Biomedical subjects
C M Rumack
Publications and source records attributed to C M Rumack.
Portal venous velocity in biliary atresia.
From December 1986 to April 1989, 38 patients with biliary atresia (eight newly diagnosed) were evaluated with doppler ultrasound of the portal venous system. Peak and mean velocities were computer derived from the spectral waveform. Good velocity was greater than 15 cm/s, intermediate velocity was 8 to 14 cm/s, abnormal velocity was less than 7 cm/s or hepatofugal. Patients were grouped according to clinical status: group 1 (n = 14), normal liver function; group 2 (n = 15), recurrent cholangitis; group 3 (n = 2), established bile drainage but complicated cirrhosis; group 4 (n = 7), failed portoenterostomy. All patients with normal liver function (group 1) had good or intermediate velocities. Thirteen of 15 patients with recurrent cholangitis (group 2) had good or intermediate velocities. Both patients in this group with abnormal velocities required transplantation. In group 3 the patient with abnormal velocity is on the transplant waiting list. In group 4, abnormal velocities preceded or coincided with deterioration of liver function in five of seven patients. Doppler ultrasound provides useful anatomic information, determines direction of flow, quantitates velocity of flow, and, when performed serially, provides adjunctive information on liver status in children with biliary atresia. These preliminary results suggest that patients with abnormal or significantly decreasing velocity are destined for transplantation. Patients with good portal venous velocity warrant ongoing, aggressive surgical management.
Absolute intracranial blood-flow velocities evaluated by duplex Doppler sonography in asymptomatic preterm and term neonates.
Thirty-eight asymptomatic preterm and term neonates were studied with pulsed Doppler sonography to assess absolute (true) intracranial blood-flow velocities. The middle and posterior cerebral arteries were evaluated by a transcranial approach, and the anterior cerebral artery was evaluated via the anterior fontanelle. Intracranial velocities were seen to increase with increasing gestational age and with increasing age of the neonate. The rate of increase in velocity was greater with higher gestational age. Middle cerebral artery velocities were greater than anterior cerebral velocities. Likewise, antegrade diastolic flow was always seen in these normal neonates. Intracranial velocities increased with gestational age and with increasing age of the child; the resistive index decreased progressively with age.
Cranial sonography: intracranial hemorrhage, periventricular leukomalacia, and asphyxia.
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Role of respiratory syncytial virus in early hospitalizations for respiratory distress of young infants with cystic fibrosis.
To determine the frequency of respiratory syncytial virus (RSV) as the cause of hospitalization for acute pulmonary exacerbations in young infants with cystic fibrosis (CF), and to assess the clinical effects of RSV infections, we prospectively followed 48 children with a diagnosis of CF after identification by newborn screening. At a mean follow-up age of 28.8 months (range 5 to 59), 18 infants (38%) had been hospitalized a total of 30 times for acute respiratory distress. At the time of admission, 18 infants (60%) were less than 12 months, 8 (27%) between 12 and 24 months, and 4 more than 2 years of age. The RSV was identified in seven hospitalized infants, as determined by fluorescent antibody, immunoassay, or culture. Before admission with RSV infection, one of the seven infants had chronic respiratory signs, none had Brasfield chest x-ray scores below 20, and a previous throat culture was positive for Staphylococcus aureus in one infant. Hospitalizations were prolonged (mean duration 22 days), and were characterized by significant morbidity, with three infants (43%) requiring mechanical ventilation and five infants (71%) requiring home oxygen therapy for persistent hypoxemia at discharge. At a mean follow-up age of 26 months, these infants more frequently have chronic respiratory signs (p less than 0.01) and lower chest radiograph scores (p less than 0.05) than other CF infants. These findings demonstrate that RSV is an important cause of early acute respiratory tract morbidity in young infants with CF, and suggest the need for studying new strategies to implement early and aggressive antiviral therapy in young infants with CF.
Kleeblattschadel anomaly. In utero sonographic appearance.
Four cases of Kleeblattschadel demonstrated on prenatal ultrasound examinations are reported. Five additional cases from the literature are reviewed. Sonographic features include enlarged trilobed skull, hydrocephalus, polyhydramnios, and frequent association with thanatophoric dwarfism. The most common error in diagnosis was misinterpretation of this skull anomaly as encephalocele.
Diagnosis of brain neuropathology in utero.
The fetal brain can be evaluated very effectively with high-resolution real-time ultrasound equipment. This paper reviews normal fetal brain anatomy with specific emphasis on artifacts of ultrasound scanning of the brain. A review of the developmental abnormalities seen in utero include ventriculomegaly, anencephaly, encephalocele, meningomyelocele, Dandy-Walker syndrome, holoprosencephaly, craniosynostosis, microcephaly, and agenesis of the corpus callosum. The destructive lesions reviewed include hydranencephaly, infection, intracranial hemorrhage, and mass lesion. Identification of these abnormalities can be extremely helpful in providing the patients with management options and providing the obstetrician with information which can help in obstetric and neonatal therapy.
The fetal spine.
Ultrasonography has made it possible to evaluate the fetal spine in utero. Vertebral ossifications are demonstrable by ultrasonography in the early second trimester. Most spinal defects are apparent by 20-22 weeks menstrual age. Early detection of spinal anomalies allows for parental counseling and appropriate obstetrical management. Real-time equipment allows the fetal spine to be examined in parasagittal, transverse and coronal planes. The transverse plane provides the best view of the three ossification centers that form each fetal vertebra. Evaluation of the neural arch ossifications in the transverse view is crucial in detecting spina bifida defects. Ultrasonography of the fetal spine complements the use of alpha-fetoprotein levels in screening for neural tube defects. Examination of the fetal spine is also important in detecting vertebral ossification defects, particularly in cases of short-limbed dwarfs and infants of diabetic mothers.
Specific skeletal dysplasias in utero: sonographic diagnosis.
A retrospective study was performed of 13 short-limbed fetuses with lethal skeletal dysplasias that were evaluated with ultrasound (US) from 1981 to 1984. The specific diagnoses were thanatophoric dwarfism, achondrogenesis, osteogenesis imperfecta, and campomelic dwarfism. Death occurred in utero or within 2 weeks after delivery in all cases. US examination showed other associated abnormalities, including polyhydramnios, hydrops, shortened femurs, and CNS abnormalities. Radiographs confirmed these findings and provided more information regarding the shape of the limbs and thorax and the appearance of the spine. The probable diagnosis of lethal short-limbed dwarfism was made antenatally using US in eleven of the fetuses. Spinal appearance, thoracic shape, and associated hydrops and polyhydramnios were most helpful in determining the specific type of dysplasia present. Lethal short-limbed skeletal dysplasia may be diagnosed confidently in utero using US examination; however, obstetric plain film radiography may be required to determine the definitive type of dysplasia. In certain cases, US may be sufficient to make a definitive diagnosis.
Hydrometrocolpos, uterus didelphys, and septate vagina: an antenatal sonographic diagnosis.
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In utero diagnosis of intracranial hemorrhage resulting in fetal hydrocephalus. A case report.
An intracranial hemorrhage resulted in fetal hydrocephalus. Antepartum fetal heart rate monitoring instituted for decreased fetal activity led to this intrauterine diagnosis. The infant had no neurologic delay at 16 months of age.
Timing and course of neonatal intracranial hemorrhage using real-time ultrasound.
Knowledge of the timing and course of neonatal intracranial hemorrhage is essential in determining the etiology of hemorrhage, since the key to preventing neurologic damage in newborns is to define preventable causes and to treat the complications of hemorrhage. In an intensive study of 49 neonates delivered less than or equal to 32 weeks gestation, the initial hemorrhage typically occurred in the first three days of life, with 36% occurring on day 1, 32% on day 2, and 18% on day 3. Most of the major hemorrhages (Grades III and IV) occurred on day 1, although ventricular enlargement occurred at a variable time after the initial insult. By the sixth day, 91% of all intracranial hemorrhage had occurred.
Partial splenic embolization in children with hypersplenism.
Partial splenic embolization (PSE) was successfully accomplished in 10 of 11 children, aged 2-9, who had portal hypertension or variceal bleeding. Nine of the 11 children had undergone portoenterostomy (Kasai operation) for biliary atresia, and two had portal vein thrombosis. After embolization these children had a longer period of fever (mean = 23.7 days) and elevated white blood cell (WBC) count (above 10,000, mean = 13.6 days) than adults who have undergone the same procedure. The leukopenia and thrombocytopenia of hypersplenism were corrected by PSE in seven of eight children, and the condition of the eighth child improved. Among ten patients who had experienced episodes of variceal hemorrhage, the frequency of bleeding episodes was reduced from an average of 2.87 per year before PSE to 0.67 per year after PSE. There were no splenic abscesses and no other significant complications of the treatment. Ultrasound (US) evaluation after embolization demonstrated hypoechogenicity of the infarcted areas and tiny, linear echoes scattered throughout the spleen typical of postinfarction intravascular gas. All nine children who underwent follow-up Tc-99m sulfur colloid scanning showed evidence of splenic regeneration, though none has had recurrence of clinical symptoms. Splenic regeneration following PSE may occur more frequently in children than in adults.
Fetal intracranial teratoma: US diagnosis of three cases and a review of the literature.
We describe three cases of fetal intracranial teratoma diagnosed by ultrasound and review the literature. Sonographic features include cranial enlargement, gross distortion of normal cerebral architecture by a hyperechoic, multicystic mass, and polyhydramnios. Despite early diagnosis, the cesarean section rate is high and the overall prognosis is dismal.
Gastrointestinal injury in polycythemic term infants.
Necrotizing enterocolitis is uncommon among term infants. In this group, necrotizing enterocolitis has been associated with two risk factors: polycythemia and umbilical catheterization. During a randomized trial of partial plasma exchange transfusion for treatment of polycythemia, an increased risk of gastrointestinal problems was noted. Eight hyperviscous patients treated with partial plasma exchange transfusion, no symptomatically treated patients, and no control infants developed typical necrotizing enterocolitis (blood in the stools, pneumatosis, and systemic signs). The incidence of necrotizing enterocolitis was significantly greater among patients treated with exchange transfusion compared with patients treated symptomatically or control subjects (P less than .001).
Cystic adenomatoid malformation: antenatal demonstration.
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Role of coagulopathy in newborn intracranial hemorrhage.
Fifty newborn infants of less than 33 weeks' gestation were followed prospectively from birth with serial coagulation and real-time ultrasound studies. A significant association of hypocoagulability in the first four hours of life with subsequent onset or progression of intraventricular or other clinical hemorrhages was documented. Abnormalities included lower values for fibrinogen, platelet count, antithrombin III, and factor VIII with higher values for fibrin monomer and longer Laidlaw whole blood clotting times. These abnormalities tended to correct spontaneously in surviving infants. An association between gestational complications and incidence of hypocoagulability and intracranial hemorrhage (ICH) was noted. Babies of preeclamptic mothers had fewer abnormalities and babies born to mothers with premature rupture of membranes and suspected amnionitis manifested more hypocoagulability and more severe intracranial hemorrhages.
Timing and antecedents of intracranial hemorrhage in the newborn.
Fifty newborn infants of less than 33 weeks' gestation were followed prospectively from birth to evaluate the temporal relationships of various clinical factors to the onset and progression of intracranial hemorrhage (ICH) in an inborn population given maximal support. ICH was diagnosed and followed with bedside ultrasound every eight hours. The incidence of intraventricular hemorrhage was 30% and of any ICH was 40% with onset from less than 2 hours to 8 days of age. Grades 2, 3, and 4 ICH correlated with Apgar scores of less than 5 at five minutes, vaginal delivery, longer labors, and intrapartum hemorrhage. There was a significant correlation between ICH and both blood pressure fluctuations of greater than 100% and rapid colloid infusions. Slow transfusions of packed red cells did not appear to precipitate episodes of ICH. In a setting of optimal care, ICH appears to be more related to prenatal stresses than to specific postnatal complications.