Sheldon J. Bond

DEATH DUE TO HIGH-OUTPUT CARDIAC FAILURE IN FETAL SACROCOCCYGEAL TERATOMA

Fetal sacrococcygeal teratoma (SCT) is being recognized with increasing frequency. Placentomegaly and hydrops fetalis are preterminal events, and it has been suggested that fetal death may be due to high-output cardiac failure from arteriovenous shunting through the tumor. We had a chance to examine this hypothesis when a 21-week fetus presented with a huge sacrococcygeal teratoma. There were marked placentomegaly, cardiomegaly, hyperdynamic ventricles, and a pericardial effusion. Doppler studies showed tremendous flow through the SCT with extreme enlargement of the inferior vena cava, consistent with congestive heart failure from increased flow through the tumor. Hydrops developed, and the fetus was delivered because of placental abruption. This case provides supportive evidence that the teratoma acts as a large arteriovenous shunt, causing high-output cardiac failure. We have now collected 18 more cases of sacrococcygeal teratoma diagnosed in utero. Of the total 45 cases of fetal SCT, 9 had placentomegaly and/or fetal hydrops and all 9 fetuses died in utero or shortly after birth. We conclude that the only hope for survival in these severely affected fetuses is to reduce blood flow to the tumor before birth.

Severity of intestinal damage in gastroschisis: Correlation with prenatal sonographic findings

From 1982 to 1986, 26 abdominal wall defects were detected prenatally. Seventeen pregnancies culminated in live births with ultrasound correctly distinguishing between omphalocele and gastroschisis in all cases. In the 11 cases of gastroschisis diagnosed before birth, we attempted to correlate the clinical outcome with the size of the abdominal wall defect, sonographic appearance of the eviscerated bowel, and known time of exposure to amniotic fluid (gestational age at birth to gestational age at diagnosis). Neither the time of exposure to amniotic fluid (median duration of 14 weeks) nor the defect size could be correlated with eventual clinical outcome. The presence of small bowel dilatation and mural thickening on prenatal sonography (four patients) had a high correlation with severe intestinal damage and poor clinical outcome. The absence of these two sonographic findings (seven patients) was associated with mild intestinal changes and benign clinical course with no morbidity or mortality. We conclude that obstetric ultrasound cannot only accurately detect the presence and type of abdominal wall defect, but it also gives an indication of the severity of intestinal damage and subsequent clinical course in prenatally diagnosed cases of gastroschisis. Early delivery of the fetus with prenatally diagnosed gastroschisis should no longer be performed to limit exposure to amniotic fluid. Now that reliable sonographic criteria of severe intestinal damage have been defined, the decision to deliver early can be restricted to those fetuses with bowel dilatation and mural thickening.

Cutaneous burns caused by sulfuric acid drain cleaner.

BACKGROUND Highly concentrated solutions of sulfuric acid are available to unclog drains. We have noted a substantial number of both accidental and intentional cutaneous burns caused by these agents. METHODS A retrospective review was conducted of children and adults who sustained sulfuric acid burns over a 13-year period ending in May 1996. Reports of injuries related to drain cleaners filed with the United States Consumer Product Safety Commission between 1991 and 1995 were also reviewed. RESULTS Twenty-one patients (13 children, 8 adults) sustained cutaneous burns caused by concentrated sulfuric acid solutions. In 8 instances, the burn was accidental, whereas in 13 cases, sulfuric acid was used as a weapon. Median total body surface area burned was 5% (range, 1-25%). Approximately 50% of burns involved the face and neck. Skin grafting was required in 14 patients (66%). It is estimated that nationwide approximately 3,000 injuries per year are related to drain cleaners and that one-third of these involve cutaneous burns. CONCLUSION Highly concentrated sulfuric acid drain cleaner can produce full-thickness cutaneous burns that require skin grafting in the majority of cases. Proper use of these agents and sequestering them from children may reduce accidental contact; however, their abuse as agents of assault remains a source of significant morbidity.

Open lung biopsy in pediatric patients on extracorporeal membrane oxygenation

Open lung biopsy has proven beneficial in the treatment of life-threatening pulmonary diseases. Its safety and efficacy in infants and children placed on extracorporeal membrane oxygenation (ECMO) for severe respiratory failure is not known. The authors reviewed eight cases (4 neonates, 3 infants, 1 child) who underwent open lung biopsy while on ECMO. The primary diagnoses were pneumonia (4), lymphoma (1), primary pulmonary hypertension (1), and complex congenital heart disease (2). The patients underwent biopsy after they had been on ECMO an average of 9.6 days (range, 1 to 14 days). Biopsy results confirmed the clinical diagnosis in five patients, two of whom had irreversible alveolar destruction resulting in ECMO withdrawal. Three patients had pathological diagnoses, which resulted in major therapy revisions (1 fungal infection and 2 noninfectious lesions that required steroid treatment). The overall average duration of ECMO treatment was 16.3 days (range, 10 to 24 days). Three patients were weaned successfully from ECMO, but only one infant survived to discharge. One nonlethal bleeding complication occurred after biopsy. Open lung biopsy is well tolerated during ECMO. It accurately determines pulmonary pathology and provides valuable prognostic information. Earlier biopsy for patients whose diagnoses are uncertain or who are not responding to ECMO may improve the mortality rate for this high-risk group.

Complicated cannula insertions and cannula dislodgments associated with extracorporeal membrane oxygenation

Extracorporeal membrane oxygenation therapy remains a life saving modality for neonates with cardiopulmonary disease that is unresponsive to conventional therapy. Vital to its success is the insertion and maintenance of appropriately sized vascular cannulas. Problematic insertion and accidental dislodgments can be life threatening. To determine the treatment and outcome of these complications, a survey of participating Extracorporeal Life Support Organization centers was undertaken. Venous cannulation complications (13 patients) were due to inadequate vein size or tearing during initial attempts at cannulation. Proximal and alternative site cannulation were used. Difficult arterial cannulations (seven patients) were most often due to creation of an intimal flap that was corrected by proximal cannulation of the same vessel. The 10 cases of accidental dislodgment were most often associated with changes in position. Despite copious hemorrhage in many instances, 6 of 10 patients survived with intact neurologic status. Methods for dealing with these life threatening cannula related complications are described. ASAIO Journal 1998; 44:175–178.

Lipoma as a pathological lead point in a child with ileocolic intussusception.

Intussusception is a common cause of mechanical bowel obstruction among children. Most cases occur during the first year of life. Intussusception can occur anywhere along the gastrointestinal tract, but most frequently originates near the ileocecal valve. Children presenting at older ages are more likely to have a pathologic lead point causing the intussusception. Herein we present a case of pediatric ileocolic intussusception with 3 intestinal lipomas as its pathologic lead point. Lipomas are rare tumors of the gastrointestinal tract with no malignant potential. Children with a discrete pathologic lead point generally require surgical exploration and bowel resection.

Evaluation of post extracorporeal membrane oxygenation follow-up testing

The purpose of this study was to evaluate all post extracorporeal membrane oxygenation (ECMO) tests for their ability to detect any change in the incidence of unanticipated medical problems, and their charge to the patient. The current post ECMO protocol consists of the following tests: brain stem auditory evoked response, head computed tomography, cerebral blood flow, head ultrasonography, electroencephalography, eye examination for retinopathy of prematurity, and pneumocardiography. A retrospective review was conducted for all surviving neonatal ECMO patients treated from January, 1985, to December, 1994. The results of each test were classified as either normal, having a minor abnormality, or having a major abnormality. Statistical analyses were performed on each test comparing the incidence of minor and major abnormalities to all neonates in the neonatal intensive care nursery. Two hundred ninety-six infants survived their course on ECMO, and composed the study population. There were no significant differences between the incidence of abnormal results compared with the expected values for the following tests: cerebral blood flow (p = 0.13), the eye examination (p = 0.54), and pneumocardiography (p = 0.22). The analyses for the brain stem auditory response, head computed tomography, head ultrasonography, and electroencephalog-raphy showed higher than expected incidences of abnormal results (p < 0.01). The data also were evaluated for major abnormalities on computed tomography and head ultrasonography. Of 161 infants who had both-tests performed, 11 (6.8%) had normal head ultrasonography results, yet had a major abnormality noted on computed tomography (p < 0.01). This study is the first to review the current post ECMO protocol comprehensively, and the results suggest excluding the cerebral blood flow, eye, and pneumocardiography tests. This would result in a significant savings of

Etiology of intestinal damage in gastroschisis. I: Effects of amniotic fluid exposure and bowel constriction in a fetal lamb model

1,400 without compromising patient care. In addition, comparisons of neur-oradiographic studies indicate that computed tomography of the head is sensitive enough to detect all central nervous system abnormalities that were found by ultrasonography. Excluding the post ECMO head ultrasonography, an additional savings of

Survival of Patients with congenital diaphragmatic hernia during the ECMO era: An 11-year experience

300 would occur. These recommended changes reflect the current post ECMO protocol at Kosair Childrens Hospital. ASAIO Journal 1998; 44:171–174.