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To determine the extent of progress in the treatment of sacrococcygeal teratomas (SCTs), we evaluated the experience with 73 patients over 40 years. The medical records of the children were reviewed for demographics, histology, clinical course, and outcome. Therapy differed depending on the type of SCT, histology, and decade of diagnosis. The female:male ratio was 4.2:1 and did not vary significantly with the histology of the tumor. Fifty-seven patients presented with benign disease. There were five recurrences in this group, only one of which did not have an initial coccygectomy. One tumor, originally thought to be benign with immature elements, had a local recurrence at 7 months, 10 months, and 16 months after the original operation and was discovered to have embryonal carcinoma with nodal involvement. This child was treated with chemotherapy and is disease-free at 7 years. Morbidity in the benign group included 3 postoperative wound infections. Three infants died, one before operation with beta-hemolytic Streptococcus sepsis. Two neonates died in the early postoperative period, one on day 1 with a ruptured subcapsular hematoma of the liver, and the second on day 2 with disseminated intravascular coagulation/sepsis. Benign SCT occurs at a younger age than malignant tumors. The average age of presentation of benign tumors is 20 days versus 468 days in children with malignant disease. The technique of wide resection of benign lesions with coccygectomy is helpful in preventing recurrence and has changed little over the last four decades. Malignant SCT occurred in 16 children (22%).(ABSTRACT TRUNCATED AT 250 WORDS)

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Optimal management of children with perforated appendicitis remains a controversial clinical problem. Until very recently, the criteria for hospital discharge on our surgical service included the absence of fever and leukocytosis for a period of 48 hours following completion of antimicrobial therapy, uncomplicated wound healing, a normal rectal examination, and unimpaired gastrointestinal function. With the introduction of cost-containment programs, the necessity for the period of inpatient observation following cessation of antibiotics was questioned. The records of 87 consecutive children with perforated appendicitis were analyzed prospectively in order to determine if our discharge policies were medically sound and cost-effective. Seventy-five patients (86%) recovered uneventfully while 12 children required prolonged hospitalization for management of various postoperative complications. The 12 patients who developed complications were all identifiable early in the postoperative period because of persistent fever, leukocytosis, and elevated band counts. Of the 75 children who recovered uneventfully, all met standard discharge criteria on the final day of antibiotic therapy with the exception of completing the mandatory 48-hour period of inpatient observation. These children were maintained in the hospital a total of 142 additional days following discontinuation of antibiotics. The average cost per patient day for children with perforated appendicitis during the study period was $506.32, which represented unnecessary hospital charges of $71,897.44. It was concluded that inpatient observation following cessation of antibiotic therapy in children who experience an uneventful recovery from perforated appendicitis is neither necessary nor cost-effective.(ABSTRACT TRUNCATED AT 250 WORDS)

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A long-term evaluation of 43 children with extrahepatic portal hypertension indicates a high success rate and excellent subsequent health when a splenorenal or mesocaval shunt can be performed. On the other hand, makeshift shunts inevitably fail. Direct operations are considerably less successful than standard shunts. Esophagogastric resections with interpositions result in long-term freedom from bleeding in only about half the cases, and portoazygous disconnection procedures have been uniformly disappointing. Sclerotherapy in a relatively recent experience has been quite successful, but long-term results are presently unavailable. The condition carries a significant mortality rate. Complications from failed operations, division of the vena cava, and multiple transfusions are numerous. The general health of long-term survivors is excellent in those with successful operations, and is surprisingly good for patients whose operations have been unsuccessful and for those who have had no operations.

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In the period January 1982-July 1984, 1512 cases of suspected child abuse were evaluated in the emergency department of a major children's hospital, of which 256 (17%) required hospitalization. Failure-to-thrive with caloric malnutrition was present in 66 (26%), burns in 56 (22%), central nervous system injury in 53 (22%), soft tissue trauma in 21 (8%), ingestions in 20 (8%), skeletal injury in 15 (6%), neglect of an underlying disease in 10 (4%), sexual abuse in nine (3%), near-drowning in four (1%), and abdominal trauma in two (1%). Two-thirds of the children required surgical care and one-third of the surgical group needed operations. The majority of the patients were toddlers between 18 and 36 months of age. A long hospitalization occurred with a mean stay of 9.3 days. Mortality was 7% for the entire group, but children with central nervous system injury had a much higher mortality (26%) and morbidity (21%).

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During the past five years, sclerotherapy has been used at our institution in 13 children for the management of recurrent major variceal bleeding. The varices were secondary to extrahepatic portal hypertension in seven patients and to intrahepatic portal hypertension in the remaining six. Sclerotherapy was performed under direct vision using either rigid or flexible endoscopic equipment, and the sclerosing agents were injected directly into the varices. The average age at initiation of sclerotherapy was 9 years (range: 1 to 19 years). The follow-up has ranged from 2 to 4 1/2 years with a mean of 3 1/2 years. Complete obliteration of all varices was obtained in eight of these patients. Two children have minimal residual varices, in one of whom 17 sclerotherapy procedures have been performed to date. One additional patient had a severe episode of bleeding during esophagoscopy, and transesophageal ligation of varices was required for control. Two patients have died following initiation of sclerotherapy. In neither case was the death the result of bleeding esophageal varices or a complication of endosclerosis. Bleeding from varices was the major clinical problem in all of these children, and this problem has been largely corrected by the sclerotherapy program. With one exception, there have been no episodes of variceal bleeding requiring transfusion in these patients following initiation of this therapy. One child developed an esophageal ulcer postinjection, but none have developed esophageal strictures. One patient developed an allergic reaction to the sclerosant that was treated during subsequent injections with prior administration of an antihistamine (diaphenhydramine chloride) and steroids.(ABSTRACT TRUNCATED AT 250 WORDS)

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Fifty-seven of 101 Nissen fundoplications during the 4-year period, July 1979 to July 1983, were performed on neurologically impaired children. Mean age at the time of surgery was 5.9 years (range 1 month to 22 years). Indications for operation included: persistent vomiting, 57 patients (100%); failure to thrive, 49 patients (86%); repeated episodes of pneumonia, 49 patients (86%); esophagitis, 18 patients (32%); hiatal hernia, 14 patients (25%); episodes of apnea, 10 patients (18%); and esophageal stricture, six patients (10%). Forty-six of the 57 patients had previously failed a standard trial of nonsurgical management. Gastroesophageal reflux was documented by barium esophagograms in 51/56 patients (91%), chalasia scans in 28/32 patients (88%), esophagitis or stricture at endoscopy in 21/23 patients (91%), and acid reflux on pH monitoring in 13/16 patients (80%). Operative management included gastrostomy in 55 of the 57 patients and this was permanent in 50. Gastrostomies had previously been performed in nine patients but had failed to provide a reliable method of enteral feeding because of chronic reflux and aspiration. The surgical complication rate was 12%. Intraoperative esophageal perforation occurred in two patients, splenic tear in one, hepatic vein laceration in one, and a tight wrap in one. After surgery, bowel obstruction from adhesions developed in one patient and a midgut volvulus in another. Five of the children have died, none from causes related to the surgical procedure. Clinical and radiologic follow-up evaluations of all survivors have been done, with a mean follow-up of 3 years. In four patients the repair was felt to be inadequate. One patient had an esophageal stricture and three had recurring episodes of pneumonia. Three children showed radiologic evidence of persistent reflux, but only two were symptomatic. Two patients required a second antireflux procedure for reflux and are now free of symptoms. Nissen fundoplication appears to be a safe and beneficial procedure in neurological impaired children. Long-term follow-up evaluation of these patients showed satisfactory growth as well as a significant decrease in pulmonary disease associated with aspiration.

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In the management of children with acute lymphoblastic leukemia, L-asparaginase has become established as an effective drug in the usual multi-agent therapy; and the significance of pancreatitis as a complication of this drug is well recognized. Less well appreciated, however, is the progression of such pancreatitis in some patients to pseudocyst formation and the possible necessity for surgical management. Two adolescent girls who developed pancreatic pseudocysts while being treated with L-asparaginase are described in this report. Both were being treated for acute lymphoblastic leukemia for periods of 18 and 4 months, respectively, prior to the onset of pancreatitis. Both were in remission of their leukemic disease when typical clinical and laboratory manifestations of acute pancreatitis developed. In one girl, a pancreatic pseudocyst became apparent 2 weeks following the diagnosis of acute pancreatitis and in the other girl, this complication developed over a period of 8 weeks. The usual nonsurgical management of pancreatitis over protracted periods of time was ineffective in the treatment of the pseudocysts. Surgical drainage (internal in one and external in the other) was successful in both in eradicating the pseudocyst, and in neither did further evidence of pancreatic disease subsequently occur. In both resumption of chemotherapy, omitting L-asparaginase, was well tolerated. One has been in remission of leukemia and in good health for a 3-year period of follow-up observation, while the other subsequently had a relapse of leukemia and died 18 months following the onset of pancreatitis.

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Between 1973 and 1983, eight children who had undergone successful multimodal management of malignant tumors developed secondary thyroid neoplasms. The primary tumors were acute lymphocytic leukemia in three, Wilms' tumor in two, and Hodgkin's disease, rhabdomyosarcoma, and ganglioneuroblastoma in one each. During this period, 174 long-term survivors with these five diagnoses were enrolled in our tumor registry, yielding a 4.6% incidence of secondary thyroid neoplasms. All eight patients received both radiation and chemotherapy. The mean radiation dose was 2,700 r with a calculated thyroid dose of 2,140 r (range, 5 to 4,200 r). Age of diagnosis of the primary tumors ranged from 1 to 8 2/12 years (mean, 5 years), and the latent period between treatment and development of the thyroid lesions averaged 6 1/2 years. Thyroid neoplasms presented at an average age of 11 4/12 years. Five patients developed solitary adenomas, one presented with multiple adenomas, and two had follicular carcinoma with regional lymph node metastases. Although thyroid neoplasms are rare in childhood, clinically apparent thyroid tumors have been observed in up to 2.5% of children following radiation exposure (mean follow-up, 24 years). The reported latent period before the development of thyroid neoplasms in irradiated patients is at least 10 years, with the peak incidence occurring 20 to 25 years after exposure. This study documents a 4.6% incidence of subsequent thyroid neoplasms in pediatric cancer patients within a relatively short follow-up period (mean, 11 years). These thyroid tumors occurred at an earlier age (mean, 11.5 years) and with a shorter latent period (mean, 6.5 years) than would be predicted from previous studies.(ABSTRACT TRUNCATED AT 250 WORDS)

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To determine if splenic implants in the body regain vascularization and grow, and to determine the effects of any interaction between implant and remnant on growth of these spleen fragments, we conducted experiments on rats. 1) Spleen fragments were implanted in the omentum (20 cases), in the subcutaneous space (20 cases), or in the liver (10 cases), and the weight of splenic portions and its histological findings were observed 12 and 24 weeks after operation. The best splenic growth was observed in the group of implant in the omentum. 2) Total, 4/5, 2/3, 1/3 and 1/5 spleen were implanted in the omentum and the weight of implants were measured 6 weeks after the operation. The rate of growth of implanted spleen was the highest in the group of 1/5 and was the lowest in the group of whole spleen. 3) Two groups of rats underwent 1/3 splenectomy, and the 1/3 spleen portions was implanted in the omentum. One group had the remaining 2/3 spleen removed, the other group had the remaining 2/3 spleen left in place. In group at 6, 12 and 24 weeks postoperatively the splenic portions was weighed. The implants regenerate with time in rats of both groups. The rate of weight-gain was, however, slow in the group with 2/3 spleen and there was no regeneration on the remaining 2/3 spleen.

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In the measurement of the length of preserved intestine after massive resection of the intestine, measurement of the length of intestine as it is at the time of operation may produce a large error when compared to the length of the intestine at normal times. Therefore, attention was paid to the number of arteriae rectae of the intestinal artery and a comparison was made between such number and the length of the intestine at normal times in rats and man. As a result, the conclusion was drawn that the measurement of arteriae rectae of the intestinal artery is useful for the estimation of preserved intestine at normal times.

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Partial splenectomy is often preferred to total splenectomy as a method to prevent serious post-operative sepsis. However, most of the previous researches on spleen function following partial splenectomy have failed to provide accurate data on the minimum increase of residual spleen in weight necessary for protection against postoperative sepsis. This is not only to the lack of appropriate model but also to the negligence of the effects of spleen regeneration after partial splenectomy. To produce an accurate model for partial splenectomies, and to know how the spleen regenerates following partial resection, about 500 rats underwent partial splenectomies. From these experiments, the following results were obtained. These partial splenectomies based on length of the spleen were satisfactory models as partial splenectomy. Absolute volume of regenerated spleen was finally almost the same in every model. However, the less the remnant spleen was the longer period was necessary until the spleen regeneration completed.

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To improve the therapeutic results of high undescended testis, we conducted experiments with division of spermatic vessels and replacement of the testis into the scrotum after preparation of undescended testis in rats. The results of this experiment show that 1) testis in which the vessels were divided at the inguinal level showed distinct degeneration from infarction one month postoperatively, 2) testis in which the vessels were divided at the abdominal level appeared normal, and 3) in the rats with preparation of undescended testis, bringing down the testis at some interval after division produces better results as to the recovery of the testis.

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A prospective, randomized, double-blind clinical trial was undertaken comparing gentamicin, ampicillin, and clindamycin (GAC) to gentamicin, ampicillin, and placebo (GAP) in children with complicated appendicitis. Of the 64 patients enrolled in this study, 33 were assigned to the GAC group and 31 received GAP. A single GAC patient (3%) was considered a therapeutic failure in comparison to seven children (23%) in the GAP group (P less than 0.05). Duration of fever was significantly prolonged in the GAP patients (4.7 +/- .8 days versus 2.9 +/- .5 days) when compared to the clindamycin treated children (P less than 0.05). Duration of leukocytosis was 3.2 +/- .4 days for GAC patients and 4.9 +/- .9 days for those on the GAP protocol (P = 0.08). On the basis of this experience the routine use of gentamicin, ampicillin, and clindamycin is recommended for all children with complicated appendicitis.

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In an experimental study, catheterization of the abdominal aorta with small umbilical artery catheters consistently produced significant intimal injury. The duration of catheterization varied from 1 hr to 7 days, and recovery periods following catheter removal varied from 1-150 days. Endothelial disruption was observed in all animals sacrificed within 24 hr of catheter removal. Healing progressed rapidly when the duration of catheterization was short, but was protracted with longer periods of catheterization. Thrombi were frequently found adhering to the exposed subendothelial surface. Routine light, scanning, and transmission electron microscopy demonstrated consistent cellular changes, which depended upon the duration of catheterization and the length of recovery periods. A consistent relationship between the degree of intimal injury and the duration of catheterization was observed.

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The Blue Rubber Bleb Nevus syndrome is a rare disease characterized by a distinctive type of hemangioma which involves the skin and the gastrointestinal tract. In the latter location, these lesions are often responsible for chronic blood loss and secondary anemia, and in rare situations may act as a leading point for an intussusception. The diagnosis of intussusception in children older than 3 or 4 yr is frequently difficult and delayed. In a child with typical skin lesions of the Blue Rubber Bleb Nevus syndrome, an acute illness with manifestations of intestinal obstruction should indicate the possibility of an associated intussusception.

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Extrahepatic portal hypertension in children continues to be a poorly understood entity in many respects. The etiology in most patients is undetermined. The natural history of the condition is not clearly appreciated, particularly with respect to the eventual fate of esophageal varices and the risk of variceal bleeding. Considerable difference of opinion exists in regard to the results of surgical intervention to control or prevent bleeding as well as in regard to the long term affects of such operations. Forty-one children have been followed at this hospital over the past 30 years. A recent and thorough follow-up evaluation has been completed. The purpose of this paper is to analyze this group of patients in regard to these poorly understood areas. Particular attention is paid to the natural history of the condition, the results of therapy, the mortality, and the overall current status of these patients.

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Increased acetylthiocholinesterase (AchE) reactivity in the rectal lamina propria and lamina muscularis mucosae was used to diagnose Hirschsprung's disease. We processed 131 specimens with the AchE reaction; 43 were suction biopsy specimens and the rest were full-thickness specimens. Of the 68 specimens in which neurocytes were present, none demonstrated a diffuse increase in the number of nerve fibers. However, 15 showed focal increases in the numbers of fibers that were not large enough to be regarded as indicative of Hirschsprung's disease. All patients with a diffuse increase in nerve fibers, regardless of the type of biopsy, were shown to have Hirschsprung's disease. The AchE staining reaction did, however, produce a 29% rate of false-negative reactions (16 of 56 specimens) in patients with Hirschsprung's disease. These data demonstrate that an abnormal pattern of AchE reaction is diagnostic of aganglionic megacolon, whereas a normal pattern does not exclude the disease.

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During the past 6 years, 68 consecutive children suffering from splenic trauma have been treated according to a selective management plan. Twenty-two patients (32%) underwent splenectomy, parenchymal repair was performed on 16 occasions (24%), and nonoperative treatment was employed in 30 children (44%). Overall results have been good in all three groups. Two children (3%) died as a result of their injuries, and 10 complications were recorded. Both deaths and 7 of the 10 complications occurred in the patients undergoing splenectomy. By utilization of a basic management plan that favors nonoperative treatment over splenic repair and repair over splenectomy, splenic salvage has been successfully accomplished in 86% of the 49 patients treated since 1977.

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The ability of external imaging to demonstrate intestinal infarction in neonatal necrotizing enterocolitis (NEC) was prospectively evaluated. The radiopharmaceutical technetium--99m diphosphonate was injected intravenously and the patients subsequently underwent abdominal scanning. Clinical patient care and interpretation of the images were entirely independent throughout the study. Of 33 studies, 7 were positive, 4 were suspicious, and 22 were negative. One false positive study detected ischemia without transmural infarction. The second false positive scan occurred postoperatively and was due to misinterpretation of the hyperactivity along the surgical incision. None of the suspicious cases had damaged bowel. The two false negative studies clearly failed to demonstrate frank intestinal necrosis. The presence of very small areas of infarction, errors in technical settings, subjective interpretation of scans and delayed clearance of the radionuclide in a critically ill neonate may all limit the accuracy of external abdominal scanning. However, in spite of an error rate of 12%, it is likely that this technique will enhance the present clinical, laboratory, and radiologic parameters of patient management in NEC.

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