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Extracorporeal membrane oxygenation (ECMO) is an approved therapy for some neonates who have respiratory failure that is due to hyaline membrane disease, meconium aspiration, persistent pulmonary hypertension, congenital diaphragmatic hernia, or sepsis. The major complication of this therapy is hemorrhage, with intracranial hemorrhage having the highest morbidity and mortality. Seizures, incisional bleeding and bleeding in the pleural space, hypoxic-ischemic encephalopathy, renal failure, and cardiovascular complications account for most of the other complications. Cranial sonography provides an ideal imaging modality for baseline evaluation and daily follow-up; however, computed tomography and magnetic resonance imaging, because of better sensitivity, are important for assessment after ECMO. The changes in intracranial blood flow related to ECMO can be noninvasively evaluated by Doppler ultrasound modalities.
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PMID:Neurosonographic findings in infants treated by extracorporeal membrane oxygenation (ECMO). 268 79

Living related liver transplantation offers several advantages in comparison to transplantation of cadaver organs. To achieve maximal donor safety evaluation, selection criteria and complications of the donor operation were retrospectively analyzed in living donors of segmental liver transplants. Seventy-three liver donor candidates were evaluated between October 1991 and June 1994. The median age of 42 mothers and 31 fathers was 31 years (range, 19-50 years). The median volume of the left lateral liver lobe comprised 230 ml (100-350 ml). Twenty-four of 73 (33%) donor candidates were not accepted for living donation. Rejection was due to unsuitability of the donor's liver as a graft (n = 13) or due to an increased risk for living donation (n = 11). Of 35 living donations performed so far, one was a full left hemihepatectomy and 34 were left lateral segmentectomies. The length of the donor operation was, on average, 4.3 hr. No heterologous blood was needed. Postoperative complications included death due to pulmonary embolism (n = 1), seizure due to a previously undiagnosed ependymoma (n = 1), bile duct injury (n = 1), incisional hernia necessitating late revision (n = 2), and duodenal ulcer (n = 2). Long-term follow-up revealed no persistent complications. Using our standardized protocol, 33% of young, presumably healthy donor candidates were rejected for living donation.
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PMID:Selection of the living liver donor. 757 Sep 74

Since 1973, 7667 neonates have been treated with extracorporeal membrane oxygenation for severe respiratory failure and their cases reported to the Extracorporeal Life Support Organization Registry. The overall survival was 81% in these neonates, who were thought to have a survival of 20% without extracorporeal membrane oxygenation. A total of 4322 mechanical complications (0.56 +/- 0.84 per case) and 13,827 patient complications (1.80 +/- 2.12 per case) were reported overall. The most common mechanical complications included clots in the circuit (19%), cannula placement (9%), oxygenator failure (4%), and others (9%). Common patient complications included cardiopulmonary (43%), neurologic (35%), bleeding (35%), metabolic (32%), renal (25%), and renal (25%), and infectious (9%). From the initial experience to 1988 the average number of mechanical complications per case was 0.27 per case and this significantly increased during 1990 to 1992 to 0.75 per case (p < 0.05). Likewise, from 1973-1985 to 1988 the average patient complications per case were 1.44 per case and this significantly increased during 1990 to 1992 to 2.10 per case. During the same periods, patient survival significantly decreased from 84% (1973-1985 to 1988, n = 2463) to 80% (1990 to 1992, n = 4005). Venovenous double-lumen single cannula extracorporeal membrane oxygenation had a higher survival than venoarterial extracorporeal membrane oxygenation (91% versus 81%) and a lower rate of major neurologic complications. The incidence and survival with seizures (6% and 89% venovenous versus 13% and 61% venoarterial) or cerebral infarction (9% and 69% venovenous versus 14% and 46% venoarterial) was significantly lower with the venovenous method and appeared to have a substantial impact on overall survival. The correlation of patient complication rate and total complication rate with survival was highly significant, however, causality cannot be established. Explanations for the increase in complications, relative to a decrease in survival, despite a growing nationwide experience include (1) increased complexity of cases as many programs expand entry criteria (more premature infants, infants with grade 1 or 2 intracranial hemorrhage, and complex congenital diaphragmatic hernia), (2) a growing number of programs with fewer cases per program, yet greater accessibility, (3) less reluctance to report complications encountered during extracorporeal membrane oxygenation as group experience grows, and (4) changes in the Extracorporeal Life Support Organization data form to be more inclusive of more minor complications.
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PMID:Complications of neonatal extracorporeal membrane oxygenation. Collective experience from the Extracorporeal Life Support Organization. 812 13

It is often thought that survivors of congenital diaphragmatic hernia (CDH) have an isolated problem related to lung hypoplasia, and little data exist regarding the extrapulmonary problems of high-risk CDH patients who do survive. In 1990, the authors began a multidisciplinary follow-up clinic for CDH patients. Members of the program include representatives from the departments of surgery, pulmonary medicine, development, nursing, and nutrition. Since this program began, the authors have followed up on 33 infants who survived after treatment of high-risk CDH, ie, those who were symptomatic within 6 hours of birth. Twenty patients were treated with extracorporeal membrane oxygenation (ECMO). Neurological problems were common in these patients: seven children (21%) required hearing aids, and seven others had abnormal results with brain-stem auditory evoked response (BAER) testing. Extraaxial fluid collections or enlarged ventricles were present on head computed tomography scans of 10 children, and four children had clinical seizure activity. Fifteen patients had developmental delays, which improved rapidly once the children began to thrive. Six patients required eyeglasses or had strabismus, and one patient is congenitally blind. There were a variety of problems related to growth and nutrition, with six patients needing fundoplications, and 13 patients below the fifth percentile for weight. Of 10 patients with patch repairs, two had recurrent hernias. Six others required surgery for bowel obstruction. Eleven patients had pectus excavatum, usually mild, and four had mild to moderate degrees of scoliosis. There were undescended testicles in five boys, vesicoureteral reflux in two patients, and kidney stones in two patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Congenital diaphragmatic hernia: the hidden morbidity. 817 2

Before a health education program can be established, one must first know what the target population believes and does with respect to the disease in question. Therefore, we performed a study among Tanzanian rural inhabitants to identify their knowledge, attitude, and practice (KAP) toward epilepsy: 3,256 heads of households (mean age 40.2 years, range 15-90 years; M/F ratio 1:1) were interviewed. Of the respondents, 32.9% said they had never seen a seizure; 67.7% said they did not know the cause of epilepsy; 33.3% mentioned various causes including heredity, witchcraft, infection of the spinal cord, hernia; 40.6% believed epilepsy was infectious through physical contact, flatus, breath, excretions, sharing food; 36.8% believed epilepsy could not be cured and 17.1% believed it could not even be controlled; 45.3% believed epilepsy could be treated by traditional healers, and only 50.8% believed hospital drugs were of any use; and 62.7% of the respondents would not allow an epileptic child to go to school for various reasons, including mental subnormality (54.0%), fear of the child falling while alone (65.9%), and fear that the epileptic child would infect other children (11.2%). Concerning what is to be done when a seizure occurs, 33.5% of the respondents would keep away and not touch the person; 16.5% would take some potentially harmful measure such as forcing a mouth gag or forcing a drink such as water (1 even mentioned urine); 5.2% would take unnecessary measures such as rushing the patient to a hospital. Only 35.7% of respondents would perform at least some of the currently recommended first-aid measures.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Knowledge, attitude, and practice toward epilepsy among rural Tanzanian residents. 824 50

The charts and cranial ultrasounds of 29 infants treated with extracorporeal membrane oxygenation (ECMO) for respiratory insufficiency secondary to meconium aspiration syndrome, primary pulmonary hypertension, congenital diaphragmatic hernia and/or sepsis were examined to identify ultrasound abnormalities. Seventeen (58.6%) developed extra-axial fluid collections, only two of which were progressive. Ten (34.5%) developed evidence of intracranial hemorrhage (ICH): seven caudate, one each in the thalamus, parietal and occipital lobes. Eight (27.65%) of the neonates had seizures while on ECMO, 5 of whom had concurrent ICH.
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PMID:Ultrasound abnormalities in term infants on ECMO. 832 5

The most common anomalies of the fetal ventral abdominal wall include omphalocele and gastroschisis. Umbilical cord hernia is another abdominal wall defect that is poorly defined and usually mistakenly considered as a small omphalocele. The present report describes the sonographic features and clinical significance of four cases of umbilical cord hernia identified transvaginally in the early second trimester of pregnancy. These cases seemed to present a different entity from that of simple omphalocele. The transvaginal sonographic approach provided a clear image of the midgut protruding into the umbilical cord, precise localization of cord insertion in the region of the umbilical ring. Doppler flow evaluation of the umbilical vessels and their relation to the protruding mass. Serial sonographic observations revealed a stable umbilical cord mass in three fetuses, and an enlarging mass in one. Normal karyotype was determined and no associated malformations were detected prenatally. However, in one case that underwent immediate correction of the hernia after delivery at term, the neonate was subsequently found to have pulmonic stenosis and severe neonatal seizures developed at 4 months of age. In two cases, pregnancy was terminated due to parental request. The fourth fetus was delivered vaginally at term and catastrophic division of the umbilical cord containing a loop of small intestine was avoided only by the diligent observation of the midwife. We suggest that umbilical cord hernia is a distinct anomaly originating at a different stage of embryogenesis, thereby having a unique clinical significance, unlike simple omphalocele. Umbilical cord hernia should therefore be defined and considered as a separate entity.
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PMID:Fetal midgut herniation into the umbilical cord: improved definition of ventral abdominal anomaly with the use of transvaginal sonography. 859 Jan 88

Pregnancy in woman with epilepsy arouses several serious medical problems and always belongs to the group of high obstetric risks. The aim of the present clinical study was the evaluation of the antiepileptic treatment efficiency during pregnancy, including risk factor, effects on pregnancy and delivery in epileptic patients. The study group consisted of 84 epileptic pregnant women which delivered between 1992-1998 in Obstetric Departments of University Medical School of Lublin. A randomised group 80 healthy pregnant women constituted the control group. The mean age of the analysed patients was 25 years. 51 epileptic patients were pregnant for the first time, 23 patients for the second time and 10 patients for the third time or more. The mean duration time of the disease was 8.6 years. In our study group: 45 (53.8%) patients experienced primary generalized tonic-clonic seizures and 39 (46.6%) patients experienced partial seizures. 26 patients were treated with monotherapy and the rest with polytherapy methods. The estimation of the seizure frequency during pregnancy in 52 (61.9%) patients did not change, in 13 (15.4%) patients increased. Among obstetric complications: urinary tract infections, hypertonia (EPH-gestosis) were observed. In 4 newborn congenital defects have been noted. Mothers of three of them were treated with Phenydantin (heart lesion, developmental anomaly of fingers). The fourth mother used Convulex (meningoarachnided hernia, hydrocephalus).
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PMID:[Analysis of epileptic pregnant women delivering between 1992-1998 in obstetric departments of the University Medical School in Lublin]. 1204 3

6q-syndrome is a rare disorder characterised by a combination of anatomic anomalies, and mental and motor retardation due to a monosomy or trisomy 6q. So far only 12 suspected cases of monosomies 6q have been reported. Hearing loss does not seem to be characteristic for this syndrome. We present the case of a girl with partial monosomy 6q. A bilateral severe sensory hearing loss was confirmed by subjective and objective audiometry at the age of 12 years. The girl was successfully equipped with hearing aids. Other features of the syndrome, i.e. mental retardation, microcephaly, asymmetric face, broad nasal bridge, hypertelorism, epicanthus, strabism, high arched palate, ventricular septum defect and seizures were seen. Additionally, a tetraplegy and diaphragmal hernia had been diagnosed. The girl was equipped with a gastrostomy tube because of nutritional disorders. In the literature, the possibility of hearing disorders in monosomy 6q is rarely mentioned, although limited verbal speech skills have been reported. A syndromic character of hearing disorders in 6q-syndrome cannot be excluded. We advise detailed and early audiological testing of children with monosomy 6q.
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PMID:Severe sensory hearing loss in del(6q)-syndrome. 1459 81

Wolf-Hirschhorn syndrome (WHS, OMIM 194190) is a chromosomal disorder characterized by retarded mental and physical growth, microcephaly, Greek helmet appearance of the facies, seizures/epilepsy. Closure defects of lip or palate, and cardiac septum defects occur in 30-50% of cases. Its cause is a deletion in the short arm of chromosome 4. We present a male patient, born after 37 weeks gestation, as the fourth pregnancy of non-consanguineous healthy parents, with unilateral cleft lip and palate, hypertelorism, a right-sided ear tag, and mild epispadias. At age 10 weeks he developed acute respiratory distress and acute bowel obstruction requiring emergency laparotomy. This revealed a left-sided posterolateral diaphragmatic defect, type Bochdalek, with incarceration of the small intestines necessitating major bowel resection. Clinical genetic investigation suggested a chromosome anomaly, but regular karyotyping was normal. However, FISH analysis showed a microdeletion in the short arm of chromosome 4 (4p-), consistent with WHS. A combination of this syndrome with congenital diaphragmatic hernia (CDH) has been rarely described. CDH can present either as an isolated defect at birth, or with multiple congenital abnormalities, or as part of a defined syndrome or chromosomal disorder. Therefore CDH, although not common in WHS, can lead to its diagnosis relatively early in life. We strongly recommend a clinical genetic evaluation of each CDH patient with facial anomalies taking into consideration 4p- deletion syndrome.
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PMID:Early diagnosis of Wolf-Hirschhorn syndrome triggered by a life-threatening event: congenital diaphragmatic hernia. 1510 10


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