A prospective, randomized comparison of traditional and laparoscopic inguinal exploration in children.

The indications for routine exploration of the asymptomatic contralateral groin during pediatric herniorrhaphy remain controversial. Laparoscopy through the open hernia sac has been described as an alternative to this traditional approach and appears to offer some advantages. In deciding whether this technique should be introduced into our clinical practice, we sought to discover whether there was a significant time difference between these two methods and if there were unique complications associated with this approach. We elected to answer this question in a prospective, randomized study. A total of 18 patients completed the study. There were no significant complications in either group. The average total surgical procedure time in the laparoscopy group was 47.5 minutes versus 41 minutes in the traditional group, which is not a statistically significant difference. We conclude from this prospective, randomized pilot study that laparoscopic exploration can be introduced into a pediatric surgical practice without a significant time penalty, and we currently offer it as a reasonable and safe alternative to our patients.

Ventilator management of infants before extracorporeal membrane oxygenation.

The aim of this project was to review the course of infants referred for consideration of extracorporeal membrane oxygenation (ECMO) to identify maximal ventilator settings that, when exceeded, did not provide clinical benefit to the patient. These settings might then be used in defining failure of conventional mechanical ventilation. We reviewed referral records and hospital charts of all infants treated for severe respiratory failure due to meconium aspiration syndrome during the 52.5 month period from March 15, 1985, to August 1, 1989. At an inspiratory pressure > 35 cm H2O, 75% (43/57) of patients eventually required ECMO, and 28% (4/14) of the infants who did not receive ECMO died. When the inspiratory pressure was > or = 40 cm H2O, 39/49 patients required ECMO, and 30% (3/10) of those not treated with ECMO died. Once the inspiratory pressure was > 45 cm H2O, 91% (29/32) of patients required ECMO, and only one third of those not treated with ECMO survived. Although the limitations for conventional therapy suggested in this paper may be helpful to clinicians, each center needs to establish guidelines for maximal conventional ventilator support. If these guidelines are clearly defined, alternative methods of therapy can be used once these criteria are achieved.

The status of pediatric surgical rotations by residents in pediatrics.

A variety of forces are reshaping the traditional relationship between physicians and their patients. One consequence of this reform movement will be increased responsibility of the pediatrician to evaluate children with surgical diseases. Pediatricians also will be encouraged to do more of the minor surgical procedures themselves. It is not clear how academic pediatric surgeons should adapt to these changes to assure that the general pediatrician is equipped with the skills to handle this increased responsibility. One obvious solution would be to have the pediatrician in training rotate on the pediatric surgery service. The authors have considered these issues at their institution, and thus became interested in learning the status of pediatric surgical rotations by pediatric house staff in this country. A survey was distributed to the 221 accredited pediatric training programs in this country, and 143 individuals responded. Only thirty-five of these programs require a rotation on pediatric surgery. Eighty-six programs offer it as an elective, but only a minority of house staff takes it. The most disturbing aspect of the survey was the 28 of the programs had required a pediatric surgical rotation in the past but had eliminated it. The most common reasons given for this action were the poor educational content of the rotation and the labor requirements of the pediatric service. Based on the survey, the authors believe that it is unlikely that mandatory rotations on pediatric surgery will be begun in pediatric training curriculums. If pediatric surgeons wish to be involved in training pediatricians, they will need to address the educational content of their electives to meet the changing educational needs of the pediatricians.

Laparoscopic cholecystectomy in children with sickle cell disease.

Since 1991, laparoscopic cholecystectomy has been utilized in children with sickle cell disease, predominantly because of the decreased pain and shorter hospitalization. We believe that outpatient laparoscopic cholecystectomy or even a 24 hour hospitalization is not indicated in the patient with sickle cell disease. Perioperative complications include bleeding diathesis, vaso-occlusive phenomena, and delayed hemolytic transfusion reactions, although clotting parameters can be normal.

Transverse colon volvulus in pediatric patients.

Volvulus of the transverse colon is rare in the pediatric population. We present the tenth known case of a volvulus of the transverse colon in a child. A barium enema demonstrated the bird beak appearance of the colon and showed an air-contrast mirror image in the proximal end. The 360 degrees volvulus found at surgery was treated successfully by detorsion of the bowel.

Cervical thymic cysts: CT appearance of two cases including a persistent thymopharyngeal duct cyst.

The differential diagnosis of cervical cysts in children includes common entities such as branchial cleft cysts, thyroglossal duct cysts, and cystic hygromas. Congenital thymic cysts are uncommon and often misdiagnosed as either branchial cleft cysts or cystic hygromas. However, they may have an appearance on CT that can be characteristic. The course of the descent of embryologic thymic tissue in the neck to the mediastinum indicates the potential site of deposition of an ectopic cervical thymic cyst. In a child, a cystic lesion that has an intimate relationship to the carotid sheath is likely to be a thymic cyst. Of the approximately 100 cases of vestigial cervical thymus or thymic cysts that have been reported in children, only 5 cases of a persistent thymopharyngeal duct cyst have been described [1-5]. In two of these five, the persistent thymopharyngeal duct cyst was demonstrated by CT [1,2]. We report one additional case of a cervical thymic cyst and one case of a persistent thymopharyngeal duct cyst both depicted by CT.

Interferon-alpha-2a for the treatment of complex hemangiomas of infancy and childhood.

OBJECTIVE: The authors describe the use of interferon-alpha-2a (IFN-alpha-2a) in the treatment of complex hemangiomas and review the role of interferon (IFN) in this example of an angiogenic disease. SUMMARY BACKGROUND DATA: Hemangiomas are the most frequent tumors of infants and children. They grow rapidly for 6 to 8 months and then resolve over a period of years. Approximately 5% produce life-, sight-, or limb-threatening complications, with mortality rates between 20% and 50%. Aggressive therapy with steroids, arterial ligation or embolization, or surgery has been used in these situations with variable results and high morbidity. Recently, IFN-alpha was found to be effective treatment in these complex hemangiomas. METHODS: Four infants and one child were treated with IFN-alpha-2a at an initial subcutaneous dose of 1 million units/m2/day and a sustained dose of 3 million units/m2/day for 5 to 11 months. Appropriate laboratory values were monitored and adverse reactions and ultimate response to therapy were recorded. RESULTS: Two patients experienced minor complications that were managed easily. Three patients had total or near-total regression of the hemangioma, one had partial (50%) regression, and one had stabilization but no regression after an average of 7.1 months of IFN therapy. CONCLUSION: Interferon-alpha inhibits angiogenesis and endothelial cell migration and proliferation in vitro. The patients in this study add to the growing number who have benefited from IFN therapy. As such, IFN-alpha should be considered as a first-line agent in treating complex hemangiomas of infants and children.

Histogenesis of vascular tumors in the Proteus syndrome.

Proteus syndrome (PS) is a congenital disorder manifesting with severe deformities, the salient features being gigantism and vascular tumors. The disorder is poorly understood, and there has been much discrepancy in the terminology regarding the vascular tumors in PS. The purpose of this study was to elucidate the histogenesis of these tumors by correlating microscopic observations with immunohistologic information. The value of immunoperoxidase studies in the pathologic evaluation of PS was also assessed. Fourteen formalin-fixed, paraffin-embedded tissue specimens obtained from vascular tumors of six children with PS were stained with Ulex europaeus agglutinin I (UEA-I) lectin and the following immunohistochemical reagents: anti-factor VIII-related antigen (FVIII-RAg) and anti-CD34. The tumors showed varied proportions of vascular, lipomatous, and fibrous tissue components consistent with vascular hamartomas. The predominant vascular channels of the tumors were morphologically consistent with lymphatic vessels. Immunostaining of the endothelium of these vessels was most consistently positive with UEA-I lectin. Although a color reaction product was present in small vessels and some larger blood vessels, anti-CD34 immunostaining spared the lumens of lymphatic channels. In addition, a striking population of dendritic spindle cells was noted with the anti-CD34 but was unnoticed with the other reagents. We concluded that the vascular tumors of PS are primarily lymphatic hamartomas. The spindle cells noted with anti-CD34 immunostaining may relate to angiogenesis and need further delineation.

Successful management of an infant with a giant hemangioma of the retroperitoneum and Kasabach-Merritt syndrome with alpha-interferon.

Giant vascular neoplasms in neonates generally require aggressive medical or surgical therapy for treatment of complications. Steroids, chemotherapy, embolization, radiation, and surgery have all been used with short-term beneficial and sometimes unknown long-term side effects. A new modality of treatment, alpha-interferon, has recently been described. The majority of hemangiomas in children involute by 8 years of age. Occasionally, hemangiomas can endanger vital structures and are associated with a consumption coagulopathy and thrombocytopenia (Kasabach-Merritt Syndrome). These hemangiomas occasionally do not respond to steroids, radiation therapy, cytotoxic drugs, or embolization. The mortality rates approach 50% in nonresponders. Alpha-interferon has been used in these children with life-threatening complications of hemangiomas with relief of symptoms. This case illustrates the potential use of alpha-interferon in the management of giant hemangiomas in children. This emerging form of biological therapy avoids the risks of radiation therapy, embolization, and surgery with only minimal side effects.

Strangulated omental hernia detected by scrotal scan.

A case of a strangulated omental hernia detected by a scrotal scan is presented. This may be the first report in which the scrotal scan showed diffuse increased isotope accumulation throughout the hemiscrotum.

Proteus syndrome: diagnosis and surgical management.

The congenital hamartomatous syndrome known as the "Proteus syndrome" (PS) manifests itself with regional giantism, lymphangiomatous hamartomas, and other variable features. Review of the medical literature shows approximately 50 cases reported to date. Since this syndrome has only recently been defined, the management of these patients has been speculative and often children are not treated. This report summarizes the characteristics of the PS and presents eight additional cases. All of the eight children had regional giantism with macrodactyly and skeletal hypertrophy. Asymmetrical leg length was pronounced in five children. All children had large lymphangiomas, the majority of which involved the trunk. Three of the children have been followed through adolescence, two into late childhood, and three into early childhood. In contrast to previous reports, we believe that early surgical reconstruction is necessary to reduce deformities due to the giantism and the large hamartomas. During extensive excisions, residual abnormal tissue is often needed in the reconstruction and it is not unusual for postoperative leakage of lymph to be prolonged. All of the children in this series have benefited both physically and emotionally from extensive surgical reconstruction.

Recurrent renal cell carcinoma arising in Wilms' tumor.

A 19-month-old black girl had a radical nephrectomy for a Wilms' tumor that contained areas of epithelium indistinguishable from renal cell carcinoma. She was treated with chemotherapy but subsequently had pulmonary metastases develop and massive abdominal recurrence. The recurrent tumor was histologically renal cell carcinoma with no identifiable Wilms' tumor elements. The child died with recurrent and metastatic tumor 13 months after nephrectomy. Pathologic, immunoperoxidase, and flow cytometric studies of this unusual case are presented.

Rectovulvar fistula in a child secondary to an unusual foreign body.

Foreign bodies in the female genital tract are well recognized as a cause of pain, discharge, secretions, and infection. In the small child, the presence of a vaginal discharge is usually associated with either a common object which has been inserted or sexual abuse complicated by a sexually transmitted disease. A 3-year-old child presented with a recurrent labial secretion and drainage due to an unusual foreign body. It was only during a second operation that the possibility of a foreign body was entertained, and diagnostic testing was begun. A third operation permitted removal of the foreign body, a large bone probably of animal origin. The fistula tract was closed after a colostomy was performed. This represents the first reported case of a rectovulvar fistula not of a congenital nature.

Hidden mortality rate associated with extracorporeal membrane oxygenation.

We reviewed the outcome of all infants referred to, and accepted in, our extracorporeal membrane oxygenation (ECMO) program during a 52-month period. One hundred sixty-seven referrals, representing 158 infants and nine mothers who had not yet delivered their infants, were accepted. Eighteen infants (11.3% of all neonates transported) died before leaving the referring hospital, during transport, or shortly after admission to our unit. Contraindications to ECMO excluded 17 (10.1%) of the 167 referrals. Sixty-two infants (37.1%) initially did not meet ECMO criteria. Two died before ECMO could be started. Sixty-eight infants (40.7%) were given ECMO therapy, and 11 died (16.1%). Nine mothers were referred because of fetal conditions that might require ECMO; of these infants, two died during delivery and three had contraindications to the use of ECMO. The four remaining infants were given ECMO therapy; three survived. The overall mortality rate was 27.5% (46/167); 18 (39.1%) of the 46 deaths were associated with transfer. The mortality rate associated with congenital diaphragmatic hernia was 63.6%. We recommend early transport of infants with this type of hernia during the postoperative "honeymoon" or during in utero transport with delivery at an ECMO center. We also recommend that infants with meconium aspiration syndrome be transported to an ECMO center when an oxygenation index of 25 is reached. The mortality rate associated with transport needs to be considered in evaluating ECMO programs. Earlier, expedited transfers may increase the survival rate.

Need for and impact on neonatal mortality of extracorporeal membrane oxygenation in infants of greater than 2500-gram birth weight.

The use of extracorporeal membrane oxygenation (ECMO) has increased significantly during the last 4 years, with more than 2400 infants now having been treated with the technique. In spite of an ill-defined role for ECMO as well as the potential for neurologic sequelae for these patients, additional centers continue to be established across the United States. To provide information regarding the number of babies who could be expected to require ECMO, the early neonatal deaths for the state of Georgia were reviewed for the 2-year period 1983-1984. By means of both linked birth and death certificate analysis and chart review of infants with a birth weight of 2500 g or more, identification was made of 36 infants whom we classified as potential ECMO candidates. These infants were thought to represent 80% of all infants who would have received ECMO during the 2 years, resulting in an estimate of 1 patient receiving ECMO per 3717 live births for the state. With a predicted survival rate of 80% for this group, the state neonatal mortality rate would have declined by 0.13 per 1000 live births, a decrease of 1.4%.

Bilateral ovarian fibromas in children.

The case of bilateral ovarian fibromas occurring in an 8-year-old black girl is reported. These lesions occur rarely in premenarchal females and may be a manifestation of Nevoid Basal Cell Syndrome. Calcifications are reported to occur rarely in ovarian fibromas but seem to occur frequently in fibromas in children. Management is guided by the benignity of the lesion and consists of surgical excision of the fibroma. Preservation of normal ovarian tissue is recommended with the acknowledged risk of recurrence of the fibroma.

Recent experience with diaphragmatic hernia and ECMO.

In the past 4 years at the Medical College of Georgia, a total of 74 patients underwent extracorporeal membrane oxygenation (ECMO) with 62 (84%) survivors. Forty-seven of these infants had meconium aspiration syndrome and 11 had diaphragmatic hernia. The use of ECMO, when indicated, after reduction and repair of the diaphragmatic hernia, results in normal oxygen delivery, allows time for pulmonary maturation, and increases survival. A total of 27 referrals for diaphragmatic hernia were studied. Six infants had surgical repair and did not require ECMO. Eleven patients, after surgical repair, were treated with ECMO and seven survived. More importantly 10 patients died before the use of ECMO. Six infants died either before or during transport from referring hospitals and four died while in the delivery room or neonatal unit before ECMO. Of these 10 infants, eight were potential candidates for ECMO. Thirteen of the twenty-seven (48%) infants survived. Seven of eleven (64%) infants who received the benefit of ECMO survived. Eight infants who met the criteria for ECMO died before its use. Had ECMO been used in those eight infants, our data suggests that at least four may have survived. The data from this report support the concept that infants undergoing surgical repair of diaphragmatic hernia, when ECMO is not available, should be referred to an ECMO center in the early postoperative period. Furthermore infants with prenatal diagnosis of diaphragmatic hernia should be delivered at a center where surgical as well as ECMO expertise are available.

Incidence of hypertension in infants on extracorporeal membrane oxygenation.

Systemic hypertension has been associated with extracorporeal membrane oxygenation (ECMO) applied in neonatal respiratory failure. To determine the incidence of ECMO-related hypertension, we reviewed blood pressure measurements from indwelling aortic catheters in 31 infants consecutively placed on ECMO. Systemic hypertension (systolic blood pressures greater than 100 mm Hg for 4 or more consecutive hours) developed in 18 of the 31. Causes investigated included the roles of renin secretion, sodium, and colloid loads. There was no evidence of increased plasma renin activities in hypertensive infants (H), when compared with their own pre-ECMO controls or with the nonhypertensive infants (NH). Sodium and colloid loads and their rates of delivery were not different between H and NH. No consistent duration of ECMO was clearly associated with development of hypertension (mean time on ECMO at onset of hypertension, 43.8 +/- 38.5 hours; range, 1 to 142 hours). Demographic information was not statistically significant. Contrary to previous reports, H did not seem predisposed to an increased incidence of intracranial hemorrhage. Development of hypertension during ECMO is not related to increased plasma renin activity, sodium or colloid loads, or their rates of infusion.

Bypass circuits as the source of thromboemboli during extracorporeal membrane oxygenation.

To determine the presence and extent of thrombus formation in the apparatus used for extracorporeal membrane oxygenation we studied various portions of the polyvinylchloride circuit from five infants who received extracorporeal membrane oxygenation for 70 to 330 hours. All infants had right-sided cannulation. Sections were cut from the circuit at the time of decannulation and subjected to light and scanning electron microscopy. The site that contained the most thrombus formation was the membrane oxygenator bypass circuit, which is subjected to repeated periods of unclamping and clamping to direct blood flow through the membrane oxygenator. Autopsy results from nonsurvivors showed evidence of pulmonary and renal infarcts, a left frontal lobe infarct, a thromboembolus of the left external and internal carotid arteries, and thrombi in the lungs, kidney, brain, and coronary arteries. One survivor had computed tomographic evidence of infarction of the left middle cerebral artery distribution. We suggest that the areas of the extracorporeal membrane oxygenation circuit subjected to repeated changes in flow dynamics may be the source of microemboli.