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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.

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.

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.

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.

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 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.

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

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.

• 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)

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.

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.

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.

Bleeding from esophageal varices may be a serious or lethal complication of portal hypertension in children. The standard therapy over the past 30 years has been to create a portosystemic shunt. In children, physiologic complications leading to high rates of perioperative morbidity and early and late thrombosis with recurrence and encephalopathy have been common. Over a 42 month period, we treated six patients, aged 5 to 18 years, with endoscopic injection of 3 percent sodium tetradecyl sulfate into the varix. Five patients required only injection, whereas one underwent direct oversewing of gastric varices followed by endoscopic sclerosis of the esophageal varices which remained. A total of 38 endoscopic procedures were performed. There has been complete cessation of bleeding in two patients, minimal subsequent bleeding in two others, and anemia requiring transfusion in the last two treated. These last two patients, although still requiring occasional transfusions, have been free from hypotensive or exsanguinating hemorrhage since beginning sclerotherapy. No deaths or serious complications were encountered in this series. Follow-up has ranged from 18 to 42 months (mean 26 months). The results of this trial suggest that repeated endoscopic sclerotherapy of varices, combined with operative oversewing of gastric varices when necessary, offered a viable alternative therapy for patients with esophageal varices.

• The technetium Tc 99m sulfur colloid liver-spleen scan is a valuable aid in diagnosis and treatment of patients with splenic injury. After reviewing the charts of 47 patients who were ill as a result of splenic trauma, we came to the following conclusions: (1) the scan identified the injury, accurately mapped its extent, and indicated the presence or absence of associated liver injuries; (2) the scans were useful in following the extent and rate of healing of the splenic injury; (3) the scan is an indirect measurement of return of splenic function; (4) the procedure can be performed in a reasonable time frame with no serious morbidity; and (5) the indications, contraindications, and timing of scans are now reasonably well established. (Arch Surg1982;117:1147-1150)

Improved survival rates in acute childhood leukemia have been associated with an increasing number of complications in the ileocecal region. An experience is reported with 11 children who developed inflammation, perforation, and/or necrosis of the ileum, appendix, and/or cecum (leukemic ileocecal syndrome). The clinicopathologic correlation, roentgenographic findings, and results of treatment are presented. Once the ileocecal complication developed, the prognosis was grave. Surgical intervention was not helpful, regardless of whether the bone marrow was in remission or relapse. The importance of recognizing this entity is emphasized because of its clinical similarity to acute appendicitis.

A segment of colon is considered best for operative substitution of a damaged esophagus because it resists peptic ulceration, it functions well during swallowing, and its blood supply remains intact after transposition. Before a child is considered a candidate for the operation, conservative treatment should be done (dilatation for esophageal stricture, portal-systemic venous anastomosis for varices). The operation was performed successfully on six of nine infants with congenital esophageal atresia, four children with strictures from ingested lye, two with persistent esophagitis, and three with bleeding varices. Failure of the operative procedure was the cause of death in one child; in the others the cause was undetermined or unrelated. The technical difficulties of the transposition are encountered in the infant, and these can usually be obviated by delaying the operation until the child is 1 to 2 years old.

Thirty-four patients with gallbladder disease, 6 weeks to 17 years of age, were seen in 20 years. There were 20 girls and 14 boys. Fifteen had associated hemolytic disorders and no discernible etiologic factor could be presumed in 14. Abdominal pain was the most common presenting symptom. Jaundice was present in 23 patients. The diagnosis was made preoperatively in 21 patients, all with the aid of radiographic findings. Laboratory determinations were of limited value. Twenty-eight patients had choleliths while six, including one with associated choledochal cyst, had choledocholiths. Thirty patients were operated upon; 19 had cholecystectomy alone and five had common bile duct exploration. Operative cholangiography was helpful in avoiding unnecessary common bile duct exploration. Six patients had cholecystectomy for acalculous cholecystitis. Three patients with an associated choledochal cyst had chronic cholecystitis. None had acute pancreatitis.

• A review of 96 patients with the established diagnosis of childhood dermatomyositis revealed that 15 patients required surgical therapy in addition to diagnostic biopsy. Four of the patients had perforation of the esophagointestinal tract, and 11 required surgical therapy for abscesses, calcific deposits, or treatment of pneumothorax. The life-threatening lesions were perforations of the esophagus and intestine. The best surgical therapy is closure, resection, and adequate drainage. Prognosis depends on treatment of the underlying medical disease. (Arch Surg1988;123:1117-1120)

Congenital dilatation of the common bile duct or choledochal cyst is a rare condition which, until recently, was usually not recognized prior to operation. Although the number of reported cases is greater than 600, the group reported in this study may be the largest from a single institution in the United States. Ten patients with this anomaly were seen at the Childrens Hospital of Los Angeles during the two decades (1948-1968) among approximately 135,000 admissions. Clinical Series A summary of the clinical course of these ten children is found in the Table. Nine of the ten patients were females. The racial background of seven was white; two, oriental; and one, Negro. Five children developed the initial symptom during the first six months of life. In contrast, two were over 10 years of age at the time of the first recognition of their disease process. Six of the ten patients had