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This is a report of a simple transanal operation performed on six patients (age range, 19 months to 18 years), who underwent unsuccessful nonoperative management of complete rectal prolapse for at least 1 month (range, 1 month to 13 years). All patients had normal sweat chloride levels, normal chest radiographs, and normal barium enemas. None of the patients were neurologically compromised. At the time of surgery, all but one patient had occurrence of reducible prolapse with minor straining or with every bowel movement. No severe mucosal ulcerations were present. Surgical therapy consisted of the transanal mucosal sleeve resection described herein. In this series, there were no anastomotic leaks, no clinically evident strictures and no recurrence of prolapse in 1.5- to 19-year follow-up. Surgical therapy for rectal prolapse in infants and children is rarely necessary. Various complicated or ineffective operations for the treatment of this condition have been recommended in the past. This technique offers a simple, safe, and effective method of treating complete, medically intractable rectal prolapse in children.

OBJECTIVE To establish the effect of an audible airleak (around an endotracheal tube) on oxygen consumption (VO2) measurements in pediatric ICU patients. DESIGN Prospective trial comparing VO2 measurements before and after deflation of the endotracheal tube cuff. SETTING Pediatric ICU in a large pediatric tertiary care center. PATIENTS Twenty critically ill infants and children receiving mechanical ventilatory support via cuffed endotracheal tube. INTERVENTIONS Deflation of endotracheal tube cuff. MEASUREMENTS AND MAIN RESULTS The presence (group 1, n = 9) or absence (group 2, n = 11) of an audible airleak with the cuff deflated was confirmed by two independent observers. The percent difference in VO2 was calculated for both groups using the following formula: ([VO2 cuff up - VO2 cuff down]/VO2 cuff up) x 100. An audible airleak associated with cuff deflation (group 1) caused a significant (p = .0012) reduction of VO2 by 45.6% (mean difference in VO2 = 45.6%). In contrast, with no audible airleak after cuff deflation (group 2), only minimal changes in VO2 (mean difference in VO2 = -0.4%) were observed. CONCLUSIONS These data suggest that if no audible airleak is detected, VO2 determined by indirect calorimetry may be reliably measured in infants and children with a noncuffed endotracheal tube.

Controversy exists over the best method of treating gastroschisis with concomitant intestinal atresia because the mortality in such patients is still high. We present our experience of 74 neonates with gastroschisis seen over a 17-year period. Four patients (5.5%) had intestinal atresia; 1 had only one small bowel atresia, 2 had double areas of atresia (one in the mid small bowel and the other in the proximal colon), and the remaining 1 had multiple areas of atresia. Atresia with gastroschisis conventionally has been treated by either primary anastmosis or by exteriorization. Three of the patients were treated by replacing the atretic bowel into the abdomen initially and exploring later (1 to 3 weeks) when the bowel appeared much more healthy, making the anastomosis easier and more secure. One patient with extreme jejunal dilatation was treated by creation of a Mikulicz fistula. All 4 of the patients survived the newborn period, but one of them died at 1 year of age due to total parenteral nutrition-induced chronic liver failure. Our experience is presented to emphasize this uncommonly used approach to this complex problem because it seems to be associated with a lower morbidity and mortality than other conventional approaches.

Recognizing the grief of parents after they have lost a child is an integral part of the practice of pediatric surgery. Parents who have experienced the loss of a child suggest the following: (1) The feelings associated with the loss of a child are not understandable unless you have lost a child of your own. (2) If you have not experienced the loss of your own child you should not state "I understand." You can't (3) The death of a child places stress on the marriage. (4) Many marriages do not survive. If the marriage does survive it becomes stronger than before the child's death. (5) During the recovery the parents see themselves as "different people." (6) Mothers and fathers react to the death differently. (7) Some parents have initiated new, beneficial projects that are in some way related to the child's death. (8) Parents react adversely to "pat" explanations and platitudes. (9) Parents want their friends and relatives to remain available. (10) The dead child should remain as an integral part of the family and no attempt should be made to "forget." (11) The self-help group that is referred to most frequently is "The Compassionate Friends, Inc.," with headquarters in Oak Brook, Illinois.

Neonates with esophageal atresia and tracheoesophageal fistula (TEF) may present with respiratory distress. Intubation and mechanical ventilation may force air from the tracheobronchial tree, through a distal fistula and into the gastrointestinal tract. We present a newborn with TEF who became morbund during mechanical ventilation. High ventilator pressures transmitted via the TEF caused over distention of the gastrointestinal tract and perforation of a Meckel's diverticulum.

In 1976, we presented our experience in the surgical management of patients with peripheral congenital arteriovenous fistulae (CAVF). This report updates our experience and specifically describes subsequent experience with intraarterial embolization therapy. Twenty-six infants and children with CAVFs have been treated at Children's Hospital of Los Angeles from 1966 to 1990. The majority of lesions were lower extremity (12), followed by upper extremity (8), shoulder girdle (3), and thorax/neck (3). Seventeen patients had lesions in multiple locations. All patients under 1 year of age had upper extremity lesions, all in the 5- to 10-year age group had lower extremity lesions, and all over 15 years of age at presentation required amputation. Twelve patients had initial embolization therapy and 12 had surgery. Seven of eight patients followed after embolization have improved. Embolization is not effective in large or ulcerated lesions. All patients with embolization alone have residual disease and three of eight have discrepancy in extremity length. Surgery has a 50% complication rate and 7 of 10 followed surgery patients have residual disease. Radiologic catheter techniques and embolization provide a valuable alternative therapy for CAVFs and should be used as the primary form of treatment in most cases.

Testicular feminization (TF) is a syndrome due to androgen insensitivity. It occurs in a complete (CTF) and an incomplete (ITF) form. We have treated 21 patients with TF over the last 24 years. Eight patients presented because of ambiguous genitalia, seven presented as "females" with inguinal hernia and testes were found at surgery, five were diagnosed by karyotyping performed for a family history of TF, and one presented with an incarcerated hernia and primary amenorrhea. Two patients had prior surgery for inguinal hernia but the diagnosis was not recognized. All patients had a 46,XY karyotype. Patients with CTF were phenotypically female while those with ITF had a variable appearance of the external genitalia depending on the degree of androgen insensitivity. Seventeen patients underwent gonadectomy and one patient planned for delayed gonadectomy was lost to follow-up. Seventeen patients had been raised as females since birth. One patient with ambiguous genitalia, who was initially raised as a male, was reassigned female gender at 1 year of age when the diagnosis of ITF was made. Three patients were raised as males even after the diagnosis of ITF was made. Patients raised as males underwent multiple genital reconstructive procedures with poor results. In view of the poor anatomic and functional results of genital reconstructive surgery and the consequent psychological problems, patients with TF should be raised as female. Careful evaluation of infants with ambiguous genitalia and documentation of absent fallopian tubes in "females" presenting with inguinal hernia will lead to early diagnosis of TF, correct sex assignment, and early gonadectomy.

Several congenital anomalies of the portal vein and inferior vena cava (IVC) have been reported; however, reports of a congenital communication between the portal vein and IVC are few. We report a patient who was found to have a natural shunt between the extrahepatic portal vein and the IVC that was discovered when she underwent a right hepatic lobectomy for an undifferentiated liver sarcoma. The patient also had agenesis of the right kidney. We have not been able to find a similar case reported in English literature.

The etiology, clinical presentation, and management of neonatal intussusception differs markedly from the more common infantile variety. This report summarizes the clinical course of three neonates with intussusception seen during the past 5 years. Bilious emesis, rectal bleeding, and abdominal distension were the cardinal symptoms in these neonates. A contrast enema was diagnostic in one patient but hydrostatic reduction of the intussusception was not possible. One patient had a Meckel's diverticulum and another had an ileocecal duplication cyst. In-utero intussusception of a Meckel's diverticulum was the cause of a gap-type ileal atresia in the third patient. Surgical excision of the causative lesion was performed in all three patients.

The patient with partial gastric antral obstruction due to a diaphragm may present with obstructive symptoms at any age in life. Including the four patients presented, there are now 50 reported cases. Since the diagnosis has been unduly delayed in many adults, it is particularly important that those physicians and surgeons caring for infants and children be aware of this entity. If properly treated in infancy and childhood, many years of suffering and debility can be obviated.

During the past 40 years, the survival of patients with esophageal atresia and tracheoesophageal fistula has increased from zero to approximately 85 percent. With the increased survival rate, the surgeon has the opportunity to follow up the patient into adult life. By so doing, the early and late complications can be anticipated and treated appropriately.

It is estimated that between 2300,000 and 4,000,OOO cases of active child abuse occur in the United States annually [I], and estimated deaths due to child abuse vary from 2,000 [2] to 50,000 annually [I]. The extreme variation in estimates is due to the difficulties inherent in proving or disproving child abuse. The child is either too young to testify on his own behalf or too frightened to do so. The abusive adult is not apt to admit to the abusive act, and those who live with him or her will assume a protective posture toward the abuser, thus denying all such allegations. Although the number of reported cases of intramural duodenal hematoma is increasing, there has been no consistent reportage of the association of active child abuse with this injury. Some of the case reports indicate “trauma of unknown etiology.” Such injuries may, in retrospect, be due to child abuse [3-81. In 1969 we reported our experience with seven patients who were treated for intramural duodenal hematoma [9]. Traumatic injury was denied by the parents of one patient, and in another the details of the trauma were not available. In retrospect, these two patients may have been injured by an abusive adult; however, at the time the manuscript was completed none of the patients was recognized as having been actively abused. During the subsequent seven years (1969-1977) eight patients have been treated for duodenal hematoma at the Childrens Hospital of Los Angeles. A retrospective review of these patients forms the basis of the present report.

During the years 1941 through 1973, 48 patients, 16 males and 32 females, with sacrococcygeal teratoma were seen at the Childrens Hospital of Los Angeles. Forty-four patients have been followed, three are lost to follow-up, and one patient died 2 wk after excision of teratoma. Of the 44 patients with follow-up, 26 had teratoma with mature tissues only, all these patients are living. Six patients had tumor containing mature and embryonic tissues. Of these, five are living and one died with metastases of malignant teratoma 1 yr after excision of the primary tumor. Of the remaining 12 patients, 11 have died during the first 4 yr of life due to malignant teratoma and only one is living without recurrence 15 yr after excision of teratoma containing frankly neoplastic tissues. Recurrence and/or metastasis of malignant sacrococcygeal teratoma was lethal in all instances.

Significant macrofistulae between arteries and veins of congenital origin may result in the extremes of life-threatening hemorrhage or simply a cosmetically displeasing mass. The most helpful diagnostic aid is that of adequate arteriography both to determine the size, location, and extent of the fistulae. After adequate clinical and angiographic evaluation, therapy should be conservative enough to afford optimal function and radical enough to prevent death due to hemorrhage. This report includes eight patients who underwent angiographic evaluation and surgical therapy for arteriovenous malformations. Of the six living patients, five are known to have residual arteriovenous shunting.

Postsplenectomy septicemia has been seen to occur not only in children but in adults as well. The reason splenectomy is still practiced when the spleen is traumatized is the fear of development of subcapsular hematoma leading to delayed rupture and massive bleeding. The aim of therapy at present is early recognition of the type of splenic injury by abdominal paracentesis, radioisotope scan, ultrasound, and/or arteriography. Four alternative methods of management are presented and discussed that lead to the preservation of splenic tissue in patients with injured spleen.

Cysts and tumors of the ovary are not common in children. At the large children's hospitals only one to four patients are seen annually with ovarian enlargement.1–11 This paper summarizes our experience with 99 patients with ovarian cysts or tumors seen at the Childrens Hospital of Los Angeles during the years 1951 through 1973.

A retrospective analysis of esophageal atresia occurring in patients who are members of a twin set indicated that twinning does occur more frequently in patients with esophageal atresia. These patients tend to be small for date, but have a similar occurrence of associated anomalies as singletons with esophageal atresia. Applying risk-grouping to the entire series and twins indicated no real difference in survival or additional anomalies by organ system, except more cardiovascular anomalies occurred in twins. Long-term follow-up of five survivors revealed severe growth retardation.

The infant who is born with a posterolateral diaphragmatic hernia who becomes symptomatic at or soon after birth requires urgent care. Surgical reduction of the diaphragmatic hernia must be accomplished quickly. Respiratory and metabolic acidosis must be treated appropriately. The parents should be informed of the gravity of their infant's problem and reassurred by appropriate explanation of the nature of the defect and the therapeutic requirements. If the infant dies, the parents are in need of empathy, reassurance, and adequate explanation so that they do not have lingering doubts regarding the etiology of the anomaly and the adequacy of the therapy. If the infant lives, the medical team can share the feeling of a job well done.

Summary One hundred and two patients with esophageal atresia possessed 237 additional anomalies. Risk grouping of these patients was useful and showed a direct relationship between the increased incidence of anomalies, low birth weight, and nonsurvival. Fifty-seven per cent of the patients with additional anomalies possessed multiple anomalies, most frequently occurring in the gastrointestinal cardiovascular, and musculoskeletal organ systems. Fifty-five per cent of the deaths appeared to result from the coexistent severe anomaly, while the remaining 45% of the deaths appeared to occur as a result of the presence of esophageal atresia. It is our suspicion that the insult producing esophageal atresia with a high incidence of associated anomalies occurs at a more critical time in organogenesis than that event that produces the esophageal atresia alone or with one moderate anomaly.

In infancy and childhood, 75% of teratomas occur in females. The most common site is the sacrococcygeal area. The second most common site is the ovary, followed by lesser numbers in many anatomic areas, exclusive of the extremities. The majority of malignant teratomas are in the sacrococcygeal group. Approximately 1/2 of sacrococcygeal teratomas are composed of benign adult tissue. Twenty percent contain embryonic benign tissue and approximately 30% have overtly malignant tissue. With the exception of central nervous system teratomas, the percentage of malignancy is less in other sites. The malignant teratoma is aggressive in its clinical course. At the present time, approximately 80% of patients with malignant teratomas die as a result of the malignancy. Subsequent to appropriate surgical therapy, aggressive radiation and chemotherapy are indicated for treatment of malignant teratomas.

Although early operation in all patients with a symptomatic duodenal hematoma has been advocated, it is our experience that some of these patients can be treated successfully with nonoperative measures if an accurate diagnosis is made and provided the patient has no significant active bleeding or an associated injury requiring abdominal exploration. Duodenal obstruction due to hematoma can spontaneously resolve while the patient is under non-operative supportive management. The proper course of action should be individualized in these cases. Criteria for nonoperative treatment are: clinical, laboratory, and radiographic evidence that there are no associated injuries; no excessive or continuous blood loss; and subsidence of obstruction within 7 to 10 days.

Summary Experience with 21 gastrointestinal complications in 20 children with acute leukemia is described. These complications appear to be increasing in frequency and are generally late manifestations of the leukemic process. Although the ultimate outcome in these patients is not encouraging, the surgeon and hematologist, working together, must be aware of this background experience and be prepared to individualize therapy for the patient with leukemia and a concomitant "surgical" condition.

Thirty-four smooth muscle tumors of the stomach in childhood and adolescence are reviewed, including two cases reported here for the first time. These are rare tumors, seldom diagnosed correctly preoperatively, and often present with gastrointestinal bleeding and anemia. Histologic differentiation between benign and malignant tumor has often proved difficult. Prognosis is good with wide surgical excision.

This report is a clinical review of 28 children with Crohn's disease hospitalized at the Childrens Hospital of Los Angeles during the past 12 yr. In addition to the usual reasons for surgery, growth failure was a common indication. Surgical resection was performed on 18 patients with a follow-up ranging from 1 to 10 yr, and encouraging early results. The importance of close medical follow-up, with surgical intervention when necessary, is emphasized.

Summary The patient with a heritable disorder of connective tissue who is being considered for a surgical procedure should be evaluated in three general areas: (1risk of general anesthesia, (2) possibility or probability of the surgical procedure's being successful and (3) possibility or probability of the surgical procedure's introducing new problems. Patients with Marfan's Syndrome who do not have severe cardiovascular lesions undergo anesthesia and surgical therapy without undue risk and with good postoperative healing. The patients with Hurler's Syndrome frequently have lesions, such as umbilical and inguinal herniae, which would ordinarily be treated by surgical means. The complications secondary to general anesthesia are common due to frequent respiratory infections and airway obstruction. The patients tend to die at an early age due to pulmonary infections and heart disease. Recurrence of the herniae is common. Tissue friability, including blood vessel weakness, is the major problem in the patient with Ehlers-Danlos' Syndrome. Wound coaptation is difficult to maintain and blood vessel rupture is common. The patient with osteogenesis imperfecta is so disabled by his skeletal weakness that operative intervention is uncommon and inadvisable except when absolutely necessary. The patient with fibrodysplasia ossificans progressiva does not appear to have difficulty with wound healing or with anesthesia. His problem is metabolic with calcification in abnormal soft tissue locations.

Proximal pouch tracheoesophageal fistulas were discovered in 13 of 170 patients (7.7%) who had esophageal atresia and/or tracheoesophageal fistula. Approximately one-third of these were discovered preoperatively, one-third intraoperatively, and the remainder during the first 10 mo postoperatively. We feel that the incidence of this anomaly will be seen to be much higher than previously reported if surgeons specifically look for it.

Summary Coagulation studies and case histories of five infants with surgical problems associated with acquired defects of coagulation are presented. The coagulopathies occurred during episodes of sepsis. Further study is necessary to differentiate hepatic dysfunction and diffuse intravascular coagulation secondary to sepsis.

1. 1. Conjoined twins who possess adequate organs for individual survival should have the opportunity of being separated so that each may lead an individual existence. 2. 2. The time at which this should be performed depends upon the anatomic and physiologic factors present in each set of twins.

Summary To our knowledge, this is the first reported case of esophageal atresia occurring in both members of a pair of twins. These twins appear to be genetically identical because of their general appearance, sex, identical anomaly, and identical serologic genotypes. Genetic predisposition for esophageal atresia in twins would seem to be possible from this study. Heretofore, it has been thought that esophageal atresia in one of identical twins has been a developmental defect rather than a genetically transmitted one. Staged therapy for the premature infant with esophageal atresia increases the chance of survival of these infants.

Summary During the past six years, among 108 hospital admissions for esophageal atresia, 31 (29 per cent) have had one of the four unusual varieties, i.e., Type A, B, D, or E (“H”). In five patients with an initial diagnosis of atresia without fistula (Type A), primary esophageal anastomosis was performed following a planned delay of from four to ten weeks. This was accompanied in three infants by an elongation procedure to lengthen the proximal esophageal segment. This regimen of delayed primary anastomosis, following proximal pouch elongation, has replaced colon interposition in the management of Type A atresia in this center; and is probably the procedure of choice in premature infants with Type C atresia. Recognition of the upper tracheoesophageal fistula has proved difficult in five infants admitted with double fistula (Type D); and anastomotic leak has resulted in a high morbidity and mortality. When lying high in the neck, division of the upper fistula through a cervical incision as a separate procedure may be preferable to complete repair via thoracotomy. Major diagnostic problems are presented by the tracheoesophageal fistula without atresia, Type E (“H”). When this anomaly is seen in newborn infants with respiratory distress, the diagnosis is most readily established by the use of contrast studies of the upper esophagus. Reduced motility in the distal esophagus is a suggestive sign. In older infants and children presenting with intestinal (or abdominal) symptoms, in contrast, endoscopic studies are most helpful. All such fistulae have been divided through cervical incisions.

IN, 1954,,UMIKER 1 ET AL. suggested the name of postinflammatory tumors of the lung to represent lesions characterized by antecedent respiratory infection, mild or no symptoms, radiographic pattern of a single sharply circumscribed mass, and the histologic features of fibroma, xanthoma, or plasma cell tumor. Since that time, pathologists have included in this category lesions variously described as xanthoma, xanthofibroma, sclerosing hemangioma, histiocytoma, xanthogranuloma, and plasma cell tumor of the mediastinum and lung. Over 50% of these tumors are discovered on routine roentgenograms with no antecedent illness or clinical symptoms referrable to the chest. Patients are from 3 to 79 yr of age. Males and females are affected equally. Those reported in children (including four cases of the authors) are reviewed in Table 1. Any lobe of the lungs may be affected, although the right side is involved twice as often as the left. Previous cases have all been solitary; case 2 was multiple. In yet another of our patients, stippled calcifications were seen radiographically and confirmed histologically. All lesions have been removed surgically without evidence of recurrence or metastases. DISCUSSION

Hepatic bilirubin uridine diphosphate glucuronyl transferase (UDPG-T) activity was 0.14 and 0.22 units in two fetuses aged 17 and 22 weeks, respectively, and less than 0.1 unit in 15 fetuses, aged 8--19 weeks compared to 0.68--1.99 units in 21 normal adults. Hepatic uridine diphosphate glucose dehydrogenase (UDPG-D) activity in 14 fetuses, aged 8--18 weeks, ranged from 6.2--15.0 units (mean = 11.3 +/- 0.7) compared to 28.8--49.2 units (mean = 39.6 +/- 2.5) in eight normal adults (P less than 0.001). There was no correlation between UDPG-D activity and gestational age. The hepatic UDPG-D activity was 16.5 units in a 33-day-old full term, female infant, 42.4 and 24.3 units in two 2-year-old infants, respectively, and 24.3 units in a 5.5-year-old child. In three human fetuses, the apparent Km UDPG was 0.54 x 10(-4) M. Thus, both hepatic bilirubin UDPG-T and UDPG-D activity are markedly reduced in the human fetus during the second trimester of gestation. Retarded development of hepatic UDPG-D may extend beyond the first month of life.

During the thirty-three years from 1941 through 1973, forty-two children with ovarian teratomas were seen. The most common complaint was that of abdominal pain and the most common physical finding was palpable lower abdominal mass. Thirty-seven patients had ovarian teratomas with nature tissues only. Of these, two patients have been lost to follow-up and the remainder are alive and well. One patient had teratoma containing mature and immature tissues (embryonic); this patient has remained well since operation. Four patients had malignant teratoma. Of these, two patients are dead due to the tumor and the two are living and well for six and nine years, respectively.

A familial syndrome of short stature associated with facial dysplasia and congenital anomalies was reported by Aarskog in 1970. 1 Subsequently, at least 53 patients in 13 affected families have been reported. 2–5 The family to be described came under investigation following referral of one of the nine siblings to the surgical clinic at Childrens Hospital of Los Angeles for the evaluation of a right inguinal hernia. Recognition of the syndrome and further evaluation of the remaining siblings led to the diagnosis of several genital anomalies requiring surgical correction.

• Fifty-five patients with congenital diaphragmatic hernias (1966 to 1976) were studied and compared to 65 similar patients reported previously (1953 to 1963). The mortality was found to be the same in the two groups despite the fact that in the current series, patients were diagnosed and operated on at a younger age. Initial uncorrected pH value was found to be of prognostic importance. All babies whose initial uncorrected pH was greater than 7.0 survived and almost all those whose pH was less than 7.0 died, with the group in-between having a 50% chance of survival. Earlier operation and correction of acidosis did not substantially improve the chance of survival. Hypoplasia of the lungs and major cardiovascular anomalies contributed to death in a number of patients. Pulmonary hypertension causing a right-to-left shunt was responsible for the deaths of others and, therefore, the use of vasodilator drugs deserves further evaluation. (Arch Surg114:1118-1123, 1979)

• Between January 1977 and December 1979 (three years), 642 appendectomies were performed at the Childrens Hospital of Los Angeles. Two hundred seventy-two of the appendectomies were performed incidentally at the time of another abdominal operative procedure. Using known incidence of appendicitis, approximately 54 cases of acute appendicitis may have been obviated. In three patients, wound infections developed. Twenty-four of the appendices were histologically normal and 30 were abnormal in a clinically insignificant respect. If incidental appendectomy is appropriate in the age group, it is of more benefit to the pediatric patient because of the frequency of appendicitis in patients under 20 years of age. ( Arch Surg 1983;118:714-716)

The true incidence of jaundice complicating hypertrophic pyloric stenosis has not been well documented. As jaundice associated with hypertrophic pyloric stenosis clears rapidly after pyloromyotomy, few clinical reports exist. Two previous retrospective studies showed clinical jaundice occurring in five of 227 patients (2.2%) and three of 157 patients (1.9%) 4 with hypertrophic pyloric stenosis. Between 1968 and 1973 there were 396 patients admitted to the Childrens Hospital of Los Angeles with hypertrophic pyloric stenosis. Thirty-two (8.1%) of these patients were recognized to have clinical jaundice as well as serum bilirubin levels of at least 2.0 mg/100 ml. This figure is undoubtedly low because it was derived from a retrospective chart review and the majority of infants did not have serum bilirubin levels determined.

This paper reports the relationship between serum cholesterol level and hepatoblastoma in nine patients. Four of the nine patients had a high (417–544 mg%) serum cholesterol. All of these patients were less than one year of age and had tumors of the epithelial type. Three of the infants died soon after being diagnosed. Three patients had a moderately elevated serum cholesterol (206–249mg%). One underwent primary hepatic resection. Two had nonresectable tumors that became resectable after chemotherapy. One died secondary to pulmonary metastases. The remaining two patients had a normal serum cholesterol level prior to treatment. Both patients had hepatic resection after chemotherapy and had no evidence of increased cholesterol postoperatively and are alive six years after diagnosis. From these data, it is suggested that the pretreatment level of serum cholesterol may be of prognostic significance in infants and children with hepatoblastoma.

This is a case report of an infant who has a normal chest x-ray early in life and later developed a left posterolateral diaphragmatic hernia resulting in life-threatening bowel infarction.

Hydrops of the gallbladder is recognized as a majorcomponent of the abdominal crisis occurring in children with Kawasaki syndrome. Sixteen patients with hydrops of the gallbladder secondary to Kawasaki syndrome have been diagnosed and treated at the Childrens Hospital of Los Angeles. One patient was treated by cholecystectomy and 15 nonoperatively without untoward sequelae. Non-operative management with serial ultrasonic evaluation and close clinical monitoring is a safe method of treatment for this entity. Pathologic and clinical data are presented and discussed. Review of diagnosis and treatment of 41 reported cases of hydrops of the gallbladder in Kawasaki syndrome from the English language literature is also presented.

Technological limitations have impeded accurate energy expenditure assessment in critically ill infants and young children. Instead, a predicted energy expenditure (PEE) is derived based on weight, heat loss, activity, growth requirements, and degree of stress. This study compared actual measured energy expenditure (MEE) with conventional predicted values in 20 critically ill infants and children using a validated metabolic cart designed for use in this age group. All patients were studied either within 4 days of major surgery or during an acute disease process necessitating intensive care. All were severely stressed clinically and were studied while mechanically ventilated in a temperature-controlled environment. The study interval ranged from 1 to 12 hr and averaged 4 hr after a stabilization period of 30 min. The mean MEE was significantly lower than the mean PEE (52.2 +/- 16 kcal/kg/day vs 101.8 +/- 17 kcal/kg/day, P less than 0.001) with a mean MEE/PEE of 52.6 +/- 17% (range 26 to 92%). In a subgroup of 7 paralyzed patients, the mean MEE was significantly lower than in the 13 nonparalyzed patients when compared with PEE and predicted basal metabolic rate (PBMR). The coefficient of variance, conventionally recognized to be approximately 15% for PEE, averaged 6.35% for MEE in this study. These data indicate that if PEE is used as the sole guide for caloric repletion in the stressed infant or child, these patients will be substantially overfed.

Recent experience with a twin who had esophageal atresia and tracheoesophageal fistula revealed complex anatomy not suspected before operation. The experience in patients with esophageal atresia and tracheoesophageal fistula who were twins was reviewed at the Children's Hospital of Los Angeles. Of 245 patients seen in the past 23 years, 16 were twins (only two of whom were siblings). Six of the 16 patients (38%) had other than the most frequent anatomy, i.e., proximal atresia of the esophagus and distal fistula. Two of these patients had extremely complex anatomy. Twenty-five per cent of the patients had a right aortic arch compared with 5% of all patients with tracheoesophageal fistula. The surgeon who encounters a twin with esophageal atresia or tracheoesophageal fistula is appropriately cautioned that significant anatomic complexity may be encountered.

Sixty-two patients with Hirschsprung's disease (operated on according to Duhamel, Swenson, or Soave) were evaluated for quality of defecation and studied manometrically. Follow-up was 30 years. Stools were normal in 30% to 50% of patients, while the rest had either constipation, loose, or increased frequency of stools per day. Continence was complete in 30% to 60%. Enterocolitis was seen in 12 patients preoperatively and in 20 patients postoperatively, regardless of type of operative procedure. This complication improved with age. To correct some of these problems, 18 had post pull-through internal sphincter myectomy with 50% improvement. Manometrically, 50% to 70% experienced a sensation of fullness and an urge to defecate following rectal balloon inflation. The resting external sphincter pressure was high in the majority of patients and further increased following rectal balloon inflation. The internal sphincter pressure showed a normal anorectal reflex in only 10% of patients postoperatively and did not seem to be related to clinical fecal continence. We concluded from the study that an appreciable number of patients in this select group with Hirschsprung's disease suffered from a variety of stooling disorders postoperatively. The majority of these complications were managed medically and/or socially but some were quite incapacitating.

We reviewed all cases of omphalitis seen at Childrens Hospital of Los Angeles from 1961 to 1981. One hundred and forty patients were seen and, of these, eight had necrotizing fasciitis. All patients with necrotizing fasciitis acquired omphalitis at home, making the incidence of necrotizing fasciitis over 10% in patients with community acquired omphalitis. Most of the infants appeared relatively well on admission with no fever but had a marked leukocytosis. The disease rapidly spread to involve most of the abdominal wall over a period of several hours to days. Seven of the eight patients died (87.5%). Five patients were operated upon but despite extensive resection of involved tissue, four died within 24 hours of surgery. A polymicrobial flora of both gram positive and gram negative bacteria was recovered in all patients cultured. The high incidence of necrotizing fasciitis following omphalitis in the newborn with its attendant morbidity and mortality mandates close observation of these infants with early surgical intervention if there is any question of the diagnosis.

The authors report their experience with early postoperative feedings in a variety of pediatric surgical patients utilizing a needle catheter jejunostomy. A small caliber catheter, similar to that used for antecubital central venous cannulation, is inserted in the antimesenteric border of the jejunum providing a subserosal tunnel. A purse-string suture is placed around the catheter and then it is secured to the abdominal wall, and the catheter is brought out through the abdominal wall via a needle puncture wound. Our experience with 27 insertions in 25 patients ranging in age from 1 day to 17 yr, has demonstrated the ease of placement and feasibility of immediate postoperative feeding. Patients were generally started on a dilute elemental diet through the jejunostomy within 12 hr of the operative procedure. Patients were administered between one and 3.5 g of protein kg/day and between 45 and 100 cal/kg/day depending on age and weight. Duration of treatment with enteral nutrition ranged from 10 to 150 days. There was no catheter-related complications utilizing the technique described. Technical details of catheter placement and protocol for administration of early postoperative feedings are discussed. The ability to provide nutritional support via the gut has obviated the need for total parenteral nutrition in the majority of these patients.