not substitute for frequent careful clinical re-evaluation and adequate nursing care. Those techniques which deserve special consideration include the following: Cardiovascular [unction. Electrocardiographic monitoring has been facilitated by the introduction of miniaturized skin electrodes; arterial pressures can be followed with indwelling catheters inserted into the umbilical, temporal (needle), brachial, radial, or femoral arteries. These same catheters provide a method for repeated sampling of
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arterial blood for the determination of Mood gas values. Umbilical artery catheters can be used for infusing fluids. Fluid balance. I n recent years the dangers of overloading the circulation of the newborn infant with excessive fluids during the postoperative period have become apparent. T h e dangers of underhydration with the potential o f hypovolemia and inadequate tissue perfusion, however, deserve equal attention. I n addition to continual clinical evaluations, a composite monitoring regimen is necessary to achieve the appropriate level of hydration; it should include recording of fluid intake and output with balances calculated at 6 hour i~tervals or more frequently if indicated; serial measurements of central venous pressure and frequent determinations of total serum osmolality and protein concentration, hematocrit, and urinary specific gravitY. T h e latter tests can be measured on small amounts ( < 0.1 ml.) of body fluids with a h a n d refractometer and microcentrifuge. 2s Serial determinations of the central venous pressure provide a relatively new and important technique to avoid overloading the circulation. A catheter is inserted into the superior vena eava or right atrium through a jugular vein. W h e n interpreted with regard to the other determinations, it provides a means for estimating cardiac filling pressure and relative intravascular expansion. It is particularly useful in postoperative infa.~ts who have sequestered significant quantities of fluids, electrolytes, and proteins within body tissues as a result of ileus, peritonitis, or t r a u m a (edema), in children with pulm o n a r y edema and heart failure, and in newborn infants with immature renal function who may have low urinary specific gravities in spite of significant dehydration. Long-term parenteral nutrition. Not infrequently the neonatal patient who has had corrective surgery for a gastrointestinal anomaly requires a relatively long time to regain enough gastrointestinal function to provide calories, fluids, and electrolytes adequate for nutrition and growth. Recently special parenteral solutions have been used to provide these patients with increased
The Journal o[ Pediatrics May 1970
amounts of calories and other essential substrates.2~, 30 These prepara~0ns are administered through central venous catheters composed of inert substances (special silicone rubber tubing) designed to minimize tissue reaction and clotting. Small volumes are continuously administered with infusion pumps in order to increase the opportunity for dilution and mixing of these hypertonic solutions with blood. REFERENCES
1. Lloyd, J. R., and Clatworthy, H. W.: Hydramnios as an aid to the early diagnosis of congenital obstruction of the alimentary tract: A study of the maternal and fetal factors, Pediatrics 21: 903, 1958. 2. Neuhauser, E. B. D.: Roentgen changes associated with pancreatic insufficiency in early life, Radiology 46: 319, 1946. 3. Wolfson, J. J., and Engel, R. R.: Anticipating meconium peritonitis from metaphyseal bands, Radiology 92: 1055, 1969. 4. Donnison, A. B., Schwachman, H., and Gross, R. E.: A review of 164 children with meconium ileus seen at the Children's Hospital Medical Center, Boston, Pediatrics 37: 833, 1966. 5. Wallgren, A.: Incidence of hypertrophic pyloric stenosis, Amer. J. Dis. Child. 62: 751, 1941. 6. Hays, D. M.: Intestinal atresia and stenosis, Curr. Probl. Surg. Oct., 1969. 7. Tandler, J.: Zur Entwicklungsgeschichte des menschlichen Duodenum in fruken Embryonalstudien, Morph. Jahrb. 29: 187, 1902. 8. Louw, J. H.: Congenital intestinal atresia and stenosis in the newborn. Observation on its pathogenesis and treatment, Ann. Roy. Coll. Surg. Eng. 25: 209, 1959. 9. Esterly, J. R., and Talbert, J. L.: Jejunal atresia in twins with presumed congenital rubella, Lancet 1: 1028, 1969. 10. Schiebler, G. L.: Personal communication. 11. Mecker, I. A., Jr., and Kincannon, W. N.: Acetylcysteine used to liquefy inspissated meconium causing intestinal obstruction in the newborn, Surgery 56: 419, 1964. 12. Simpson, T. E., Lynn, H. B., and Burgert, E. O., Jr.: Nonoperative treatment of meconium ileus: Report of a case, Mayo Clin. Prec. 43: 725, 1968. 13. Mikity, V. G., Hodgman, J. E., and Paciulli, J.: Meconium blockage syndrome, Radiology 88: 740, 1967. 14. Steichen, F. M., Talbert, J. L., and Ravitch, M. M.: Primary side-to-side colorectai anastomosis in the Duhamel operation for Hirschsprung's disease, Surgery 64: 475, 1968. 15. Berdon, W. E., Baker, D. H., Santulli, T. V., and Amoury, R.: The radiologie evaluation of imperforate anus: An approach correlated
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16. 17. 18. 19. 20.
21.
22.
23.
with current surgical concepts, Radiology 90" 466, 1968. Laird, A. P., and Kerr, M. M.: Antenatal jejunal intussusception, Z. Kinderchir. Suppl. zu Bd. 5: 76, 1968. Ravitch, M. M., and Rowe, M. I.: Surgical emergencies in the neonate, Amer. J. Obstet. Gynec. 103: 1034, 1969. Lange, M.: Meconium peritonitis presenting in scrotal hydroceles, Brit. J. Surg. 51: 942, 1964. Robarts, F. H.: Neonatal perforation of the stomach, Z. Kinderchir., Suppl. zu. Bd. 5: 62, 1968. Stevenson, J. K.; Graham, C. B., Oliver, T. K., and Goldenberg, V. E.: Neonatal necrotizing enterocolitis, Amer. J. Surg. 118: 260, 1969. Corkery, J. J., Dubowitz, V., Lister, J., and Moosa, A~: Colonic perforation after exchangeziitransfusion, Brit. Med. J. 4: 345, 1968. ': Greenbaum, E. I., Carson, M., Kincannon, W. N., and O'Laughlin, B. J.: Rectal thermometer-induced pneumoperitoneum in the newborn, Pediatrics 44: 539, 1969. Apt, L., and Downey, W. S., Jr.: "Melena" neonatorum: The swallowed blood syndrome. A simple test for the differentiation of adult
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27. 28.
29.
30.
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and fetal hemoglobin in bloody stools, J. PEDIAT. 47: 6, 1955. Spencer, R.: Gastrointestinal hemorrhage in infancy and childhood: 476 cases, Surgery 55: 718, 1964. Lewis, J. E.: Atlas of infant surgery, St. Louis, 1967, The C. V. Mosby Company. Mustard, W. T., Ravitch, M. M., Snyder, W. H., Jr., Welch, K. J., and Benson, C. D.: Pediatric surgery, Chicago, 1969, Year Book Medical Publishers, Inc. Talbert, J. L., and Haller, J. A., Jr.: Recent advances in the postoperative management of infants, J. Surg. Res. 6: 502, 1966. Thoene, J. G., Talbert, J. L., Subramanian, S., and Odell, G. B.: Use of the hand refractometer in determining total serum proteins of infants and children, J. PEDIAT. 71: 413, 1967. Wilmore, D. W., Groff, D. B., B{shop, H. C., and Dudrick, S. J.: Total parenteral nutrition in infants with catastrophic gastrointestinal anomalies, J. Pediat. Surg. 4: 181, 1969. Filler, R. M., Eraklis, A. J., Rubin, V. G., and Das, J. B.: Long-term total parenteral nutrition in infants, New Eng. J. Med. 281: 589, 1969.
AND
M ED ICIN E
NEONATAL RichardE. Behrman, Editor
Gastrointestinal surgical emergencies in the newborn infant James L. Talbert, M.D., Alvin H. Felman, M.D., and Franklin L. DeBusk, M.D. GAINESVILLE, FLA.
R E C E N T A D V A N C E S in pediatric surgery are reflected in all aspects of surgical care of the newborn infant including preand postoperative support. Gastrointestinal emergencies in the neonate, as they are at all ages, are characterized by obstruction, perforation, or bleeding. There are, however, significant differences in the etiology, diagnosis, and treatment of these conditions in the newborn infant. INTESTINAL OBSTRUCTION Diagnosis. Intestinal obstruction is the most frequent gastrointestinal emergency which requires surgery in the neonatal period. Early recognition is essential for success; the optimum time for sm'gicat intervention is usually within the first day of life, and a significant delay may increase the possibility of fatal outcome. Five major signs suggest the possible existence of intestinal obstruction: maternal hydramnios, excessive gastric aspirate, bilious vomitus, abdominal distension, and obstipation. M a t e r n a l hydramnios. I-Iydramnios is frequently associated with obstruction of the proximal gastrointestinal tract in the fetus. From the Departments of Surgery, Pediatrics, and Radiology of the University of Florida College of Medicine. Supported by United State Public Health Service Grant No. HD 03297 from the National Institute oJ Child Health and Human Development, Bethesda, Md.
Normally amniotic fluid is continually ingested and absorbed from the small intestine; some of this fluid is then transferred to the maternal circulation via the placenta and partially excreted in the urine of the mother. 1 Obstruction of the upper gastrointestinal tract interrupts this cycle and results in the accumulation of an excess of amniotie fluid. Hydramnios provides an early warning which, if correctly interpreted, may significantly decrease the delay of su@caI intervention after birth. Specific conditions which are associated with hydramnios include those producing obstruction of the esophagus, stomach, duodenum, or proximal small bowel. Obstruction of the distal small bowel or colon does not ordinarily produce hydramnios since an adequate surface for absorption remains in the proximal gastrointestinal tract. Excessive gastric aspirate. Gastric intubation should always be performed when there is maternal hydramnios; a gastric aspirate exceeding 15 to 20 c.c. of fluid, particularly if bilious, is suggestive evidence of intestinal obstruction. When there is obstruction of the upper intestinal tract, an abdominal roentgenogram obtained 4 to 6 hours after birth usually reveals distension of the intestine by accumulated gas above this obstruction. Bilious vomitus. Since obstruction proximal to the ampulla of Vater is rare (except Vol. 76, No. S, pp. 783-797
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Fig. 1. Small bowel atresia. The amount of air-filled small bowel may suggest the level of obstruction. A, duodenal atresia, B, jejunal atresia, C, ileal atresia.
by esophageal atresia), the color of the vomitus (clear or bilious) helps to distinguish the usual postprandial regurgitation of infancy from pathologic vomiting. Healthy newborn infants may spit up, but normal full-term infants do not vomit bile. In premature infants bilious vomiting may occasionally result from an immature pyloric sphincter. Abdominal distension. T h e newborn infant with intestinal obstruction or paralytic ileus develops abdominal distension owing to an accumulation of ingested air; this sign is not apparent at birth. The configuration of the distended abdomen may suggest the level of obstruction. Protuberance of the epigastrium indicates obstruction of the stomach or the duodenum, whereas generalized abdominal distension, usually apparent by 12 to 24 hours, suggests obstruction of the distal in-
testinal tract. The frequent association of abdominal distension (as a result of paralytic ileus) with peritonitis or sepsis necessitates the consideration of these entities in the differential diagnosis. Obstipation. Failure to expel meconium is a late sign of intestinal obstruction. A normal newborn infant may not have a meconium stool until 24 to 72 hours after birth. By this time other signs of intestinal obstruction are usually apparent. Occasionally small inspissated meconial stools are passed even in the presence of an obstructive lesion in the proximal gastrointestinal tract. Radiographic studies. Abdominal roentgenograms usually provide confirmatory evidence of intestinal obstruction. In congenital esophageal atresia a radiopaque catheter will not pass beyond the esophageal obstruction;
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air in the stomach indicates a distal tracheoesophageal fistula. The lungs of these infants are often overaerated and contain radiating a n d / o r confluent densities which reflect aspiration of oropharyngeal secretions and/or regurgitation of gastric contents. In gastric obstruction from a pyloric web, the stomach is distended and air is absent in the distal intestinal tract. In duodenal obstruction a characteristic "double bubble of accumulated gas" results from dual pockets of air distending the duodenum and stomach (Fig. 1, A). Complete midintestinal obstruction leads to distended, air-filled loops of jejunum and the absence of gas in the distal boweI (Fig:.. 1, B). Distal obstructions are more difficult to localize at this age because distended large and small bowel often cannot be distinguished on plain roentgenograms of the abdomen (Fig. 1, C). Diffuse distension of the bowel may be the result of a variety of obstructive lesions such as ileal or colonic atresia, aganglionic megacolon, meconium plug syndrome, intussusception, volvulus, and imperforate anus. In meconium ileus the roentgenogram may demonstrate minute bubbles of gas intermixed with meconium throughout the intestinal lumen, producing a granular "ground glass" appearance. 2 Peritoneal and scrotal calcifications and/or metaphyseal bands present at birth suggest intrauterine perforation and meconium peritonitisY, ~ An upper gastrointestinal radiographic study is needed to diagnose newborn intestinal obstruction only in those instances in which the obstruction appears incomplete or the exact site is in doubt. A barium enema may be useful in distinguishing the various types of lower bowel obstruction. Associated anomalies. The presence of intestinal obstruction in a mongoloid infant is presumptive evidence of duodenal atresia; 30 per cent of patients with duodenal atresia have mongolism. Atresias of the proximal gastrointestinal tract are often accompanied by premature birth. All forms of intestinal atresia are associated with an increased incidence of other congenital anomalies, particularly those involving the heart, spine,
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and urinary tract. Jejunal atresia may be associated with meconium ileus, intestinal malrotation, and volvulus. Malrotation of the intestine may also accompany omphaloeele, diaphragmatic hernia, or asplenia. The diagnosis of cystic fibrosis can usually be confirmed by analysis of sweat electrolytes in infants with meconium ileus; abnormalities of electrolyte a n d / o r protein content of hair, nails, and meconium have also been identified in the affected infants. Causes of intestinal obstruction. Preampullary obstruction. Repetitive nonbilious vomiting suggests obstruction proximal to the point of entrance of the common bile duct into the duodenum at the ampulla of Vater. ESOPr~AG~AL ATaESlA. Esophageal atresia is usually heralded by maternal hydramnios and after a few hours by respiratory distress in the newborn infant. With or without these signs the first feeding almost invariably produces severe choking and cyanosis owing to regurgitation and tracheal aspiration. In the rare instances of H-type tracheoesophageal fistula, the anomaly may escape detection for some days and present later with recurrent episodes of pneumonia and respiratory distress during feedings. T h e diagnosis of esophageal atresia is ordinarily confirmed by passing a No. 8 or 10 French radiopaque catheter through the nasopharynx into the proximal esophagus until obstruction is encountered approximately 10 cm. distal to the nares (at about the level of T2 vertebral body). A chest roentgenogram will then define the level of esophageal obstruction and indicate by the presence or absence of gas in the stomach whether there is an associated distal tracheoesophageal fistula. The rare association of a proximal tracheoesophageal fistula may also be excluded by injecting a small amount of contrast medium into the proximal esophageal pou@. Occasionally the diagnosis of esophageal atresia is overlooked when an excessively small or flexible feeding tube is inserted through the nasopharynx; the tube may coil in the posterior nasopharynx or rarely pass by way of the larynx, trachea, and tracheoesophageal fistu-
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la into the stomach, erroneously suggesting the presence of a patent esophagus. PYLORICl ATRESlA. Pyloric atr&ia is usually caused by a congenital web at the level of the antrum or pyloric sphincter. This condition is extremely rare and is difficult to identify, particularly v/hen it occurs in association with other forms of distal obstruction. A smal ! dimple of air may be produced by ballooning of the web into the distal duodenal lumen, simulating the double bubble sign considered pathognomonic for duodenal obstruction. At the time of surgical exploration a catheter should be passed from the stomach toward or into the duodenum to establish the level of obstruction. P Y L O R I C S T E N O S I S , Hypertrophic pyloric stenosis rarely is manifest in the first few days of life. Wallgren 5 reported no evidence of pyloric stenosis in a series of 1,000 upper gastrointestinal roentgenographic examinations of newborn infants. Statistically, 6 patients would have been expected to have had this diagnosis. Subsequently, 5 infants of the 1,000 did develop typical symptoms of pyloric stenosis, thus confirming the characteristic delay in the onset of symptoms of obstruction. Sporadic cases of hypertrophic pyloric stenosis in newborn infants have been reported, but this condition is usually characterized by progressive nonbilious vomiting in a Caucasian male infant between 2 weeks and 2 months of life. The vomiting often becomes projectile and is accompanied by weight loss and dehydration. If vomiting has been severe or prolonged, the serum electrolytes suggest a pattern of hypochloremic, hypokalemic alkalosis. Inspection of~the abdomen reveals a distended epigastrium with visible gastric peristaltic waves. In over 90 per cent of the patients a characteristic pyloric "olive" can be palpated just to the right of the midline in the epigastrium. DUODENAL ATRESIA. Vomiting is an early manifestation of this condition. Although the atretic segment is frequently located near the ampulla of Vater, bile often appears in the vomitus as the result of a proximal communication between the duodenum and the hepatobiliary system2 An abdominal roent-
The ]ournal o[ Pediatrics May 1970
genogram distinguishes this condition fl'om gastric obstruction (Fig. 1, A). Postampullary obstruction. These conditions comprise the overwhelming majority of cases of intestinal obstruction in the newborn infant. Bilious vomiting is the primary manifestation of obstruction of the small intestine. INTESTINAL ATRESIA. Approximately 25 per cent of atresias of the small bowel occur in the duodenum. Atresias of the colon or stomach are rare. Duodenal atresia is associated with mongolism. Stenosis is also more common in the duodenum than in other portions of the gastrointestinal tract. Occasionally stenosis escapes notice until adult life. It is rarely necessary t o perform extensive radiographic studies prior to surgical exploration of newborn infants with intestinal obstruction from atresia (Fig. 1). A barium enema will be helpful only in cases of distal ileal obstruction in order to exclude a lesion of the colon. The etiology of intestinal atresia is still under investigationfi Initially, the hypothesis of Tandler was widely accepted--that there was arrested intestinal development at a proliferative stage owing to a failure in the final process of recanalization. 7 Recent studies have revealed that obstruction of the lumen by epithelial growth occurs only in the duodenum of the fetus. Thus this theory does not explain the more frequent atresias of the distal small intestine. An alternative hypothesis of ischemic injury has been advanced by Louw, S and investigations have demonstrated that ligation of mesenteric blood vessels in fetal puppies will produce intestinal atresia at birth. Vascular injury of the gastrointestinal tract may be associated with volvulus, strangulation, and intussusception or with vasculitis from intrauterine infectious such as rubellaY ANNULAR PANCREAS. Annular pancreas is a rare condition caused by a fusion of the anterior and posterior analogues of the pancreas around the midportion of the duodenum. Severe stenosis or atresia of the duodenum may result. If obstruction is complete the diagnosis cannot be differentiated from
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Fig. :~ Malrotation and duodenal bands. "This infant developed bilious vomiting at 2 days of age. A, supine abdominal radiograph is normal, B, in the prone position the stomach and proximal duodenum are distended, C, barium enema shows the cecum to be in the right upper quadrant (arrow) confirming the diagnosis of malrotation. (Courtesy of Dr. John A. Kirkpatrick, Jr., St. Christopher's Hospital, Philadelphia, Pa.)
other causes of duodenal atresia. A side-toside duodenojejunostomy is the preferred operation when no other abnormalities are present; a bypass procedure with division of the constricting ring of pancreatic tissue is contraindicated. MALR.OTATION. Malrotation of the intestine is frequently associated with a large omphalocele or congenital diaphragmatic hernia; in these conditions intestinal migration may also be arrested, with segments remaining within the thoracic cavity or omphalocele sac. The diagnosis of malrotation of the intestine is suggested by the ectopic position of the cecum as demonstrated by barium enema (Fig. 2). Intestinal malrotation results from a failure of normal embryonic rotation of the cecum about the superior mesenteric vessels when the intestinal tract migrates back within the abdominal cavity. Instead of a 360 degree counterclockwise rotation whereby the cecum is positioned in the right lower quadrant, the process is ordinarily arrested when the cecum reaches the epigastrium. The mesentery of the small intestine remains suspended on a narrow pedicle which includes the superior mesenteric vessels. Lateral peritoneal bands connect the malptaced epigastric cecum to the right upper quadrant
abdominal wall and the liver. These attachments overlie the distal duodenum and may compress it, producing partial or complete obstruction of the lumen (Fig. 2). Since the small intestine is also suspended from the narrow mesenteric pedicle, the potential for obstruction from a volvulus is also present. This latter complication may occur either in utero or during early infancy/Early operative intervention is essential in cases of obstruction with midgut volvulus in order to avoid irreversible ischemic damage. The syndrome of asplenia and cyanotic heart disease may also be associated with realrotation. These cyanotic infants are distinguished by a right-sided heart, nucleated red blood cells (which may be misinterpreted as white blood cells by automatic counters, producing erroneously high white cell counts), bilaterally equal liver shadows on abdominal roentgenograms, and left axis deviation on the electrocardiogram. Any infant with this condition who develops intestinal obstrtt~tion should be immediately suspected of having a midgut volvulus? ~ MECONIU2r ILEUS. Approxmately 10 per cent of patients with fibrocystic disease of the pancreas develop intestinal obstruction from abnormally thick tenacious meconium. T h e
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diagnosis is suggested by the signs and symptoms of distal small bowel obstruction; perforation or volvulus of the intestine is a frequent complication. * The abdomen may have a characteristic doughy consistency owing to the presence of the abnormal meconium within the intestinal lumen. Perforation of the intestine results in meconium peritonitis and the development of intraabdominal and scrotal calcifications.
The Journal of Pediatrics May 1970
The instillation of acetylcysteine into the proximal gastrointestinal tract is a valuable adjunct in treating this condition; dissolution of the obstructing meconium may avoid surgical intervention. 11, 12 More frequently, a proximal temporary ileostomy is required for instillation of acetylcysteine or pancreatic enzymes into the distal bowel in order to remove the obstructing meconium. The high frequency of associated serious abdominal
Fig. 3. Meconium plug syndrome. A, diffuse intestinal distension is evident in this 2-day-old infant, B, barium study reveals a core of meconium in the rectosigmoid colon (arrow), C, on postevacuation study a segment of meeonium remains in the transverse colon (arrow).
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a n d / o r respiratory complications combine to produce an over-all poor prognosis in this condition. MECONIUM P L U G S Y N D R O M E . This syndrome is characterized by tenacious plugs of inspissated meconium which occlude the distal colon and rectum and produce temporary obstruction (Fig. 3). In most instances this entity is not associated with fibrocystic disease of the pancreas. Anorectal stenosis or atresia are rare disorders which can be distinguished from this syndrome b y digital examination of the rectum. Hirschsprung's disease can usually be excluded by a barium enema, although an occasional infant with the"'meconium plug syndrome sub,sequently ig found to have aganglionosis of the distal bowel. Digital examination of the rectum or a barium enema often results in the passage of the inspissated meconium plug with the relief of obstruction? ~ These patients should be followed, however, to exclude the possibility of a serious underlying abnormality. H I R S C I r l S P R U N G ' S D I S E A S E . Congenital megacolon results from an absence of ganglion cells in the parasympathetic myenteric nerve plexus of the colon and rectum. Rarely, this
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condition may involve the entire colon or even extend into the small intestine (Fig. 4) ; the absence of intestinal peristaltic activity is responsible for functional intestinal obstruction. In early infancy, constipation or severe diarrhea may occur. Hirschsprung's disease may also present as acute intestinal obstruction in the newborn infant. A severe, fulminant enteritis and overflow type of diarrhea can be secondary to such obstruction. Roentgenographic diagnosis may be difficult in early life, prior to the distension of the proximal intestine. Disproportionate distension of the large and small bowel suggests the usual variety of Hirschsprung's disease (Fig. 5). A competent ileocecal valve allows the colon to dilate; not uncommonly the sigmoid appears roentgenographically as a bowel loop or "mass" extending into the right upper quadrant. A careful barium enema with a straight-tipped catheter inserted just beyond the rectal sphincter usually confirms the diagnosis. The aganglionic segment appears contracted and the proximal intestine distended. Follow-up roentgenograms in 24 hours usually show significant retention of barium in the uninvolved proximal bowel. A definitive diagnosis of Hirschsprung's
Fig. 4. Malrotation and total aganglionic megacolon. This neonate presented with persistent bilious vomiting. A, moderate intestinal distension is evident, B, barium enema reveals the cecum (arrow) in the upper midabdomen. The entire colon is narrowed and the walls are irregular and serrated, C, marked retention of barium is evident on the 24 hour follow-up examination. No obstructing bands were found at the time of the operation.
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Fig. 5. Hirschsprung's disease. Two-day-old infant with vomiting and abdominal distension. A, there is diffuse gasseous distension of bowel. Note air-filled loop of bowel in right upper quadrant (arrows). B and C, barium enema confirms the diagnosis of Hirschsprung's disease with a low transitional segment (arrows). The right upper quadrant bowel seen in A is the redundant sigmoid colon. (Courtesy of Dr. John A. Kirkpatrick, Jr., St. Christopher's Hospital, Philadelphia, Pa.)
disease is established by the absence of ganglion cells in the myenteric and submucosal plexus of tissue obtained by a full-thickness rectal biopsy. Although so-called "skip-involvement" of the intestine has been reported, for practical purposes the aganglionosis usually extends continuously from distal involved to proximally uninvolved intestine. The treatment of choice is to establish colostomy at the point of transition from normal to aganglionic bowel. A definitive pull-through procedure is performed between 1 and 2 years of life, prior to the usual time of toilet training. 14 I M P E R F O R A T E A N U S . Physical examination at birth ahnost always establishes the di~tgnosis of imperforate anus. The anus may be normal in external appearance in cases of anorectal atresia or stenosis. Anomalies of the urinary tract and lumbosacral spine are frequently associated with imperforate anus and must be excluded in all patients by an intravenous pyelogram and roentgenograms of the spine. Cases of "low" rectal pouch are regularly associated with ectopic perineal openings or fistulas that extend, into the distal vagina. Cases of "high" rectal pouch usually have
an associated fistula into the urethra or bladder in males or to the upper vagina in female infants. Injection of contrast media into external fistulous tracts will usually define the level of the communicating pouch. Whenever an external fistula is absent, the urine of males or the v @ n a of females should be examined for meconium. Berdon and associates 15 have suggested that the classic upside-down abdominal roentgenogram may prove unreliable ifl predicting the length of the atretic segment of rectum (Fig. 6). Such studies may prove helpful, however, if they demonstrate passage of air into the bladder through a fistula. If none of these methods prove successful in demonstrating the level of the rectal pouch, urethrocystography may define a communication between the rectum and the bladder or urethra. The differentiation between a "high" and "low" rectal pouch has important surgical implications. If the pouch is high, a temporary colostomy is indicated and a definitive abdominoperineal pull-through procedure should be deferred; a primary perineal anoplasty is usually indicated for a "low" pouch. HERNIAS. If an infant has an incarcerated inguinal hernia at birth, reduction should
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Fig. 6. High imperforate anus. A, diffuse intestinal dilation with air-fluid levels is evident, B and, C, inverted studies reveal no air ira bladder. Following a colostomy with irrigation of the distal colon, meconium appeared in the urine.
not be attempted as is frequently the practice in older infants and children. There is a significant danger of strangulation and necrosis in these cases since the period of incarceration is uncertain. Immediate exploration a n d herniorrhaphy should be performed. MISCELLANEOUS. A variety of other conditions may produce intestinal obstruction. Intestinal bands may result from inflammatory adhesions or persistent vitelline duct remnants (i.e., Meckel's diverticulum). Intussusception is rare in the newborn infant and duplications of the intestinal tract usually produce partial intestinal obstruction. ~ INTESTINAL PERFORATION IN THE N E W B O R N
Intestinal perforation may occur prior to birth as a result of intestinal obstruction in the fetus, or it may develop following birth f r o m a variety of causes. Since the fetal intestinal contents are sterile and remain so for the first few days after birth, perforation during these periods produces a reactive, chemical peritonitis. If leakage occurs after the first few days of life, bacterial peritonitis ensuesJ 7 Antepartum perforation. Intrauterine perforation usually results from distal intestinal obstruction secondary to atresia, volvulus, strangulated hernia, or meconium ileus associated with cystic fibrosis of the pancreas.
Meconium is present in the distal intestinal tract to the level of the ileocecal valve by the fourth month of gestation and may be released into the peritoneal cavity when subsequent perforation occurs. The sterile meconium induces a severe chemical and foreign body reaction, an intense fibroplastic peritonitis. The peritoneal contents are enveloped by an adherent, fibrous membrane in which calcium is rapidly deposited. Since these events frequently antedate closure of the processus vaginalis, scrotal calcifications may also result. The point of perforation is usually sealed by the reactive process, but occasional persistent leakage may lead to the formation of a large fibrous cyst which can fill the entire abdominal cavity. If intraperitoneal leakage persists after the third postpartum day, bacterial contamination occurs. Intrauterine intestinal perforation and meconium peritonitis can be diagnosed prior to birth by maternal roentgenograms demonstrating typical calcifications within the fetal abdomen. Following birth such infants characteristically develop signs and symptoms of intestinal obstruction, either because of the original bowel lesion or as a result of the dense adhesive scar which encompasses the peritoneal contents. Intra-abdominal and scrotal calcifications a n d / o r metaphyseaI bands confirm the diagnosis, z' 3 Intraperi-
79 2
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toneal air appears within 48 t o 79 hours in those patients in whom intestinal leakage persists. Whenever intestinal "obstruction or persistent peritoneal leakage is suspected, immediate surgical exploration is required. In the absence of these indications, watchful waiting and SUldportive therapy are occasionally ~ustified? s Postpartum perforation. As in the case of the fetus, the most frequent cause of postpartum perforation is an underlying intestinal obstruction from-atresia, meconium ileus, volvulus, hernia, or Hirschsprung's disease. In ~ddition to these entities, however, several unique sources of neonatal gastrointestinal perforation should be recognized. Gastric per[oration. Neonatal gastric perforation usually occurs within the first week of life and often within the first 3 days. I9 Over 20 per cent of the patients are born prematurely. The diagnosis may be suggested initially by bilious vomiting and rapidly progressive abdominal distension. The entire abdomen, including the usual area of liver dullness, becomes tympanitic to percussion. The distension is frequently severe enough to produce secondary respiratory distress and circulatory embarrassment; signs and syrup-
Fig. 7. Gastric perforation. Three-day-old premature infant with vomiting and abdominal distension. An extensive pneumoperitoneum is evident; the faleiform ligament (arrow) is visualized. (Courtesy of Dr. Jbhn A. Kirkpatrick, Jr., St. Christopher's Hospital, Philadelphia, Pa.)
The ]ournal of Pediatrics May 1970
toms of shock may ensue. Plain abdominal roentgenograms are frequently pathognomonic of gastrointestinal perforation (Fig.
7). Neonatal gastric perforation may occur owing to gastric distension resulting from distal intestinal obstruction, positive pressure ventilation during resuscitation at birth, trauma to the stomach wall as a result of nasogastric intubation, or birth injury. The relatively high gastric acidity of the first few weeks of life, particularly in association with prematurity, intracranial lesions, or other sources o f neonatal stress, may also predispose to acute peptic ulceration and perforation. Finally, "idiopathic" gastric perforation in which none of the preceding factors are present has been recognized as a distinct entity in the neonatal period. The stomach wall characteristically ruptures along the greater curvature. Histologic examination of biopsy specimens taken from the site of perforation have suggested that a congenital deficiency of gastric musculature may be a primary or contributory factor. Regardless of etiology, surgical intervention is imperative in aIi cases; occasionally immediate relief of massive abdominal distension by needle or tube paracentesis and treatment of shock a n d / o r respiratory distress may be required. The situation is comparable to that encountered in tension pneumothorax, with impairment of diaphragmatic excursion, compression of the lungs, and obstructed venous return to the heart. If necessary, abdominal decompression may be maintained during transport of the child to surgery by attaching the paracentesis tube to an underwater seal. At the time of surgery, along with exploring the surface of the stomach, the surgeon must explore the intestine for the possibility of an associated distal intestinal obstruction. The site of perforation should be debrided of all devitalized tissue and closed with a double layer of inverting sutures. A gastrostomy is a useful adjunct for maintaining gastric drainage during the immediate postoperative period and subsequently for initiating intestinal alimentation. Neonatal necrotizing enterocolitis. Perfora-
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tion of the colon or terminal ileum in the newborn infant is often associated with an idiopathic necrotizing enterocolitis. 2~ T h e syndrome commonly occurs in the premature infant within the first few days of life, although it may appear in the full-term newborn infant as late as 2 months of life. The typical patient is a premature infant who has had mild respiratory distress at birth but subsequently did well until the third to fifth day of life when he developed vomiting, progressive respiratory distress, and abdominal distension. Signs of sepsis and shock with hypothermia and poor peripheral perfusion develop rapidly. Diarrhea or passage of bloody mucus per~xectum may also appear. Abdominal roentgenograms usually demonstrate free peritoneal gas, pneumatosis intestinalis, or hepatic portal venous gas. Occasionally the roentgenogram may confirm the diagnosis on the basis of these findings before the clinical syndrome becomes evident. A milder form of the disease is characterized by gross rectal bleeding; in contrast, rectal bleeding plays a relatively insignificant role in the more fulininant disease which rapidly progresses to perforation. Therapy should include intestinal decompression, parenteral fluid therapy, and antibiotics. A surgeon should be consulted immediately and should follow the patient carefully with the pediatrician for any evidence of progression of the process. Intraperitoneal perforation requires immediate exploration. If an operation is required the involved bowel should be resected and either an endto-end anastomosis or a temporary diverting colostomy or ileostomy should be performed. A survival rate of 67 per cent has been reported when these principles of management were employed. The etiology of this unusual condition remains unclear but there is a frequent association with previous cannulation of the umbilicM artery or v e i n y Histologic sections of the involved segments of bowel often suggest ischemic changes. Iatrogenic intestinal perforation. Traumatic perforation of the intestinal tract by nasogastric intubation or anorectal insertion
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of enema tubes, thermometers, or diagnostic catheters presents a particular threat to the newborn infantY 2 The distance between the anus and the sigmoid colon within the peritoneal cavity may be less than 3 cm. at this age. Therefore, insertion of any firm object into the infant's rectum involves a significant risk. This complication can be prevented by using soft rubber catheters for rectal cannulation, limiting the insertion of rectal thermometers to a distance less than 2 cm., and the careful administration of any enema in the newborn infant. Neonatal appendicitis. Appendicitis is extremely difficult to diagnose in the newborn infant. The disease is rare at this age and the symptoms are relatively nonspecific? 7 The rarity of this condition in the infant is attributed to a relatively wide appendiceal lumen with a sparsity of intestinal lymphoid tissue. Appendiceal obstruction, the initiating factor in most cases of appendicitis, is therefore less likely to occur at this age than in older children or adults. An association of Hirschsprung's disease with perforation of the appendix has been documented, and rectal biopsy should be considered in all such cases in infants. (If the diagnosis of Hirschsprung's disease is confirmed by an absence of ganglion cells in the specimen, a diverting colostomy should be performed immediately.) Treatment of intestinal perforation. The treatment of any form of intestinal perforation in the newborn infant includes nasogastric decompression, restoration of fluid balance, correction of electrolyte and acid-base disturbances, and administration of antibiotics. Judicious haste is imperative; the goals are early restoration of homeostasis and surgical exploration within 3 to 4 hours. Immediate abdominal paracentesis may be required in some patients to relieve massive abdominal distension and associated cardiorespiratory embarrassment. In assessing fluid rr ments it should be noted that the total peritoneal surface is roughly equivalent to 75 per cent of the body surface area. Accordingly, fluid losses in a patient with peritoneal inflammation may approximate those encountered in a major body surface burn. The
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monitoring of serial determinations of central venous pressure, total serum prot_eins, hematocrit, and urinary output and specific gravity is particularly helpful in maintaining an optimum balance of fluid and electrolytes. GASTROINTESTINAL
BLEEDING
The high mortality rate from gastrointestinal hemorrhage in the newborn infant emphasizes a need for prompt diagnosis and treatment. The loss-of 60 ml. of blood in the full-term infant is comparable to a loss of 600 td 700 ml. in the adult. Seemingly small amounts of blood in the stool of an infant may represent a significant loss. Extrinsic sources of blood as well as intrinsic sites of bleeding must be considered in the differential diagnosis. Sources of fieonatal bleeding. Maternal sources--swallowed blood syndrome. Swallowed maternal blood usually appears in the stools on the second to third day of life. It may be swallowed by the infant during delivery or ingested during breast-feeding if there is a fissure of the nipple. The test of Apt and Downey 23 will distinguish between maternal and infant blood on the basis of the greater resistance to alkali denaturation of red blood cells containing fetal hemogiobin. Newborn bleeding disorders. Hemorrhagic disease of the newborn infant secondary to hypoprothrombinemia may cause a bleeding disorder at this age and may require treatment by administration of vitamin K and fresh blood or plasma. Less frequently, congenital or acquired deficiencies of coagulation factors or platelets may also cause bleeding in the newborn infant. Intestinal ischemia. Intestinal ischemia and subsequent gangrene can occur when the mesenteric blood supply of the bowel is compromised. Malrotation of the intestine with associated volvulus is an example of this complication; in the newborn infant the presence of a palpable abdominal mass, intestinal obstruction, and bloody stools usually indicates a volvulus rather th~n an intussusception. Other situations associated with intestinal
The Journal of Pediatrics May 1970
ischemia, such as incarcerated hernia and neonatal necrotizing enterocolitis, are infrequent in the newborn period. Stress ulcer. Stress ulcers of the stomach and duodenum may lead to rapid exsanguination of the small infant. 24 Gastric lavage with cold saline solution or topical thrombin has been advocated as treatment, but frequently early exploration for direct suture ligation of the bleeding vessel in the base of the ulcer is necessary. An upper gastrointestinal radiographic series may be helpful in demonstrating an acute stress ulcer of the stomach or duodenum, but the presence of blood in the stomach in association with rapid hemorrhage is usually adequate indication for immediate laparotomy. Extensive gastric surgery, such as resection or vagotomy and pyloroplasty, is not required. Gastric drainage through a large gastrotomy tube is a useful adjunct to ensure gastric decompression and can then be used for postoperative feeding. SPECIAL SURGICAL TECHNIQUES
The following surgical techniques may provide palliative relief until definitive surgery is indicated. Pediatricians should be aware of file technical details of some of these procedures if they participate in postoperative management. Gastrostomy. In patients with a tracheoesophageal fistula, a gastrostomy provides a means for decompressing the gastrointestinal tract and preventing regurgitation of acidic gastric contents into the tracheobronchial tree. This technique, in combination with aspiration of the proximal blind esophageal pouch, may facilitate a delay of definitive surgery for hours or even days and provide for the infant's condition to stabilize, reducing the risk of subsequent operation. The gastrostomy may be combined with closure of the tracheoesophageal fistula when longer periods of support are required. A number of excellent techniques have been developed by pediatric surgeons to perform gastrostomies under local anesthesia in the newborn infant. 2s, 26 After intra-abdominal neonatal surgery a
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gastrostomy provides postoperative gastrointestinal decompression and avoids the potential complications of prolonged nasogastric intubation, e.g., airway obstruction by intrusion of the tube into the nasopharynx, erosion of the mucosa of the stomach or esophagus, or inadequate decompression by a small nasogastric tube. A mushroom type of catheter may be preferable to the Foley balloon catheter for gastrostomies in newborn infants; the balloon at the Foley catheter tip may be too large relative to the size of the stomach and produce partial gastric or pyloric obstruction. The Foley balloon can also pass into the duodenum and produce complete obstruction. A ivariety of techniques have beer~ advocated f0 prevent this latter complication, including speciaI double balloon catheters, stabilization with spools, etc., but a simple, adequate method is to secure the catheter at the skin level with a bolus of gauze snugly wrapped about it. Various devices have also been proposed for administering gastrostomy tube feedings and achieving simultaneous decompression while the feeding is progressing (an automatic "burper). A reliable approach is to attach a large, glass, Y tube to the end of the gastrostomy tube. If this system is then elevated, the height of the fluid column within the tube represents the maximum pressure which can be exerted within the stomach itself. Feedings may be administered through one side arm of the Y tube while simultaneous decompression is achieved through the opposite arlTl.
Enterostomy. Another technique helpful in initiating early feedings in infants after a gastrointestinal operation is the insertion of an enterostomy tube. It is either threaded through the gastrostomy into the distal small bowel or inserted into it through a separate incision. These tubes must be made of a pliable material which retains its soft consistency when exposed to the digestive juices of the intestinal tract. Silicone rubber tubing with an internal diameter of a No. 18 needle has proved optimal for this purpose. Other plastic materials frequently become rigid when inserted into the gastrointestinal tract,
Gastrointestinal surgical emergencies
795
a characteristic which invites subsequent erosion and/or perforation. The enterostomy tubes are connected to constant infusion pumps for administration of feedings since the diameter is too small for a free gravity flow of formula (even when special noncurd formula mixtures are employed). Gastric aspirate from the gastrostomy tube may also be replaced into the distal gastrointestinal tract with this system. Colostomy. If the specific requirements of the small infant are not recognized, the maintenance of colostomies at this age may be associated with a variety of surgical complications2S, 2s; with proper attention t o detail, however, the postoperative management of a colostomy should be almost as easy as caring for a normal infant. The pediatrician must guard against the development of constipation or of stenosis of the colostomy orifice; it may be necessary to dilate the colostomy stoma periodically a n d / o r to add stool softeners. Irritation of the skin around the colostomy orifice is caused by excessive moisture. This complication should be treated by applying powdered cornstarch to the skin. If maceration develops, karaya gum powder should be substituted for cornstarch. When the colostomy has been performed as a palliative step, final corrective surgery should be undertaken prior to the time of initiating toilet training. 9 Monitoring techniques. A variety of monitoring techniques are necessary to manage gastrointestinal surgical emergencies successfully in the newborn infant. 27 These supportive measures often provide the critical margin for success, but they do not substitute for frequent careful clinical re-evaluation and adequate nursing care. Those techniques which deserve special consideration include the following: Cardiovascular [unction. Electrocardiographic monitoring has been facilitated by the introduction of miniaturized skin electrodes; arterial pressures can be followed with indwelling catheters inserted into the umbilical, temporal (needle), brachial, radial, or femoral arteries. These same catheters provide a method for repeated sampling of
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arterial blood for the determination of Mood gas values. Umbilical artery catheters can be used for infusing fluids. Fluid balance. I n recent years the dangers of overloading the circulation of the newborn infant with excessive fluids during the postoperative period have become apparent. T h e dangers of underhydration with the potential o f hypovolemia and inadequate tissue perfusion, however, deserve equal attention. I n addition to continual clinical evaluations, a composite monitoring regimen is necessary to achieve the appropriate level of hydration; it should include recording of fluid intake and output with balances calculated at 6 hour i~tervals or more frequently if indicated; serial measurements of central venous pressure and frequent determinations of total serum osmolality and protein concentration, hematocrit, and urinary specific gravitY. T h e latter tests can be measured on small amounts ( < 0.1 ml.) of body fluids with a h a n d refractometer and microcentrifuge. 2s Serial determinations of the central venous pressure provide a relatively new and important technique to avoid overloading the circulation. A catheter is inserted into the superior vena eava or right atrium through a jugular vein. W h e n interpreted with regard to the other determinations, it provides a means for estimating cardiac filling pressure and relative intravascular expansion. It is particularly useful in postoperative infa.~ts who have sequestered significant quantities of fluids, electrolytes, and proteins within body tissues as a result of ileus, peritonitis, or t r a u m a (edema), in children with pulm o n a r y edema and heart failure, and in newborn infants with immature renal function who may have low urinary specific gravities in spite of significant dehydration. Long-term parenteral nutrition. Not infrequently the neonatal patient who has had corrective surgery for a gastrointestinal anomaly requires a relatively long time to regain enough gastrointestinal function to provide calories, fluids, and electrolytes adequate for nutrition and growth. Recently special parenteral solutions have been used to provide these patients with increased
The Journal o[ Pediatrics May 1970
amounts of calories and other essential substrates.2~, 30 These prepara~0ns are administered through central venous catheters composed of inert substances (special silicone rubber tubing) designed to minimize tissue reaction and clotting. Small volumes are continuously administered with infusion pumps in order to increase the opportunity for dilution and mixing of these hypertonic solutions with blood. REFERENCES
1. Lloyd, J. R., and Clatworthy, H. W.: Hydramnios as an aid to the early diagnosis of congenital obstruction of the alimentary tract: A study of the maternal and fetal factors, Pediatrics 21: 903, 1958. 2. Neuhauser, E. B. D.: Roentgen changes associated with pancreatic insufficiency in early life, Radiology 46: 319, 1946. 3. Wolfson, J. J., and Engel, R. R.: Anticipating meconium peritonitis from metaphyseal bands, Radiology 92: 1055, 1969. 4. Donnison, A. B., Schwachman, H., and Gross, R. E.: A review of 164 children with meconium ileus seen at the Children's Hospital Medical Center, Boston, Pediatrics 37: 833, 1966. 5. Wallgren, A.: Incidence of hypertrophic pyloric stenosis, Amer. J. Dis. Child. 62: 751, 1941. 6. Hays, D. M.: Intestinal atresia and stenosis, Curr. Probl. Surg. Oct., 1969. 7. Tandler, J.: Zur Entwicklungsgeschichte des menschlichen Duodenum in fruken Embryonalstudien, Morph. Jahrb. 29: 187, 1902. 8. Louw, J. H.: Congenital intestinal atresia and stenosis in the newborn. Observation on its pathogenesis and treatment, Ann. Roy. Coll. Surg. Eng. 25: 209, 1959. 9. Esterly, J. R., and Talbert, J. L.: Jejunal atresia in twins with presumed congenital rubella, Lancet 1: 1028, 1969. 10. Schiebler, G. L.: Personal communication. 11. Mecker, I. A., Jr., and Kincannon, W. N.: Acetylcysteine used to liquefy inspissated meconium causing intestinal obstruction in the newborn, Surgery 56: 419, 1964. 12. Simpson, T. E., Lynn, H. B., and Burgert, E. O., Jr.: Nonoperative treatment of meconium ileus: Report of a case, Mayo Clin. Prec. 43: 725, 1968. 13. Mikity, V. G., Hodgman, J. E., and Paciulli, J.: Meconium blockage syndrome, Radiology 88: 740, 1967. 14. Steichen, F. M., Talbert, J. L., and Ravitch, M. M.: Primary side-to-side colorectai anastomosis in the Duhamel operation for Hirschsprung's disease, Surgery 64: 475, 1968. 15. Berdon, W. E., Baker, D. H., Santulli, T. V., and Amoury, R.: The radiologie evaluation of imperforate anus: An approach correlated
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16. 17. 18. 19. 20.
21.
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with current surgical concepts, Radiology 90" 466, 1968. Laird, A. P., and Kerr, M. M.: Antenatal jejunal intussusception, Z. Kinderchir. Suppl. zu Bd. 5: 76, 1968. Ravitch, M. M., and Rowe, M. I.: Surgical emergencies in the neonate, Amer. J. Obstet. Gynec. 103: 1034, 1969. Lange, M.: Meconium peritonitis presenting in scrotal hydroceles, Brit. J. Surg. 51: 942, 1964. Robarts, F. H.: Neonatal perforation of the stomach, Z. Kinderchir., Suppl. zu. Bd. 5: 62, 1968. Stevenson, J. K.; Graham, C. B., Oliver, T. K., and Goldenberg, V. E.: Neonatal necrotizing enterocolitis, Amer. J. Surg. 118: 260, 1969. Corkery, J. J., Dubowitz, V., Lister, J., and Moosa, A~: Colonic perforation after exchangeziitransfusion, Brit. Med. J. 4: 345, 1968. ': Greenbaum, E. I., Carson, M., Kincannon, W. N., and O'Laughlin, B. J.: Rectal thermometer-induced pneumoperitoneum in the newborn, Pediatrics 44: 539, 1969. Apt, L., and Downey, W. S., Jr.: "Melena" neonatorum: The swallowed blood syndrome. A simple test for the differentiation of adult
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24. 25. 26.
27. 28.
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and fetal hemoglobin in bloody stools, J. PEDIAT. 47: 6, 1955. Spencer, R.: Gastrointestinal hemorrhage in infancy and childhood: 476 cases, Surgery 55: 718, 1964. Lewis, J. E.: Atlas of infant surgery, St. Louis, 1967, The C. V. Mosby Company. Mustard, W. T., Ravitch, M. M., Snyder, W. H., Jr., Welch, K. J., and Benson, C. D.: Pediatric surgery, Chicago, 1969, Year Book Medical Publishers, Inc. Talbert, J. L., and Haller, J. A., Jr.: Recent advances in the postoperative management of infants, J. Surg. Res. 6: 502, 1966. Thoene, J. G., Talbert, J. L., Subramanian, S., and Odell, G. B.: Use of the hand refractometer in determining total serum proteins of infants and children, J. PEDIAT. 71: 413, 1967. Wilmore, D. W., Groff, D. B., B{shop, H. C., and Dudrick, S. J.: Total parenteral nutrition in infants with catastrophic gastrointestinal anomalies, J. Pediat. Surg. 4: 181, 1969. Filler, R. M., Eraklis, A. J., Rubin, V. G., and Das, J. B.: Long-term total parenteral nutrition in infants, New Eng. J. Med. 281: 589, 1969.