Urgent Surgical Problems in Childhood

Urgent Surgical Problems in Childhood SAMUEL R. SCHUSTER, M.D., F.A.C.S.* ANGELO J. ERAKLIS, M.D.** DAVID S. TRUMP, M.D.***

The surgical problems presented in this paper are, for the most part, peculiar to the formative period of life, and have been restricted to those that differ in etiology, diagnosis or therapy from those occurring in the adult. Although children present with a multiplicity of problems, it is a surprising fact that only a few of these require immediate surgical intervention as a lifesaving measure. There are several features of general management that relate particularly to infants. The margin of safety or, in some instances, the margin of error in these small patients is strikingly less than that which normally applies to an adult. Acute blood loss in an infant reaches critical levels early. An average 7-lb. infant with a total blood volume of approximately 225 cc. loses almost 25 per cent of this volume with a 50-cc. blood loss. What appears to be a minor loss in an older individual becomes critical in the infant. Conversely, blood replacement must be accomplished accurately to avoid overtransfusion which readily results in pulmonary edema. In another parameter, a newborn infant with respiratory disease may develop pneumothorax with only 20 to 25 cc. of air in the pleural space. This small limitation of pulmonary expansion in an already limited patient may prove critical since the normal tidal volume of a small neonate may be as little as 10 or 15 cc. Transport of the Acutely III Infant Rickham has said that "approximately two children out of every From the Surgical Service, Children's Hospital Medical Center, and the Department of Surgery, Harvard Medical School, Boston, Massachusetts. * Clinical Assocate in Surgery, Harvard Medical School; Associate Surgeon, Children's Hospital Medical Center ** Assistant Clinical Professor of Pediatric Surgery, Boston University School of Medicine; Assistant in Surgery, Children's Hospital Medical Center ••• Resident in Surgery, Children's Hospital Medical Center

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thousand live births will require urgent surgery." Often these problems are reeognized in one hospital and transportation to another is required for definitive surgical therapy. Certain principles should be rigidly followed to avoid compounding the problem during transport. In patients with intestinal obstruction, esophageal atresia and diaphragmatic hernia, a short nasogastric tube aspirated periodically with a simple bulb syringe may prevent vomiting, aspiration pneumonitis and bowel distention. In addition, care in keeping a patient with tracheo-esophageal fistula in a semi-upright position is helpful in preventing regurgitation of acid gastric contents into the lungs by way of the fistula. Administration of oxygen en route to the hospital in certain instances may prevent anoxia with subsequent cardiac arrest and brain damage. Finally, maintenance of a normothermic state in these infants is of great importance; it has been demonstrated that severe acidosis in infants may develop in association with a hypothermic state. TRAUMA

Abdominal Injuries in Childhood Accidents are the leading cause of death during childhood, causing onethird of all deaths in children under 14 years of age. Head injuries, fractures and burns account for the majority of the patients admitted. During the period 1951-1964, 71 children underwent abdominal exploration at the Children's Hospital Medical Center as a result of trauma. Only 18 per cent of the injured children were involved in automobile accidents. The majority of injuries were due to a play situation such as sledding, bicycling or other athletic activities. Twelve patients were under two years of age at the time of operation and the youngest was less than 24 hours of age. Four infants were explored for severe injury of abdominal organs incident to the trauma of birth, and three children for trauma inflicted by a parent. The spleen is the organ most often injured, with the kidney, bowel, liver and pancreas next in order of incidence. The history and physical findings often are most difficult to interpret. Admission to the hospital for careful observation is essential for all children with suspected abdominal injuries. Although the principles of surgical management do not vary, early surgical exploration is advised when the findings are not clear. In two patients of the Children's Hospital Medical Center series, abdominal exploration was entirely negative; in six others, injuries were noted but no specific surgical therapy was needed. All these patients recovered. A negative exploration in 10 per cent of the cases seems to be an acceptable degree of error to avoid missing a potentially correctable intraabdominal injury.

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Traumatic Pancreatitis

Pancreatitis in children is most commonly related to blunt trauma of the upper abdomen. A serum amylase determination is advised in all children with a history of abdominal injury; however, an elevated serum amylase level should not preclude surgical exploration if a suspicion of multiple organ injury exists. Surgery in the child with pancreatitis does not carry the same poor prognosis as in the adult. A prolonged elevation of the serum amylase in the child with traumatic pancreatitis may correlate with the development of a pancreatic pseudocyst. In our series a six year old boy was explored and found to have traumatic pancreatitis; he ran a persistently high serum amylase level postoperatively (300 to 700 Somogyi units). Six weeks later a pancreatic pseudocyst was noted and a cysto-gastrostomy performed. Following this procedure the amylase levels returned to normal. The second patient with a pseudocyst of the pancreas was~a 15 month old child admitted with a diagnosis of "failure to thrive." A pancreatic pseudocyst was suspected by clinical and x-ray findings. At exploration a thick-walled pseudocyst of the body of the pancreas was noted and a Roux-en-Y cysto-jejunostomy was done. Both patients have done well with no recurrence of the disease. Ruptured Spleen

Twenty-nine patients had a splenectomy for splenic rupture. The youngest in this series was a 36 hour old infant admitted with pallor and lethargy as the chief complaints. The hematocrit was 18.5 per cent and a flat plate of the abdomen suggested the presence of free fluid in the peritoneal cavity; diagnostic paracentesis showed free blood. Splenectomy was done for bleeding from the splenic pedicle; postoperatively the child was proved to be a classic hemophiliac. A 10 day old infant was admitted with a history of profound shock, a hematocrit of 10 per cent and a distended abdomen. At exploration a 3-cm. laceration of the convex surface of the spleen was noted. Both infants made an uneventful recovery. In these infants there was no history of trauma other than the stress incident to the delivery itself. The incidence of delayed rupture of the spleen is lower in children; only one occurred in our series. Earlier reports suggesting an increased susceptibility of a child to serious infections following splenectomy for trauma have not been confirmed. In our series of 48 patients followed for an average of five years there was no instance of meningitis, septicemia or other forms of overwhelming infection. A series of 465 patients who underwent splenectomy at the Children's Hospital Medical Center for a variety of reasons was reviewed. The results indicate that the primary hematologic disease is the leading factor related to the increased incidence of fatal infection. Hepatic Injuries

Hepatic injury is common and carries a higher mortality in the pedia-

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tric than in the adult patient. Moritz lists hepatic injury second to head injury as the leading cause of death from trauma. Six children in the Children's Hospital Medical Center series sustained severe hepatic injuries from blunt trauma to the abdomen. One infant explored at 48 hours of age had sustained injury to the liver incident to a breech delivery. Four children were involved in accidents and the remaining child fell from a bicycle. Two children died as a result of their injuries. The convex surface of the right hepatic lobe was the common site of injury and the size and extent of the liver laceration is the single most important factor determining the prognosis. One child admitted with minimal signs of injury following a bicycle accident was discharged only to be readmitted one month later with a large epigastric mass. Exploration proved this to be a 15 by 20 cm. intrahepatic sterile abscess containing bile; the abscess was marsupialized to the skin. Extensive contusion of the left lobe of the liver leading to formation of an intrahepatic hematoma and subsequent biliary fistula is the presumed pathogenesis for this lesion.

Contusion and Perforation of the Bowel Of the 71 operative cases following abdominal trauma in the Children's Hospital Medical Center series, 16 were found to have a perforation or severe contusion of the intestine or intestinal mesentery. The trauma often seemed insignificant. One boy, four years of age, fell from a chair sustaining a perforation of the jejunum. X-ray examination of the abdomen, with the patient in fiat and upright positions, showed no evidence of pneumoperitoneum in this case or in eight other cases of isolated small bowel perforations. At exploration the fact that the small bowel was found to be in severe spasm may account for the lack of free air in the peritoneum. The antimesenteric wall of the jejunum near the ligament of Treitz is the most common point of injury. One of the younger patients in this series was a 12 day old infant who was dropped from her mother's arms into a wicker basket. At exploration 12 hours later a linear tear of the greater curvature of the stomach was found. Despite repair the infant died of severe peritonitis. A careful inspection of the small bowel mesentery should be made in any child explored with a history of trauma. An otherwise insignificant tear in the mesentery may be the source of a lymph-peritoneal fistula. A recent report from the Boston City Hospital of a two month old child with debilitating persistent chylous ascites due to a tear of the mesentery is noteworthy.

The Physically Abused Child The child who is noted by history, physical examination or roentgenogram to have sustained multiple and unusual fractures and contusions or burns may be the victim of assault. The true incidence of the physically

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abused child is unknown. An increasing awareness of this syndrome by physicians and the recent development of state-sponsored agencies for investigation and prosecution of offenders has resulted in a marked increase in the number of proven cases. Of the 71 children explored for abdominal trauma at the Children's Hospital Medical Center, the injury sustained by three was proved to have been inflicted by the parents. A seven month old child was admitted with a diagnosis of intestinal obstruction. On examination the child was lethargic and had a markedly distended abdomen; x-ray examination showed diffuse small and large bowel distention with air fluid levels. At exploration a small volume of serosanguineous fluid was noted with multiple tears of the small and large bowel serosa and large hematomas scattered in the wall of the bowel, mesentery and retroperitoneum. Foul play was suspected and later confirmed by the Social Service Department. Another patient, a three year old child, was kicked by an adult and on exploration a perforation of the jejunum was repaired. The third child, a 15 month old boy, was admitted with "failure to thrive" and proved to have a pseudocyst of the pancreas. A skeletal survey showed many old and recent fractures of the ribs and long bones. All suspected cases should have a skeletal survey and a report made to the Social Service Department and the hospital administration. This will lead to a discrete investigation which may benefit not only the patient but also the siblings. RESPIRATORY DISTRESS

Diaphragmatic Hernia The congenital diaphragmatic hernia is one of the few problems of the neonate which may require emergency surgical intervention as a lifesaving measure. Patients with this problem usually present within the first 24 to 48 hours of life with variable symptoms of respiratory inadequacy. Cyanosis may be apparent immediately after birth or may not develop until later as a result of dilatation of that part of the gastrointestinal tract present within the chest. Dilatation of these herniated loops of intestine results in further compression of the lung on the affected side, as well as a shift of the heart and mediastinum to the opposite side with compression of the contralateral lung. In our series of 150 diaphragmatic hernias of childhood, excluding hiatal hernias, 90 per cent occurred through a defect in the posterolateral part of the left diaphragm known as the foramen of Bochdalek. Physical examination, although rarely in itself diagnostic, is helpful in establishing the diagnosis. The most frequent findings consist of a child in respiratory distress, distant breath sounds on the affected side, bowel

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Figure 1. X-ray of a newborn infant with a left congenital diaphragmatic hernia and shift of the mediastinum to the right.

sounds over the chest, and sometimes absence of bowel sounds in a scaphoid abdomen. The heart sounds are often best heard on the side away from the hernia. Definitive diagnosis depends upon roentgenographic examination of the chest. A plain anteroposterior view of the chest usually demonstrates the loculations of gas representing loops of bowel above the diaphragm. Often it can be shown that the loops of air-filled intestine in the chest are in continuity with other similar shadows beneath the diaphragm. The lung on the affected side may be partially or completely collapsed and, depending on the degree of dilatation of the herniated bowel, there may be a striking shift of the mediastinum to the contralateral side. A classic x-ray picture of a congenital diaphragmatic hernia is shown in Figure 1. Rarely there may be some difficulty in determining whether the loculations above the diaphragm represent herniated bowel, congenital lung cyst, staphylococcal pneumonia or even pneumothorax. When there is doubt about the diagnosis the administration of a small amount of barium by mouth may reveal a diaphragmatic hernia. Infants with this problem are often in such severe respiratory distress that significant relief is provided only by rapid surgical reduction of the hernia. Certain helpful measures which may be carried out preoperatively include the insertion of a nasogastric tube for gastrointestinal decompression as soon as the diagnosis is established, placement of the baby with the affected side down while in a semi-upright position and an environment with a high oxygen concentration. Operative correction may be accomplished by way of an abdominal or

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thoracic incision. We prefer the abdominal approach for three reasons: (1) A large percentage of these patients have an associated malrotation of the bowel which may lead to early intestinal obstruction; it is well to correct this at the initial operation. (2) Reduction of the herniated viscera into the abdomen may increase the intra-abdominal pressure, resulting in marked elevation of the diaphragm and postoperative respiratory distress. This may also compromise the venous return to the heart. An intentionally created incisional hernia of the abdomen may relieve the intra-abdominal pressure sufficiently to avoid this problem in the postoperative period. (3) Reduction of the herniated viscera from above may be very difficult. The high mortality associated with this lesion is especially related to those infants presenting with severe respiratory distress during the first 24 hours of life. There has been virtually no mortality in our series in newborn infants whose symptoms have not been severe enough to require admission and surgery during the first 24 hours of life.

Pneumothorax Pneumothorax stands high on the list of the urgent problems of the newborn. The factors which influence the development of this condition in the newborn are (1) overly excessive efforts at resuscitation, and (2) underlying pulmonary disease. Very vigorous attempts to insufflate the lungs of a newborn infant have been well documented as a cause for rupture of subpleural blebs and resultant pneumothorax. Although these resuscitative measures are necessary it is well to keep in mind the serious complications which arise following overly vigorous expansion of the neonatal lung. Hyaline membrane disease which occurs almost exclusively in prematures is often complicated by the development of pneumothorax. Conversely, full-term infants have been shown by Emery to be more prone to the development of pulmonary interstitial emphysema, which is also often complicated by the development of pneumothorax. Pulmonary interstitial emphysema usually develops in a previously diseased lung. The most common underlying problems are aspiration, pneumonia, hemorrhage and hypoplastic lungs. Symptoms resulting from a pneumothorax may appear immediately following birth or there may be a delayed onset following a period of apparently normal respiration. With the onset of difficulties, breathing becomes labored, irregular and shallow, and there is retraction of the intercostal muscles. As emphysema or pneumothorax progresses there may be ballooning of the chest, tracheal shift and cardiac displacement. This may be difficult to detect by physical examination alone, since the signs are similar bilaterally. Although the diagnosis may be suggested by physical examination, it is necessary to confirm it radiologically. The clinician must have a high

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Figure 2. X-ray of an infant in severe respiratory distress with marked bilateral tension pneumothorax.

index of suspicion in any neonate with sudden onset of respiratory distress, particularly if any other pulmonary disorder is known to be present. Figure 2 is a roentgenogram of an infant with severe respiratory distress due to a bilateral pneumothorax. Definitive treatment of a pneumothorax requires the use of closed chest thoracotomy with underwater seal low pressure suction (2 to 3 cm. water). Although success has been reported with needle aspiration, it should not be used routinely since it does not control a continuing air leak and may cause further difficulties as a result of laceration or puncture of the lung by the aspirating needle.

Mediastinal Emphysema Most cases of mediastinal emphysema are mild and need no specific therapy. Very rarely, mediastinal emphysema may be severe enough to threaten life. Most of these patients respond to cervical mediastinotomy if care is taken to incise through the pretracheal fascia. If this procedure fails, tracheostomy is recommended. This decompresses the mediastinum and precludes the further expulsion of air into the interstitial tissue by preventing the development of high intrabronchial pressures during coughing.

Lobar Emphysema This condition is characterized by undue expansion of a lobe resulting from obstruction of the bronchus permitting ingress but little or no egress of air. The typical clinical picture is that of an infant who has a variable degree of respiratory distress, depending on the size of the emphysematous lobe and the amount of displacement of the other intrathoracic structures.

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Symptoms may present within a few days or weeks of birth and are progressive in severity. Not only is the function of the emphysematous lobe markedly diminished, but that of the remaining lung tissues may be compromised. Respiratory reserve is decreased as the diaphragms are pushed down by the increased intrathoracic pressure. Finally, mediastinal displacement and increased intrathoracic pressure may combine to inhibit venous return to the heart. The severity of symptoms depends on the extent to which these changes occur. Figure 3 is a roentgenogram of an infant with obstructive lobar emphysema of the left upper lobe. There is compression of the adjacent lobe and shift of the mediastinum to the right. Approximately half the patients have symptoms in the first day or two of life. These youngsters have the more severe form of the disease and almost invariably come to operation within a few weeks. Cou'gh or wheezing may be prominent. Rarely a newborn infant exhibits the fulminating form of the disease in which there is a rapid progressive lobar distention with marked impairment of respiratory and, ultimately, cardiac function. Oxygen, although helpful, is not of great value and these patients can die in a few hours unless emergency lobectomy is done as a lifesaving measure. The treatment of obstructive lobar emphysema is surgical resection of the affected lobe. Temporizing measures are both useless and hazardous. Of previously reported cases, the mortality of "expectant" treatment is greater than 60 per cent. At the Children's Hospital Medical Center nonoperative treatment of patients with onset of symptoms in the neonatal period has been 100 per cent fatal.

Figure 3. Infant with obstructive lobar emphysema of the left upper lobe. There is compression of the adjacent lobe and shift of the mediastinum to the right.

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Twenty-nine patients at the Children's Hospital Medical Center have been treated by lobectomy. All of these had onset of symptoms during infancy and all patients have survived operation and have done well since operation with no recurrence of symptoms. The urgency with which lobectomy must be carried out varies from patient to patient. The occasional neonate with this problem must be operated upon as an emergency procedure, whereas the older infant with less demanding symptoms may do well for a considerable period of time. Occasionally, however, a patient of this kind will die suddenly. It is therefore most prudent to do a lobectomy soon after the diagnosis is established.

Acute Laryngotracheobronchitis and Epiglottitis (Croup) Although many children with mild forms of this condition need nothing other than a humid environment and antibiotics, those few with severe edema of the larynx, epiglottis, and trachea are most desperately in need of urgent surgical intervention. These youngsters have marked retraction of the thorax and accessory respiratory muscles, extreme pallor and cyanosis, and marked inspiratory stridor. Until very recently urgent tracheostomy was the only manner whereby many of these youngsters could be kept from asphyxiation. More recently the use of dexamethasone (Decadron) in association with antibiotics has obviated the need of tracheostomy in some patients. Despite this, any patient who presents with the above findings is a candidate for tracheostomy; procrastination may be hazardous. The procedure is most readily carried out after the child has already been intubated with a small endotracheal tube, which permits control of the situation and an unhurried and carefully executed procedure. Choanal Atresia Bilateral complete choanal atresia, a rare entity, is 3 serious threat to life in the newborn. Neonates have not yet learned mouth breathing and are completely dependent upon the nares for respiration. The diagnosis must be entertained in any newborn with respiratory difficulties. These youngsters are particularly symptomatic at rest, do poorly while sucking, and seem to improve when crying. The diagnosis is established by the inability to pass a small nasal catheter (No.8 F.) into the nasopharynx, or radiographically by the use of an aqueous contrast material inserted into the nose. Temporary relief is obtained by use of an oral airway until definitive operative correction can be accomplished. Pierre Robin Syndrome The physical complex of micrognathia, cleft palate, glossoptosis, and occasional cleft lip constitutes a cause for respiratory distress in neonates and is a serious threat to life. Because of the hypoplastic mandible, the

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genioglossus muscles which usually attach near the symphysis of the mandible are unable to exert their full force forward. Consequently, the tongue falls backward into the hypopharynx pressing on the epiglottis, and acts as a ball valve. Egress of air is allowed while ingress is inhibited and saliva tends to seal the oropharynx resulting in cyanosis, dyspnea, substernal retraction and poor feeding. Without adequate treatment the affected baby may die of anoxia or even from inanition. Over the years there have been various methods of traction devised to maintain the tongue in a forward position. These included traction by head

Figure 4. A, Newborn infant with Pierre Robin syndrome in the preferred prone position to avoid airway obstruction. Note roll under shoulders. B, Proper method of feeding an infant with Pierre Robin syndrome using a Breck feeder. Note that nurse holds the mandible forward during feeding.

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braces, direct tractibn sutures through the tongue, mandibular wires, and suturing of the tongue to the lower lip. There have been attempts to solve feeding difficulties by using various plastic prostheses to cover the cleft palate and allow the youngster to suck. None of these methods has been . consistently effective. In our experience it has not been necessary to resort to these methods. Proper positioning of the patient in a prone position with a roll under the shoulders, allowing the head to drop to a lower level than the thorax, is effective in preventing respiratory distress. Figure 4, A, shows one of these infants in this position. Feeding has been accomplished in almost all instances by the use of a Breck cleft palate feeder, as noted in Figure 4, B. The baby is held in an upright position with t4.e index finger and thumb of the nurse applied to the angles of the mandible pushing it forward. No attezppt should be made to use a nipple. These methods, carried out by carefully trained nursing personnel, will almost invariably carry the patient through the first 2 to 3 critical weeks. During this time the mother is instructed in the method and usually has no difficulty once the patient is discharged. In rare instances it is necessary to do a tracheostomy and even more rarely one finds it necessary to do a feeding gastrostomy to prevent inanition. The problems associated with this syndrome rapidly disappear after the first few months of life, as the mandible continues developing in a more normal fashion.

Cystic Hygromas and Lymphangiomas Lymphangiomas and cystic hygromas are congenital malformations of the lymphatic system. These tumors are composed of lymph-containing endothelial-lined spaces varying in size from channels of capillary caliber to large cyst-like spaces, often multiloculated, which may be several inches in di:1meter. Some are small and well localized whereas others are diffuse and "infiltrating" in nature. Approximately 50 to 65 per cent of thElse lesions are present at birth and 80 to 90 per cent are detected before the end of the second year of life. Landing and Farber classified lymphangiomas and cystic hygromas into three groups: (1) lymphangioma simplex, composed of capillary-sized thin-walled lymphatic channels, (2) cavernous lymphangioma, composed of dilated lymphatic channels and (3) cystic lymphangioma (cystic hygroma), composed of cysts varying from a few millimeters to several centimeters in diameter. All three types may be found together and, in addition, there is an occasional cavernous hemangioma associated with one of these lymphatic malformations. The term lymphangioma is used to describe lesions composed of micrQscopic or smalllymph:filled channels, and the term cystic hygroma includes those cOPlposed .of macroscopic lymph-containing cysts. The form of tumor often varies with the location. Those found in the

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Figure 5. A, Infant with a massive cystic hygroma and hemangioma of the neck causing airway obstruction. Tracheostomy was required. B, Lymphangiomatous involvement of the tongue in infant causing airway obstruction and feeding difficulties. This patient also required a tracheostomy.

neck, arm, axilla and mediastinum are predominantly cystic hygromas, whereas those of the face and tongue are almost invariably of the lymphangiomatous variety. Respiratory obstruction is the most serious presenting complaint in the neonate and is usually associated with large hygromatous masses of the neck and/or lymphangiomata of the tongue or larynx. Problems of deglutition often occur in patients with respiratory obstruction. Figure 5, A, shows a newborn infant with a massive cystic hygroma and hemangioma of the neck, who required tracheostomy because of airway obstruction. Figure 5, B shows an infant with airway obstruction and feeding difficulties caused by lymphangiomatous involvement of the tongue. He also required tracheostomy. The only effective therapy for these lesions is surgical excision. The type of surgical procedure employed is dictated by the location of the tumor and its type. In general, total excision is performed whenever possible; however, the lesion frequently involves vital areas which cannot be excised or where the risk of irreversible injury is such that a less extensive resection is done. In some patients with extensive tumor involving the tongue, partial glossectomy, often preceded by tracheotomy, is the most effective therapy. Tracheo-esophageal Anomalies

Some years ago the definitive repair of these anomalies was considered a surgical emergency. More recently it has become apparent that the emergent features of these anomalies are related primarily to the prevention of aspiration and chemical pneumonia.

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The type C anomaly with the tracheo-esophageal fistula communicating with the distal esophageal segment represents over 86 per cent of these patients. The details of the initial management of this group serve to illustrate the basic early surgical measures necessary for prevention of aspiration pneumonia. When the diagnosis is first established a sump suction catheter is placed in the upper esophageal segment for removal of the salivary secretions. As an additional precaution the infant is kept in a semi-upright position to prevent regurgitation of the very irritating acid gastric contents into the lungs through the tracheo-esophageal fistula. Immediately following admission a gastrostomy is done regardless of the patient's condition. If the patient is a full-term infant in good condition, a planned repair of the esophagus and division of the tracheo-esophageal fistula is completed within the first 24 to 36 hours of hospitalization. If severe pneumonia is present, only an emergency gastrostomy is done under local anesthesia and the esophageal repair and division of the fistula is postponed for 2 to 3 days while the pneumonia is under treatment. The gastrostomy provides a "bubbling" or venting mechanism for the baby, and therefore aids in the prevention of reflux through the fistula during the delay prior to definitive esophageal repair and division of the fistula. Early definitive repair of esophageal atresia in premature infants is associated with higher mortality rates than the same procedure in full-term infants; they are therefore managed differently. A staged procedure for repair of the atresia and fistula has proved effective in decreasing the mortality rate. The staged procedure consists of an immediate gastrostomy under local anesthesia followed by a retropleural division of the tracheoesophageal fistula. After the infant has attained a weight of 6 to 7 lbs. definitive repair of the atresia is accomplished. The early division of the fistula prevents regurgitation of gastric contents into the lungs during the long delay before definitive repair is completed. During this period of growth the baby is fed through the gastrostomy, and continuous sump suction of the salivary secretions is maintained under constant nursing supervision.

GASTROINTESTINAL HEMORRHAGE

Severe hemorrhage necessitating urgent surgical control is not a frequent occurrence in infants and children. The lesions most frequently encountered are described below. Peptic Ulcer Disease It has been pointed out on a number of occasions recently that peptic ulcer disease in childhood is increasing in incidence or is being recognized more frequently.

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The peptic ulcer of childhood comprises two major groups, the acute and the chronic forms. The acute ulcer, peculiar to infancy and early childhood, usually presents with an abdominal catastrophe such as perforation or hemorrhage without any previous symptoms. In most cases of gastric hemorrhage due to this lesion, a program of conservative medical management with blood replacement will usually suffice. When severe hemorrhage persists despite adequate blood replacement, surgical control of the bleeding becomes necessary. The term "childhood" covers such a variable period of age and body size that no single rule can serve for all age groups. An infant bleeding massively from a peptic ulcer may require surgery much sooner than a child of six or seven years. The decision as to when operation must take place depends on the findings and clinical course of the individual patient. Several of our own patients have died of hemorrhage because of undue persistence in medical management. It is rare, however, to find a death from hemorrhage following operative control of bleeding. There should be no reluctance to operate once a reasonable attempt at medical management has failed. The operative procedure of choice in the treatment of acute ulcers consists of simple ligation of the bleeding point in the base of the ulcer. In contrast, the chronic ulcer represents the childhood counterpart of peptic ulcer disease in the adult and is presumably due to a similar derangement of gastric physiology. It most commonly makes its appearance in the pre-adolescent and adolescent years in patients who have a history of repeated episodes of abdominal discomfort. Persistent gastric hemorrhage in the child with chronic ulcer requires surgical intervention just as it would in the adult. The operative procedures available are not different, but certain inherent considerations in the young influence the choice of procedure. The postoperative nutritional hazards associated with subtotal gastric resection may be as disabling as the condition for which the patient was treated. Such nutritional problems are a major concern in any patient, but in the critical periods of growth before and during adolescence they are of extreme importance. It is for this reason that surgery for chronic peptic ulcer disease in the child does not include resection of a large part of the stomach, but should alter the gastric physiology sufficiently to avoid recurrence of the problem. The procedures of choice are vagotomy with pyloroplasty or vagotomy and antrectomy. Esophageal Varices

Bleeding from esophageal varices produces the most severe form of gastrointestinal hemorrhage seen in childhood. The etiology of the portal hypertension in the majority of children is an extrahepatic occlusion of the portal venous system probably resulting from omphalitis during infancy. A definite history of newborn omphalitis is seldom elicited. A few patients in our series had had an exchange transfusion through the umbilical vein.

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In a smaller number of cases the obstruction is due to an intrahepatic occlusion resulting from posthepatic cirrhosis or cirrhosis resulting from exposure to toxic agents, glycogen storage disease or cystic fibrosis. Massive bleeding in these children is treated by blood replacement, sedation and, if necessary, compression of the esophageal and gastric varices with a Sengstaken-Blakemore tube. Occasionally massive hemorrhage persists despite all of the above measures. It is best, under these conditions, to ligate the esophageal varices and plan to do the definitive shunt as an elective procedure at a later date. Our experience with shunting procedures in the very young child has generally been unsatisfactory. We feel it is best to delay a definitive shunt procedure as long as possible, and at least until the child weighs 50 pounds. Since most of these youngsters have had thrombosis of the portal vein, the procedure of choice for decompression of the portal system is a splenorenal shunt. Meckel's Diverticulum In a child under two years of age, an ulcer in a Meckel's diverticulum is a common cause of massive hemorrhage presenting with either tarry or burgundy red stools. If upper gastrointestinal bleeding from esophageal varices and peptic ulcer is excluded and bleeding persists, or is recurrent, laparotomy is advised. Bleeding from a Meckel's diverticulum almost invariably arises from a small peptic ulcer at the neck of the pouch or in the nearby jejunum. The peptic digestion results from the action of the hydrochloric acid and pepsin secreted from aberrant gastric mucosa lining the diverticulum. Treatment should consist of resection of the diverticulum as well as a section of contiguous jejunum; continuity is re-established by an end-to-end anastomosis. The bleeding ulcer may be missed by simple excision of the diverticulum or wedge resection. When a Meckel's diverticulum is not found, thorough exploration may reveal other less common causes of massive bleeding, such as duplications of the gastrointestinal tract, intestinal polyps, or hemangiomata of the bowel wall.

MISCELLANEOUS GASTROINTESTINAL PROBLEMS

Duodenal Obstruction and Midgut Volvulus Although all congenital duodenal obstructions require early laparotomy, there is only one such high obstruction in the neonate which dictates urgent surgical correction. Duodenal obstructions such as atresia, annular pancreas and stenosis may be decompressed by nasogastric tube and corrected electively within the first 24 to 36 hours of hospitalization. In contrast, malrotation of the bowel with midgut volvulus often presents with an identical appearance on

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Figure 6. A, X-ray of a newborn infant with congenital duodenal stenosis. B, X-ray of a newborn infant with malrotation and volvulus of entire midgut. Shows marked similarity to appearance of x-ray in A.

plain x-ray of the abdomen and yet, unless rapid operation with reduction of the volvulus is done, the entire midgut may become gangrenous. Figure 6, A, shows an abdominal roentgenogram of a neonate with duodenal obstruction resulting from an intrinsic duodenal stenosis. Figure 6, B, shows a very similar picture, but with slightly less air in the stomach and duodenum, of an infant with malrotation and volvulus of the entire midgut. It is therefore necessary to clearly differentiate the various forms of duodenal obstruction. A simple barium enema will suffice to determine the location of the cecum. Abnormal fixation of the cecum indicates the presence of a malrotation and dictates immediate laparotomy. Operative treatment consists of counterclockwise reduction of a midgut volvulus, and lysis of the congenital bands extending from the cecum and across the duodenum to the right posterior abdominal wall. Perforations of the Gastrointestinal Tract

Perforations of the gastrointestinal tract may be secondary to trauma, ulcer, intestinal obstruction with massive dilatation, muscular deficiencies of the intestinal wall, and arterial emboli. Neonates with perforation are listless and acutely ill with marked distention and hyperresonance of the abdomen. The abdomen may not show any muscle spasm or rigidity. The diagnosis is established by x-ray examination, which often reveals a striking pneumoperitoneum. In some cases pneu-

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Figure 7. A, X-ray of a newborn infant with sigmoid perforation following breech delivery requiring use of forceps. Marked pneumoperitoneum is evident. B, X-ray of a newborn infant with gastric perforation of undetermined etiology showing pneumoperitoneum and extravasated barium.

moperitoneum is not evident on x-ray study, but physical examination indicates the presence of an acute surgical process which requires urgent laparotomy. Figure 7, A, is a roentgenogram of a neonate with an extensive pneumoperitoneum due to a sigmoid perforation following breech delivery requiring the use of forceps. Figure 7, B, is a similar roentgenogram of a newborn infant with massive pneumoperitoneum and extravasated barium following gastric perforation of undetermined etiology. Adequate preparation of these infants for surgery includes decompression through a nasogastric tube, placement of an intravenous cannula, and administration of plasma, electrolytes, and fluids. At operation, perforations of the stomach and higher small bowel are either closed primarily or the area is resected and a primary anastomosis completed. When the perforation is in the ileum or large intestine, we prefer a Mikulicz ileostomy, ileocolostomy, or a loop colostomy at the site of perforation. The diagnosis of aganglionic megacolon (Hirschsprung's disease) must be suspected with a perforation of the ileum or large intestine.

Ingestion of Caustic Agents It is not uncommon for children to ingest caustic agents with resultant severe erosion of the oropharynx, esophagus and stomach. Today there is a

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greater diversity of causative agents as a result of the many forms of cleaning solutions and dishwashing agents available to the exploring hands and mouths of young children. There is, in our opinion, nothing to be gained by early esophagoscopy or dilatation. The hazards of perforation associated with these procedures more than outweigh any benefit to be derived. Recently there has been evidence to suggest that early use of a combination of steroids and antibiotics is helpful in preventing severe scarring and contracture. Only a small percentage of all children admitted with a probable diagnosis of caustic ingestion have actually sustained a severe hurn of the mouth or esophagus. These youngsters must be under close hospital supervision in the event that complete esophageal obstruction occurs or if complete erosion of the stomach leads to perforation and its consequences. Both complications are uncommon but occasionally do occur. In most instances, early complete esophageal obstruction is transient and disappears as the edema subsides. Occasionally, however, a feeding gastrostomy and tracheostomy become necessary because of the patient's inability to swallow and severe edema of the hypopharynx resulting in respiratory distress. The most frequent complication of ingestion of caustics is chronic stricture of the esophagus necessitating frequent esophageal dilatations and occasional esophageal resection. In some cases resection may be limited to a short segment of severely scarred esophagus with end-to-end anastomosis. In other instances the entire esophagus may be so scarred that almost total resection with esophageal replacement by a colonic segment is required.

Omphaloceles Newborn infants with omphaloceles require urgent surgical attention. There are basically two forms: (1) the omphalocele with intact membrane, and (2) the omphalocele with ruptured membrane. The initial management of either of these prior to surgery consists of covering the omphalocele with moist sterile saline sponges and wrapping these with sterile towels. Prolonged delay may lead to rupture of the membranes with increased contamination and trauma to the exposed bowel. A small omphalocele can be reduced and the abdominal wall repaired without difficulty. In contrast the large omphalocele creates many problems owing to its great size and a lack of space within the abdominal cavity. Forceful reduction of this huge ventral hernia may end disastrously because of the severely increased intra-abdominal pressure which leads to respiratory distress and diminished venous return to the heart. Several methods have been proposed to accomplish repair. One method consists of repeated application of tincture of mercurochrome to the membrane, producing an eschar which gradually shrinks and brings about closure by overgrowth of skin. This is a long and not always successful process. The most frequently used method includes simple skin coverage of

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the mass, creating an intentional ventral hernia; complete repair of the abdominal wall is then completed when the child is older. Secondary repair of this ventral hernia is also fraught with hazard due to the absence of space within the abdomen. A more recent method which holds promise is the use of multiple staged procedures to obtain complete anatomical repair of the abdominal wall within the first six to eight weeks of life. The management of the large ruptured omphalocele is an even greater problem, particularly when the mass of herniated viscera is large and, as in an antenatal rupture, there is gross engorgement and contamination of the structures. Until very recently, almost all of these babies died regardless of the method of treatment. There has been striking improvement in the mortality rate with a multiple staged procedure utilizing plastic materials as temporary cover while the abdominal wall is gradually closed over the omphalocele mass.

URINARY PROBLEMS

Urological emergencies in newborn infants can be divided into two basic groups: (1) those that are a result of congenital anomalies leading to urinary outflow obstruction, and (2) those resulting from trauma. The most important signs of a urological emergency in the neonate are failure to urinate and sepsis. This may be due to renal, ureteral or urethral agenesis or atresia, or to complete obstruction of the bladder outflow. Bilateral renal agenesis, or dysgenesis and multicystic kidneys will be fatal until the time arrives when we can successfully do kidney transplantations. The most serious indication for urgent surgical intervention is partial urinary obstruction affecting both kidneys. The most frequent causes are ureteroceles and posterior urethral valves. In these children the distended bladder and hydronephrotic kidneys are palpable as masses. Occasionally, a female infant may present with a ureterocele prolapsing from the urethra. These conditions must be recognized and treated immediately. A catheter is passed into the bladder as both a diagnostic and a temporary therapeutic measure. An intravenous pyelogram is advised even if the blood urea nitrogen is elevated, as this may be on the basis of dehydration. Pyelogram usually shows marked bilateral hydronephrosis and hydroureter with tortuosity of the ureters. Following this, a voiding cystourethrogram usually shows dilatation of the bladder and proximal urethral and bilateral reflux. This occurs in patients with posterior urethral valves as well as in those with a ureterocele obstructing the bladder outlet. Figure 8 is a cystogram of a neonate with posterior urethral valves demonstrating all these features. Cystograms of patients with ureteroceles also demonstrate a filling defect in the bladder.

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Figure 8. Cystogram ofan infant with posterior urethral valves. This shows free reflux into markedly dilated and tortuous ureters and pelves. The site of the urethral valves is well localized at the distal point of urethral dilatation.

Although a bladder catheter offers temporary decompression of the upper urinary tracts, it is often not completely effective owing to the tortuosity and kinking of the dilated ureters. Bilateral nephrostomies or loop ureterostomies may be necessary to adequately decompress the kidneys until the ureters regain tone and a more normal peristaltic action. Definitive therapy of the posterior urethral valves involves urethrotomy and excision of the valves, whereas the ureterocele is best treated by cystotomy and enlargement of the ureteral orifice on the affected side. Torsion of the Testes

Occasionally acute torsion of one of the testicles occurs. This may happen before birth, during birth, or at any time during childhood. This condition must be recognized immediately and is characterized by bluish discoloration of the scrotum associated with marked edema and diffuse tenderness. The proper treatment is immediate exploration through an inguinal incision. The torsion most commonly occurs within the tunica vaginalis; however, it may occur in the spermatic cord structures above the testicle. Unfortunately, most of these testes have already become necrotic and there is very little that one can do to save them. If there is any doubt, the testicle should not be removed. When torsion has occurred on one side there is an increased probability that a similar anomaly is present on the opposite side and prophylactic fixation of the other testicle is advised.

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30. Oeconomopolous, C.: The value of glossopexy in the Pierre Robin syndrome. New England J. Med. 262: 1267, 1960. 31. Po, B. T. and Whitlock, W. G.: Idiopathic neonatal gastric perforation. South. M. J. 58: 720, 1965. 32. Pecora, D. V., Yegian, D., Hochwald, A. and Brook, R.: Tracheotomy in the treatment of severe mediastinal emphysema. J.A.M.A. 160: 354, 1958. 33. Potter, E. L.: Fetal and neonatal deaths. A statistical analysis of 2,000 autopsies. J.A.M.A. 115: 996, 1940. 34. Potter, E. L.: Pathology of the Fetus and the Infant. Chicago Year Book Medical Publishers Inc., 1962. 35. Robin, P.: Backward lowering of the root of the tongue causing respiratory disturbances. Bull. Acad. Med-Paris 89: 117, 1923. 36. Schuster, S. R. and Gross, R. E.: Peptic ulcer disease in childhood. Am. J. Surg. 105: 324, 1963. 37. Schuster, S. R.: Diaphragmatic hernias in infants and children. Quart. Rev. Pediat. 15: 171, 1960. 38. Schuster, S. R.: Recognition and management of inguinal hernia in infants and children. Quart. Rev. Pediat. 12: 201, 1957. 39. Schuster, S. R. and Gross, R. E.: A new method for the treatment of omphalocele. To be published. 40. Shwachman, H., Kulczycki, L. L. and Khaw, K. T.: Studies in cystic fibrosis: a report on sixty-five patients over 17 years of age. Pediatrics 36: 689, 1965. 41. Sieber, W. K. and Girdany, B. R.: Rupture of the spleen in newborn infants. New England J. Med. 259: 1074, 1959. 42. Weinstein, E. C., Cain, J. C. and Re Mine, W. H.: Meckel's diverticulum. J.A.M.A. 182: 251, 1962. 43. Zollinger, R. M. and Ellison, E. H.: Nutrition after gastric operations. J.A.M.A. 154: 811, 1954. 300 Longwood Avenue Boston, Massachusetts