Perinatal/Neonatal Case Presentation

Transcription

1 Perinatal/Neonatal Case Presentation & & & & & & & & & & & & & & Bilateral Thalamic Lesions in a Newborn with Intrauterine Asphyxia After Maternal Cardiac Arrest a Case Report with Literature Review Maya Caroline Banerjea Christian P. Speer Hypoxic ischemic brain damage in preterm and term infants is one major cause of neonatal neurologic morbidity. Depending on the gestational age and the extent of hypoxia, different pathologic findings have been observed. Hypoxic ischemic lesion of the thalamus is the least common form of cerebral injury. Although long- term outcome with spastic or extrapyramidal cerebral palsy is known, clinical features in the neonatal period are not well described. We report an infant with bilateral hypoxic ischemic thalamic lesions after maternal cardiac arrest at 28 weeks of gestation. Clinical features and diagnostic results of our patient are compared to information given in the literature to define the clinical entity of hypoxic ischemic thalamic lesions in neonates better. Journal of Perinatology 2001; 21: INTRODUCTION Hypoxic ischemic brain damage in preterm and term infants is one major cause of neonatal neurologic morbidity. Depending on the gestational age and the extent of hypoxia, different pathologic findings have been observed. In premature infants periventricular leukomalacia is the dominating form of cerebral injury whereas parasagittal cerebral injury and status marmoratus of basal ganglia and thalamus occur more often in mature infants. 1 Hypoxic ischemic lesion of the thalamus is the least common form of cerebral injury, the exact number of infants affected is unknown. 1 Although long-term outcome with spastic or extrapyramidal cerebral palsy is known, clinical features in the neonatal period are not well described. Childrens Hospital, Department of Neonatology, University of Wuerzburg, Wuerzburg, Germany. Address correspondence and reprint requests to M. C. Banerjea, MD, Childrens Hospital, Department of Neonatology, University of Wuerzburg, Josef - Schneider - Strasse 2, D Wuerzburg, Germany. Journal of Perinatology 2001; 21: # 2001 Nature Publishing Group All rights reserved /01 $17 We report an infant with bilateral thalamic lesions after an antenatal hypoxic ischemic insult in the 28th week of gestation. Detailed information about clinical features and diagnostic findings are described to define the clinical entity of hypoxic ischemic thalamic lesions better. CASE REPORT N. C. was born to a 29-year-old primipara after 35 weeks of gestation. The pregnancy was complicated by a maternal cardiac arrest due to decompensation of a combined aortic valve malformation at 28 weeks of gestation. The mother was found lying unconscious on the street and resuscitation was started immediately. The exact duration of cardiac arrest had been unknown. On admission she was able to walk and to communicate appropriately. Because of severe hypotension requiring inotropic medication she was electively intubated several hours later and was ventilated for 1 day. She recovered quickly without neurologic sequelae and the pregnancy was carried on. Obstetric examination, electronic fetal monitoring, and fetal ultrasound examination were done repeatedly with unremarkable results. No polyhydramnion was reported. Following uncomplicated labor at 35 weeks gestation, N. C. was delivered vaginally (birth weight 2035 g, head circumference 31 cm, both 10th percentile). The APGAR scores were 8, 10, and 10 at 1, 5, and 10 minutes, respectively. Labor and delivery were closely monitored and there were no signs of intrapartal asphyxia (umbilical arterial cord gases: ph 7.32, pco 2 44 mm Hg, po 2 15 mm Hg, base excess 4 mmol/l). Post delivery the infant showed signs of a mild and transient respiratory distress requiring nasal CPAP with a maximum inspiratory concentration of 30% to 40% oxygen. The neurologic findings and information regarding labor and delivery are summarized in Tables 1 and 2. Until the age of 5 months N. C. stayed in the neonatal intensive care unit because of major feeding problems and frequent aspiration pneumonias requiring a percutaneous endoscopic gastrostomy. He is now 3 years old and has developed extrapyramidal cerebral palsy with a strong dystonic component and severe mental retardation. Cerebral ultrasound (days 1, 2, 3, 6, 7, 14, 31): normal. Day 45: bilateral hyperechoic echogenicity in the posterior part of the thalamus (Figure 1). Otherwise normal examination without signs of hemorrhage or periventricular leukomalacia, respectively. 405

2 Banerjea and Speer Bilateral Thalamic Lesions in a Newborn Table 1 Clinical Features in Hypoxic Ischemic Thalamic Lesions ( Case of Our Patient and Literature Review) Sucking Absent Absent: 34/ 34 Swallowing Absent Absent: 34/ 34 Facial expression Weak Weak: 15/ 34 Normal: 11/ 34 I.m.*: 8/ 34 Retrognathia + 3/ 34 I.m.: 31/ 34 Muscular tonus at birth Hypotonic Hypotonic: 14/ 20 survivors; hypertonic: 6/20 survivors; I.m. 14/34 Type of cerebral palsy Spastic + y Extrapyramidal + + y Eye movement and Normal Normal: 4/34; abnormal: 16/34; I.m.: 14/34 pupil s reflexes Seizures? ( tonic jerking) 23/ 34; I.m.: 11/ 34 Temperature regulation Pathologic ( intermittent episodes I.m. of increased temperature without adequate explanation) Need for ventilation: No (N-CPAP for 44 days) 29/34 Death / 20/34 (2 days to 7 months) z Only case reports with information regarding obstetric history, labor, delivery, and postnatal course and with confirmation of thalamic lesions by CT, MRI, or autopsy are included. Infants with malformations, metabolic, or genetic disorders are excluded ( Tables 1 and 2). *I.m., information missing. y Exact number of cases with different types of cerebral palsy is missing. z Cause of death: pneumonia 3/20, respiratory arrest 1 /20, withdrawal of intensive care 3 /20, unknown 13 /20. Cranial CT: not performed. Head MRI (day 20): bilaterally increased signal intensities both in T1- and T2-weighted images of the lateral and posterior thalamic nuclei (Figure 2). These findings are compatible with hypoxic ischemic lesions in the thalamus. Otherwise normal examination. Day 80: Identical signal intensities in the lateral and posterior thalamic nuclei. Myelinization is adequate for age. EEG (days 14, 30, 60): normal. Brainstem auditory evoked potentials (BAEP): mild to moderate conductive hearing loss of 45 db. Urinary amino acids/serum organic acids: normal. An overview comparing diagnostic tests in our patient and information provided in the literature are summarized in Table 3. DISCUSSION Intrauterine or perinatal hypoxic ischemic insults leading to thalamic lesions are rare. The exact number of infants with these specific lesions is unknown but it is the least common pattern of brain injury. 1 Although long-term morbidity with spastic or Table 2 Information Regarding Obstetric History, Labor, and Delivery of the Patients Included in the Review Gestational age (wk) (27 41)* Birth weight ( g) ( ) Presumed timing of asphyxia yz Antepartum + 6/ 34 Intrapartum 24/34 y Unknown 4/34 Cause of hypoxic ischemic insult Maternal cardiac arrest Placental abruption: 2/ 34; umbilical cord prolapse: 4/ 34; uterine rupture: 2/ 34; torn umbilical vein: 1/ 34; maternal trauma: 1/ 34; respiratory arrest after Streptococcus A infection: 1/34; unknown: 23/34 Polyhydramnion Absent Present: 6/ 34; absent/ unknown: 28/ 34 *Only one infant was less than 33 weeks gestation at birth. y Twenty children were included in the literature review that were preselected ( study of Roland et al. 4 ) insofar as only term babies with proven intrapartum asphyxia and specific CT findings were included. z Determined by obstetric history, history of labor and delivery, mode of delivery, APGAR values, and presence of spasticity at birth. 406 Journal of Perinatology 2001; 21:

3 Bilateral Thalamic Lesions in a Newborn Banerjea and Speer Figure 1. Ultrasound imaging ( day 45). Coronal and sagittal image. Note the increased echogenicity in the thalamic area. The ventricles appear normal. There is no intraventricular hemorrhage and no evidence of periventricular leukomalacia. extrapyramidal cerebral palsy is known, the clinical symptomatology in the neonatal period is not well described. Comparing clinical features of our patient with information given in the literature, we think that hypoxic ischemic thalamic lesions represent a distinct clinical entity that should be considered if specific neurologic findings are present. Children with hypoxic ischemic thalamic lesions are usually born at term although cases about prematurely borne infants exist. 2,3 They typically show signs of respiratory distress often requiring mechanical ventilation. The neurologic examination characteristically shows absent sucking and swallowing whereas other brainstem nerves are unaffected. Because of the impairment in swallowing a history of antenatal polyhydramnion is frequent. Without exception the infants have major feeding problems requiring feeding by nasogastric tube. Seizures in the neonatal period seem to be a common finding although symptoms may be discrete. Detailed information about types of seizures is missing. Hyperthermia in children with thalamic lesions has not been described yet. In our case we could observe periods of increased temperature (38.58C) without adequate explanation. We cannot Figure 2. MRI (day 20). Axial T1 (TR/TE 776/11 milliseconds) and T2 (TR/TE 2300/80 milliseconds) weighted images. Note bilateral, triangularshaped hyperintensity in the lateral and posterior thalamic nuclei. The cortex and white matter appear normal. Journal of Perinatology 2001; 21:

4 Banerjea and Speer Bilateral Thalamic Lesions in a Newborn Table 3 Diagnostic Findings in Hypoxic Ischemic Thalamic Lesions ( Case Report and Literature Review) Sonographic findings Bilaterally increased echogenicity in the posterior part of the thalamus (day 45 of life) Normal (day 1 7): 6/34; bilaterally increased echogenicity in thalamic region (day 1 5): 4/34; I.m.*: 24/34 EEG Normal (days 14, 30, 60 of life) Normal (days 1 7): 4/34; unspecific changes: 22/34; yz seizure activity (day 28): 1/34 I.m.: 7/34 CT Not done Normal: 0/ 34; bilaterally increased echogenicity in thalamic region ( days 5 28): 5/ 34; bilaterally decreased echogenicity in thalamic region (days 1 7): 23/34; x bilateral thalamic calcifications: 3/34; I.m.: 3/34 MRI Bilateral hyperintensity of the lateral thalamic nuclei (T1 and T2) (day 20 and 80) Normal (day 4): 1/34; bilateral hyperintensity in the thalamus and basal ganglia and/ or atrophy of the dorsal part of the brainstem: 4/34; y I.m.: 29/34 BAEP Mild to moderate conductive hearing loss (45 db) Normal: 0/34; abnormal: 2/34; y,k I.m.: 32/34 *I.m.: Information missing. y Age at examination missing. z Multifocal paroxysmal discharges without definite seizure activity, slow background activity. x Twenty of these children were preselected ( study of Roland et al. 4 ) insofar that only term babies with proven intrapartum asphyxia and decreased tissue attenuation on CT examination were included. k Only wave I at 95 db, wave I and a very low amplitude of wave II at 110 db. exclude the possibility that these episodes were caused by a disturbance in thalamic function. Besides the characteristic absence of sucking and swallowing, infants with thalamic lesions often show facial weakness and generalized hypotonia. Some authors, however, have observed hypertonia or even spasticity at birth. 3,5 7 The presence of hypotonia or hypertonia, respectively, might vary depending on the time of occurrence of ischemia and the extent of hypoxic injury. Different diagnostic findings in hypoxic ischemic thalamic lesions have been described but a systematic evaluation of these examinations in their reliability, reproducibility, and accuracy is missing. The optimal age for different diagnostic testing has yet to be defined. Ultrasound imaging may show bilateral areas of hyperechoic echogenicity in the thalamic region but changes may be subtle and may not be present at birth. In cases of definite thalamic hyperechoic echogenicity the distinction between hypoxic ischemic lesion and hemorrhagic lesion may be difficult. Information regarding cerebral CT findings in hypoxic ischemic thalamic lesions is available but is inconsistent. Some authors describe hypodensities 2,6 whereas others describe hyperdensities with 3,8 or without calcifications. 5 7,14 The largest study 9 about CT findings in children with bilateral thalamic lesions includes 20 infants with proven intrapartum asphyxia and hypodensities on CT examination. The CT studies were performed at a mean age of 61 hours. We assume that the appearance of hypo- versus hyperdensic lesions depends on the current stage of pathology. It may be possible that hypodense lesions are found in the stage of thalamic edema whereas hyperdensities are found when reactive gliosis is already present. Follow- up examinations in short intervals documenting changes in signal intensities would give valuable additional information. MRI findings are described as hyperintensities both in T1- and T2-weighted images in the thalamic region, globus pallidus, putamen or brainstem, respectively. 9,10 Comparison between CT and MRI in selected cases 9 suggests that MRI might provide more information about the extent of brain injury. The impaired function of lower brainstem nerves suggests that not only the thalamus but also the brainstem is affected by hypoxic ischemic damage. 4 The prognosis for infants with hypoxic ischemic thalamic lesions appears to be uniformly poor. Twenty of 34 children described above died between the first week and 7 months of age, some as a result of pneumonia following aspiration. Because severe obstructive apneas are a common finding, home monitoring of respiratory function and oxygen saturation should be considered. All of the surviving infants have developed signs of either spastic or extrapyramidal cerebral palsy. The frequency of these different types of cerebral palsy is unknown. Hypoxic ischemic thalamic lesions have to be distinguished in their prognosis from hemorrhagic lesions. Hemorrhagic lesions that appear spontaneously without preceding asphyxia seem to have a better prognosis 11 whereas thalamic hemorrhages following severe asphyxia seem to have a similarly poor prognosis as hypoxic ischemic lesions alone. 12 The rate of mental retardation is unknown but appears to be impressive. 13 In view of the poor prognosis there have been reports about withdrawal of intensive care in infants with ventilator dependency. 6 Acknowledgements The authors thank both K. Drews, MD, Department of Radiology, University of Tuebingen, and M. Bitzer, MD, Department of Neuroradiology, University of Tuebingen, for performing the excellent ultrasound scans and neuroimaging studies on our patient and for their advice in selecting the adequate images. 408 Journal of Perinatology 2001; 21:

5 Bilateral Thalamic Lesions in a Newborn Banerjea and Speer References 1. Volpe JJ. Neurology of the newborn. Third Edition. Philadelphia: WB Saunders; p Cohen M, Roessmann U. In utero brain damage: Relationship of gestational age to pathological consequences. Dev Med Child Neurol 1994;36: De Vries LS, Eken P, Groenendaal F, Rademaker KJ, Hoogervorst B, Bruinse HW. Antenatal onset of hemorrhagic and/ or ischaemic lesions in preterm infants: prevalence and associated obstetric variables. Arch Dis Child Fetal Neonat Ed 1998;78:F Roland EH, Hill A, Norman MG, Flodmark O, MacNab AJ. Selective brainstem injury in an asphyxiated newborn. Ann Neurol 1988;23: Bordarier C, Moktari M, Rodriguez D, Adamsbaum C, Robain O. Antenatal thalamic lesions in the newborn: two anatomoclinical cases. Brain Dev 1997;19: Eicke M, Briner J, Willi U, Uehlinger J, Boltshauser E. Symmetrical thalamic lesions in infants. Arch Dis Child 1992;67: Parisi JE, Collins GH, Kim RC, Crosley CJ. Prenatal symmetrical thalamic degeneration with flexion spasticity at birth. Ann Neurol 1983;13: DiMario F, Clancy R. Symmetrical thalamic degeneration with calcifications of infancy. Am J Dis Child 1989;143: Roland EH, Poskitt K, Rodriguez E, Lupton BA, Hill A. Perinatal hypoxic ischemic thalamic injury: clinical features and neuroimaging. Ann Neurol 1998;44: Natsume J, Watanabe K, Kuno K, Hayakawa F, Hashizume Y. Clinical, neurophysiologic, and neuropathological features of an infant with brain damage of total asphyxia type (Myers). Pediatr Neurol 1995;13: Trounce JQ, Fawer CL, Punt J, Dodd KL, Fielder AR, Levene MI. Primary thalamic haemorrhage in the newborn: a new clinical entity. Lancet 1985;Jan 26: Donn SM. Possible mechanisms of primary thalamic haemorrhage in newborn. Lancet 1985;Apr 6: Kyllerman M, Bager B, Bensch J, Bille B, Olow I, Voss H. Dyskinetic cerebral palsy: I. Clinical categories, associated neurological abnormalities and incidences. Acta Paediatr Scand 1982;71: Okumura A, Hayakawa F, Kato T, Kuno K, Watanabe K. Bilateral basal ganglia thalamic lesions subsequent to prolonged fetal bradycardia. Early Hum Dev 2000;58(2): Journal of Perinatology 2001; 21: