Annotation Intrauterine subdural hemorrhage Cigdem Inan Akman* MD, Chief Resident; Joan Cracco MD, Professor of Neurology, Division of Pediatric Neurology, State University of New York, Health Science Center at Brooklyn, New York, USA. *Correspondence to first author at Harvard Medical School, Children’s Hopital, Clinical Neurophysiology, 300 Longwood Avenue, Boston MA 02115, USA. E-mail: akman@A1.harvard.tch.edu Subdural hemorrhage (SDH) detected postnatally in the newborn infant is usually related to trauma at the time of vaginal delivery1. With improvement in obstetric methods, the incidence of this problem has declined. However, with advances in obstetric ultrasonography, it has been recognized that SDH may occur in utero before the onset of delivery. Intrauterine SDH remains a rare event. A review of the literature revealed only 32 cases to date (Tables I and II). Neonatal SDH is venous in origin and associated with trauma in most cases. Etiopathogenesis of neonatal SDH is explained by shearing of bridging veins or other venous structures caused by trauma1. Instrumental delivery (forceps or vacuum extraction) causes distortion of the infant’s cranium with elongation of the falx and angulation of the tentorium, leading to tearing of posterior fossa venous structures and bleeding into the subdural space. Rupture or tearing of large venous channels, such as the great vein of Galen, straight or transverse sinus or falx laceration with rupture of the sagittal sinus, result in accumulation of blood in both the supratentorial and infratentorial compartments1–3. However, etiopathogenesis of intrauterine SDH is less certain. The fetus is usually well protected from direct abdominal trauma by maternal structures and amniotic fluid. Fetal intracranial vessels may be susceptible to shearing or acceleration/deceleration forces because of the following features: first, the head is large and neck muscles weak; this allows more rotational movement with angular acceleration. Second, the subarachnoid space is larger, allowing the brain to move within the cranium easily. Third, the fetal brain has a higher water content that increases its mass and allows it to develop more momentum when acceleration is applied3, 4. These unique conditions could make the fetus more susceptible to developing subdural hematomas and allow the development of subdural hematomas without obvious trauma. Maternal trauma such as falls, assaults, or motor vehicle accidents, is the best-documented cause of SDH in the fetus before the onset of labor1,4. Although trauma to the fetal head is the most common factor involved in intrauterine SDH, consideration should be given to other potential contributing factors such as fetal or maternal thrombocytopenia5,6, coagulopathies secondary to hemophilia7–9, hepatic disease or hydrops fetalis6, and intrauterine infections or sepsis with disseminated intravascular coagulopathy8,10. Exposure to various drugs, especially aspirin and other anticoagulants11, and congenital vascular malformation12 have also been reported in association with fetal SDH (see Table II). It should be noted, however, that abnormalities of the complete blood count and coagulation profile may not be primary but secondary to consumption of blood and clotting factors within an expanding intracranial hematoma. In our case, fetal SDH occurred without any evidence of obvious causes or predisposing risk factors (see case report below). This condition is defined as spontaneous intrauterine SDH12–18. There are only seven reported cases of intrauterine spontaneous SDH in the world literature in addition to our own (see Table I). Mac Donald reported the first case of spontaneous intrauterine SDH in 197713. Of the five other case reports of spontaneous intrauterine SDH, two were stillborn16, 18; two died in the early neonatal period14, 17, and only one had normal development12 (see Table I). Gunn performed postmortem studies on 20 stillborn infants with unexplained intracranial hemorrhage delivered by mothers from the Pacific Islands19. He observed that all stillborn infants died unexpectedly before the onset of labor and had various degrees of intracranial hemorrhages. A subdural component was noted in 16 infants, half were located bilaterally. Hemosiderin deposition confirmed that the hemorrhages occurred before death in utero. Although etiology was unknown, the possibility of trauma was considered because of the history of abdominal massage to pregnant woman by traditional Pacific Islander healers. Subsequently, he reported three live-born infants with unexplained intrauterine SDH14. Due to the mothers of these two newborn infants also being immigrants from Pacific Islands, the same risk factor was considered as a cause of intrauterine SDH in those cases as well. CASE REPORT We recently encountered a neonate with extensive unilateral SDH present before delivery. This male infant was delivered by Cesarean section at 34 weeks’ gestation because of fetal distress and suspected hydrocephalus. Obstetric sonogram at 28 weeks’ gestation was normal, but a subsequent scan at 32 weeks’ gestation was reported as showing hydrocephalus. The Developmental Medicine & Child Neurology 2000, 42: 843–846 Downloaded from https://www.cambridge.org/core. University Library Frankfurt, on 30 Jan 2018 at 15:59:35, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms. https://doi.org/10.1017/S0012162200001559 843 and the head circumference reduced to 34 cm. At 4 days of life, external drainage was discontinued. Head circumference remained stable. At 13 days, CT showed minimal subdural collection without midline shift. Repeat blood count, PT, thrombin, PTT, and fibrinogen were normal. TORCH studies, blood, and urine cultures were negative. The etiology of SDH was unknown. The infant’s development, assessed at 2 and 4 months, was normal. At 11 months of age, he had three brief generalized seizures. An EEG revealed spikes over the left hemisphere with slow background activity. Neurological examination was normal except for questionable speech delay. A repeat CT showed chronic right residual subdural effusion and minimal atrophy of the left hemisphere with left periventricular leukomalacia (Fig. 1b). At present the child is 21⁄2 years old and has moderate speech delay. His seizures are controlled with phenytoin. mother stated that there had been no trauma, infection, or drug ingestion. Her prothrombin time (PT)/partial prothrombin time (PTT), platelet count, Hb, and hematocrit (Hct) were normal. Apgar scores were 9 and 9 at 1 and 5 minutes, respectively. The child’s head circumference was 35.5 cm (>95th centile); the anterior fontanel was slightly bulging. No lacerations or hematomas were noted. He was active and alert. Right-sided ptosis and a mild left hemiparesis were present. Initial laboratory data showed the following: Hb 10 gr/dL, Hct 35%, plt 256/cu mm. PT was 16.5 seconds (12 to 21 s for preterm baby) and PTT was 58.3 s (70 s for preterm baby). Head CT on day 1 showed a right-sided extensive SDH with marked midline shift and transtentorial herniation (Fig. 1a). Burr holes were performed and 85 cc of ‘motor oil’ type subdural fluid was drained. Over the next 24 hours, an additional 65 cc was drained. The anterior fontanel became depressed Table I: Spontaneous subdural hemorrhages Authors n Gestation Apgar (wk) 1,5 min Clinical signs CT findings Site Location CBC Mac Donald 197713 Gunn 198514 Mateos 198713 Demir 198918 Nougeria 199215 1 1 1 1 1 38 42 35 33 37 2, 6 9, 9 7, 9 – 9, 10 Lethargy L, ptosis Hypotonia – Macrocephaly BL L R L L Convexity Convexity Convexity Convexity Convexity Normal Normal Normal – Normal Kawabata 199316 Sohda 199612 1 1 35 37 – 8, 10 – Macrocephaly R Convexity R Convexity Post fossa – Normal Therapy Outcome Subdural tap Hypotonia at 6 mo Subdural tap Died at 3rd day Surgical evacuation Died at 14th day – Stillbirth Surgical evacuation Spastic diplegia Moderate developmental delay – Stillbirth at 35 wk Subdural tap Normal at 13 mo CBC, complete blood count; L, left; R, right; BL, bilateral; post, posterior; –, not available. a b Figure 1: (a) CT without intravenous contrast taken immediately after birth showing large right isodense subdural hemorrhage with midline shift and transtentorial herniation. (b) At 11 months of age, minimal right chronic subdural collection and diffuse tissue loss of left cerebral hemisphere are present. Note paucity of white matter at left posterior cerebral hemisphere compatible with periventricular leukomalacia. 844 Developmental Medicine & Child Neurology 2000, 42: 843–846 Downloaded from https://www.cambridge.org/core. University Library Frankfurt, on 30 Jan 2018 at 15:59:35, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms. https://doi.org/10.1017/S0012162200001559 extension of intracranial hemorrhages in both the prenatal and neonatal period. MRI is better for posterior fossa SDH because it delineates the posterior fossa elements more effectively than a CT scan. Advanced neuroimaging techniques also provide the advantage of estimating the age of the hemorrhage to delinate underlying causes. CLINICAL ASPECTS The two principal locations of SDH in the fetus and newborn infant are over the cerebral hemisphere and posterior fossa1. Review of the literature showed that SDH in the fetus appeared to be more common supratentorially rather than infratentorially, which is opposite to SDH in the newborn infant due to traumatic delivery (see Table I and II). Clinical presentation depends on the size and localization of the hemorrhage i.e. the degree to which it is confined to one area. Large SDHs, especially in the posterior fossa, cause focal neurological symptoms and signs at an early stage due to increased intracranial pressure and brain-stem compression21–23. Massive hemorrhage may be associated with shock, coma, and rapid demise. In contrast, infants with small hemorrhages may have mild symptoms or no symptoms at all. Asymptomatic increased head size might be the only finding either pre and/or postnatally. Therefore, SDHs should be included in the differential diagnosis of macrocephaly at birth (see Table I and II). As small asymptomatic SDHs may not be detected, it is difficult to determine the incidence of intrauterine SDH accurately. Owing to improvements in in utero imaging techniques, smaller SDHs have been recognized more frequently in recent years1, 9. The ultrasonogram may give a false negative or an erroneous result. In our case, the sonogram was interpreted as showing hydrocephalus and SDH was not suspected. However, fetal hydrocephalus is not surprising in association with intrauterine SDH5, 11, 24. Ultrasonographic demonstration of increase in ventricular size associated with or without an abnormal intracranial mass should encourage further diagnostic imaging. Either the study should be repeated or other imaging techniques should be used until the intracranial structures are clearly delineated. CT and MRI16 provide better visualization of the distribution and MANAGEMENT AND OUTCOME In the absence of extensive experience with intrauterine SDH, it is essential to optimize close antenatal follow up and delivery management in the expectation of fetal survival. Cesarean section may be necessary because of considerable head enlargement. After birth, as in the cases of postnatal SDH, the localization and extension of hemorrhage and the clinical condition of the newborn infant are the essential factors in deciding on surgical or conservative management. Review of the literature on all SDHs and our patient demonstrate that prompt treatment is indicated in patients who present with increased intracranial pressure and clinical deterioration5–7, 12, 14. Surgery can be life saving when there are signs and symptoms of brain-stem dysfunction. Indications for surgery in the case of posterior fossa SDH, outlined by Tanaka and Govaert2 are: (1) signs of brain-stem compression; (2) acute obstructive hydrocephalus with raised intracranial pressure; (3) a posterior fossa clot >15 cm. There should be no delay if the newborn infant has clear neurological deterioration. If the posterior fossa hemorrhage is small and not expanding surgical intervention is not necessary. On the other hand, acute convexity SDH requires careful observation2. Surgery is not mandatory if the infant remains neurologically stable. Subdural taps can be used to reduce intracranial pressure. However, surgery should be considered in the presence of a large hemorrhage, if there are signs of increased intracranial pressure and neurological deficits Table II: Intrauterine subdural hemorrhages with risk factors Authors n Robinson 1 1980 (11) Gunn 16 1984 (19) Gunn 1 1985 (14) 1 Hanigan 1985 (6) Atluru 1987 (5) De Sousa 1988 (7) 1 1 Risk Gestation Apgar (wk) 1,5min Warfarin (mother) Abdominal massage Abdominal massage Abdominal massage Coagulopathy Hydrops Low PLT Clinical CT findings signs Site Location CBC Therapy Prognosis 34 – Shock BL Convexity – Supportive therapy Died at 3 hr – – – – – Stillbith Normal – Quadriparesis Normal Surgical evacuation Covexity Abnormal PT/PTT BL Convexity Low PLT Subdural tap Microcephaly Developmental delay Normal at 3 mo Subdural tap Spastic quadriparesis 40 7,8 Macrocephaly BL Convexity (n=8) BL Covexity 41 5,8 Macrocephaly BL Convexity 38 6,5 BL 43 Hydrops 1 Factor X deficiency 37 9,10 Macrocephaly R hemiparesis – – Rotmensch 1 1991 (8) Catanzarite 1 1995 (20) Barozzino 1 1998 (9) Coagulopathy 37 3,5 Macrocephaly Asphyxia 28 – – Covexity Abnormal Subdural tap Normal development PT/PTT Fresh frozen plasma Died at 7 mo from acute hemorrhage BL Convexity Abnormal Supportive therapy Died at 6 hr Post fossa PT/PTT BL Covexity Anemia Supportive therapy Stillbirth Maternal factor XI deficiency 30 8,9 – BL Convexity L Normal Spontaneous resolution Normal at 9 mo CBC, complete blood count; PT, prothrombin time; PTT partial prothrombin time;PLT, platelet; –, not available. Annotation Downloaded from https://www.cambridge.org/core. University Library Frankfurt, on 30 Jan 2018 at 15:59:35, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms. https://doi.org/10.1017/S0012162200001559 845 indicating transtentorial herniation2, 25. Prognosis of the fetus affected by SDH is dependent on the severity of the hemorrhage and the underlying cause. The prognosis has generally been poor both in the cases of SDH with known risk factors and in the group with spontaneous SDH (see Table I and II). Of the 32 reported cases of intrauterine SDH, only 13 of the affected infants were live-born2–6, 8–11, 13, 15. Four of these infants died during the neonatal period8, 11, 14, 17, five had neurological impairment3, 13–15, and four had normal development6, 7, 9, 12. Unfortunately, there is no information on long-term followup of these individuals. It is of interest that spontaneous intrauterine resolution of SDH was observed in one infant9. Conclusion Etiology of intrauterine SDH is unknown in many cases. With wider use and improved obstetric imaging techniques, intrauterine SDHs are being recognized more often. Ultrasonography, along with other imaging modalities may help in identifying specific etiology and defining optimal management of this problem. Early diagnosis and appropriate treatment should improve survival and minimize brain damage in these newborn infants. Optimal management and outcome of these infants will require close cooperation between obstetricians, neonatologists, neurologists, and neurosurgeons. References 1. Volpe JJ. (1995) Intracranial hemorrhage: subdural, primary subarachnoid, intracerebellar, intraventricular (term infant), and miscellaneous. In: Volpe JJ, editor. Neurology of the Newborn. Philadelphia: WB Saunders. p 373–402. 2. 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(1984) Subdural hematomas of the posterior fossa in normal-weight newborns. Journal of Neurosurgery 61: 972–4. Developmental Medicine & Child Neurology 2000, 42: 843–846 Downloaded from https://www.cambridge.org/core. University Library Frankfurt, on 30 Jan 2018 at 15:59:35, subject to the Cambridge Core terms of use, available at https://www.cambridge.org/core/terms. https://doi.org/10.1017/S0012162200001559