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Background Patients with esophageal atresia with or without tracheoesophageal fistula (EA/TEF) historically have had a high risk of neonatal mortality but the majority of patients are now expected to live into adulthood. However, the long-term burden of care among recent EA/TEF survivors has not been documented. Methods A single-institution retrospective review of newborns with EA/TEF treated from 2001-2005 was conducted, including initial and total hospitalization length of stay, and number of clinic visits and procedures requiring general anesthesia in the first three years of life. Exposure to and number of radiological studies involving ionizing radiation (IR) were recorded. Results Seventy-one of 78 (91%) patients survived to discharge and 69 were included for analysis. Mean length of initial hospital stay was 51.3 (range 9-390) days. By age 3 years, patients required 4.5 (mean, range 1-23) procedures performed under general anesthesia, attended 13.5 (mean, range 3-40) outpatient visits and were exposed to 17.4 mSv (mean, range 3.0-59.9) of IR from 40 (mean, range 5-165) radiological studies. Conclusion Patients with EA/TEF need complex and frequent hospital-based care from infancy to early childhood. Opportunities to critically review clinical services and imaging needs should be explored to improve the experience of patients and their families.

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 β-hemolytic Streptococcus sepsis. Two neonates died in the earlypostoperative 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%). Seven of these children are long-term survivors with a mean follow-up of 4.5 years. The average survival was 9.3 months in the nine children who died of disease (range, 1 to 28 months). Long-term survival in the last two decades was achieved more often and may be attributed to modern chemotherapy.

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. These data indicate that multimodal management of pediatric cancer patients utilizing both radiation and chemotherapy increases the risk for the development of secondary thyroid neoplasms. Furthermore, it strongly suggests that the occurrence of these neoplasms will proportionately increase with time, making careful evaluation of the thyroid gland an important component of follow-up care in survivors of childhood malignancies.

In an experimental study, catheterization of the abdominal aorta with small umbilical artery catheters consistently produced significant intimal injury. The duration of chatheterization 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 dependend 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.

Partial splenectomy, with resection of the lower one-fourth to one-third spleen, was performed in 10 children with Hodgkin's disease as part of an otherwise standard staging laparotomy. The technique proved to be safe and practical, with no postoperative complications. No evidence to date indicates that splenic involvement was missed, and all the children have thus far continued to do well, with no deaths from either underlying disease or postsplenectomy sepsis. The procedure is undergoing a prospective clinical trial in an effort to determine whether or not it is comparable from a diagnostic point of view to total splenectomy. Available data indicate that the risk of missing intraabdominal disease in a staging laparotomy with a partial splenectomy is quite low. Such a procedure should eliminate the substantial risk of postsplenectomy sepsis in such children.

Twenty-one of 25 episodes of ileocolic intussusception were successfully reduced by the barium enema hydrostatic technique supplemented by glucagon. No apparent serious complications occurred from the use of this drug. The comfort of the children and the ease of filling the colon and small bowel seemed enhanced by the glucagon.

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 identificable 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. These patients can be safely discharged when their antibiotics are discontinued. Patients who develop postoperative complications are easily identifiable early in the postoperative period because of persistent fever, leukocytosis, and bandemia.

Small focal areas of intestinal infarction were produced experimentally in mice by two techniques: (1) intussusception and (2) suture strangulation of a loop of jejunum. The radionuclide technetium 99m diphosphonate ( 99m Tc-diphosphonate) was subsequently administered intravenously, and abdominal scans were obtained. The scans demonstrated focal infarcted lesions accurately, and every positive scan was associated with an area of infarction, as demonstrated by pathologic examination. However, negative scans did not definitely exclude an area of infarction: in the intussuception study there were five false negative scans in 25 animals, and in the loop ligation study there were two false negative scans in 28 animals. The technique may have clinical application in such intestinal diseases as necrotizing enterocolitis in infancy in which early diagnosis of small areas of necrosis is currently difficult by clinical and radiologic methods.

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.

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.

Transfer of a critically ill child to a pediatric center is indicated when patient needs have exceeded local expertise or facilities. It is illogical to impose on the referring hospital the added burden of transporting such a patient. The receiving hospital is better equipped to extend its specialized services by assuming responsibility for interim management and transfer of the patient. An aeromedical transport system capable of quick response was built around National Guard helicopters. In 21 cases, doctors, nurses, and equipment were flown to children in community hospitals, and urgent treatment was begun in half the time formerly possible. Only after the patients were stabilized were they returned safely under continuing appropriate management. The helicopter is a versatile and speedy tool to deliver expertise and equipment when and where needed. Timely arrival of specialty services can be expected to improve patient care and salvage.

Summary Six malignant ovarian tumors (8%) were found during 77 operations for cysts and tumors of the ovary in children. Two of the six patients already had widespread abdominal metastases. The other four with localized disease were treated by unilateral salpingoovariectomy and are alive and free of disease. Ovarian cancer in children is rare, and apparently is found either very early or very late in its course. This experience suggests that the initial operation should be restricted to unilateral salpingoovariectomy. Abdominal hysterectomy and contralateral salpingoovariectomy can be completed soon thereafter if the surgical specimen shows a malignant tumor which has spread beyond the confines of the ovary.

Summary The technic described demonstrates that the split-thickness grafts of a practical size can be obtained readily and efficiently from fetuses at postmortem. The advantages of such grafts in extensively burned children are briefly discussed.

The technique of staged reduction herniorrhaphies, without the ancillary use of pneumoperitoneum or of synthetic prosthetic sheeting, is a practical method for the management of huge ventral hernias. In 12 patients treated in this fashion, the success rate was satisfactorily high. No deaths occurred, and complications were few and relatively minor.

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.

A standard type of ileocolic intussusception was produced in 69 puppies and the effect of glucagon on the hydrostatic reduction of the intussusceptions evaluated in a prospective, double-blind study. The reductions were attempted at intervals varying from 18 to 60 hr following the production of the intussusceptions. The overall reduction rate was 70% and there was no statistical difference in this rate between the animals receiving glucagon and those receiving placebos. None of the gangrenous intussusceptions were reducible with the hydrostatic pressure technique, irrespective of whether glucagon was used or not. A further evaluation was made of those animals in whom successful reductions were accomplished by the hydrostatic pressure method. Glucagon did result in significantly easier reductions, and an earlier return of normal vascular supply as measured by color and by arterial pulsations as compared to the control group.

A canine model simulating Meckel's diverticulum was used to evaluate the effects of pentagastrin, glucagon, and a combination of the two hormones on the gastrointestinal localization of 99m Tc. Pentagastrin produced accelerated uptake of the radionuclide in both the stomach and the ectopic gastric mucosa but resulted in early visualization of the duodenum and a "washout" effect on the radioisotope within the Meckel's diverticulum. Glucagon provided prolonged retention of the radionuclide in the stomach and ectopic mucosa and prevented duodenal visualization. A combination of both hormones produced increased activity in the stomach and experimental Meckel's and decreased duodenal filling. This enhanced target to background contrast and provided improved visualization fo the ectopic gastric mucosa.

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 intiation 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. Nine of 13 children have shown evidence of distal esophagitis that has been well controlled with medical therapy. Esophageal sclerotherapy is a safe and effective method of management for control of bleeding esophageal varices in children. The majority of patients have achieved effective control of their recurrent hemorrhage, and no significant long-term sequelae have been recognized. This method of management has the obvious advantages of avoiding extensive surgical procedures required for a shunt or some of the direct operations (eg. portoazygous disconnection). Clearly it is applicable for young children whose veins are deemed too small for a shunt and for those in whom a shunt has either failed or who are "unshuntable." In children with intrahepatic disease for whom a liver transplant may eventually be required, sclerotherapy may well be the preferred method of controlling bleeding varices.

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.

Congenital drainage of the vas deferens into a ureter permits retrograde passage of urine through the vas to the epididymis. If this anomaly is accompanied by urinary tract pathology with vesicoureteral reflux, hydroureter, stasis, and infection, the passage of urine into the vas can very well lead to epididymitis of a chronic or recurring nature and secondary scrotal abscesses. Two cases of this rare anomalous connection are presented in which such scrotal infections occurred. The diagnosis should be suspected on clinical grounds and can be confirmed by cystograms in which the dye refluxes up the ureter and passes into the vas. The association of rectal anomalies is of embryologic significance and may be additionally important clinically by further suggesting the possibility of the ureteral-vas anomaly.

Sixty-four infants with gastroschisis have been managed in the 9-yr period, 1970-1979, with four postoperative deaths. The silo technique has been the standard method of management in this series, permitting initial expansion of the abdominal cavity without increased abdominal pressure and respiratory embarassment. Removal of the silo and complete closure of the abdominal wall deficit were possible 5-12 days later. A high proportion of the infants were below 2500 g in weight at birth (61%); and although 3 of the 4 postoperative deaths occurred in the low birth weight group, this did not appear to be a factor in mortality. Associated anomalies occurred in 25 of the 64, but only the coincident intestinal atresias (6) were of major significance. These additional anomalies were not responsible for deaths. Two deaths occurred from problems dating from birth, one from aspiration and the second from sepsis. The other two resulted from postoperative complications resulting in infarction of the midgut. Both were caused in part by failure to adequately enlarge the abdominal wall defect at the time of the initial procedure. Other postoperative complications were relatively few. Although all required intravenous nutritional support, the long term results in terms of growth and development and of intestinal function were quite satisfactory.

Polyurethane moisture-vapor permeable film (MVPF), a new product for the management of superficial wounds, was studied in an uncontrolled clinical trial in 50 pediatric patients. MVPF was used in the management of skin graft donor sites, partial thickness burns, traumatic abrasions, decubitus ulcers, intravenous skin sloughs, and a variety of other wounds. Advantages of this material over standard dressing techniques include: more comfort, freedom of motion of the injured part, fewer dressing changes, and apparently faster epithelialization in many cases. Folliculitis, noted in one patient, was the only complication. MVPF is easily adaptable to outpatient wound management, and has gained rapid acceptance by nurses, patients and parents.

Eleven infants with colon atresia have been managed by staged procedure with survival and good health in ten. Complications have been relatively few. At the initial operation the proximal atretic segment is exteriorized as an end colostomy. Such a procedure is simple and safe, results in rapid relief of the obstruction, and permits normal feeding by mouth within a few days. At a second procedure several weeks or months later, intestinal continuity is established by an end-to-end or end-to-side anastomosis. Gastroschisis has been an associated anomaly in four of these cases. This association gives additional support to the concept that intrauterine interference to the blood supply to a segment of intestines is the etiologic factor responsible for such atresias.

Clamping of an occult omphalocele has led to complete division of an entrapped loop of ileum in two instances. The proximal end has been sealed in the process, producing an iatrogenic ileal atresia. The clinical picture in both instances differed from that usually found with ileal atresia. Escape of a small amount of meconium from the transected cord was noted in one, and the stump of the cord in the other appeared red and engorged. Clamping the umbilical cord routinely at least 5 cm from the abdominal wall is recommended.

Replacement of the thoracic esophagus with an interposed segment of colon was done in 29 children with esophageal atresia, stricture, or varices. There were no deaths. One failed because of vascular insufficiency of the transplant. The incidence of early postoperative complications was high. Long-term clinical and radiological evaluations were generally quite satisfactory in terms of normal growth, absence of pulmonary and intestinal symptoms, and rapid emptying of the transplant.

Although the clinical course of most infants with neonatal idiopathic thrombocytopenic purpura is benign and self-limited, extracutaneous bleeding is relatively common and potentially dangerous. Intracranial bleeding is the most common complication of this disease, and its occurrence demands prompt control. Emergency splenectomy has been most effective in accomplishing such control in this series. Splenectomy should be avoided as an elective procedure in early childhood. However, in the life-endangering situation of central nervous system bleeding with this disease, the operation has been dramatically successful and thoroughly justified.

Summary A striking improvement in the results of infants with gastroschisis has occurred in recent years. The overall survival of 47 patients in a 25-yr period was 53%. In the first 20 years of this experience, only six of 20 lived (30%). In the past 5 yr, 19 of 27 have survived (70%). Two methods have given very satisfactory results. The skin-closure technique involves a major postoperative problem of respiratory support and a lesser problem of impaired wound healing. Furthermore, later hospitalizations for staged repairs of the ventral hernias are required. Despite these problems, there have been only two deaths in the 13 patients handled in this fashion in the last 5 yr. The silo technique is much simpler from the standpoint of the initial procedure and has the great advantage of avoiding significant postoperative respiratory embarrassment. Early technical problems with the material used. separation or tearing of the silo, and infection have largely been solved and the recent results have been very satisfactory. Although the recent experience with both techniques has been comparable in terms of mortality, the much reduced morbidity with the silo technique recommends it as the preferred method.

Complete pyloric obstruction caused by a duplication was relieved by excision without resection of the contiguous pylorus. Histologically all of the normal layers of the pylorus were present. In addition a few foci of pancreatic tissue showing acute inflammation were found.