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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.

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.

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.

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 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.

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.

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.

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 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.

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.

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.

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.

The authors describe a 6 1/2-month-old boy with a large hemangioma involving the left buttock associated with a severe consumptive coagulopathy. Cosmetic improvement and resolution of the coagulation defect followed occlusion of the nutrient vessels to the hemangioma with polyvinyl alcohol. This is the first report of successful treatment of this condition with therapeutic embolization.

• 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)

From 1941 through 1983, a total of 66 patients with sacrococcygeal teratoma were seen, representing 41 percent of the total of 162 patients with teratomas from all anatomic sites seen over this period. Forty-six (70 percent) of the patients with sacrococcygeal teratomas were female and 34 (52 percent) were neonates. Younger patients had a significantly better prognosis. Approximately half (48 percent) of the patients had benign tumors. Of the other 34 patients, 19 (29 percent) had frank malignancy, being either a purely malignant tumor or a tumor with malignant mixed with benign elements, and 15 (23 percent) patients had tumors containing embryonic but no malignant components. Patients with tumors containing mature adult or embryonic tissues have a good prognosis. However, malignant sacrococcygeal teratoma portends a bleak prognosis irrespective of therapy (in this series only one patient survived among 19 patients with an average survival of 16 months after diagnosis).

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.

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.

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