Clinical Brief Idiopathic Chronic Subdural Hematoma, MCA Infarct and Cortical Atrophy with Status Epilepticus in Infants Raj Kumar, Namit Singhal and A.K. Mahapatra Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India ABSTRACT Spontaneous chronic subdural hematomas in infants are extremely rare. A very limited number of cases are known and reported in literature. The clinical presentation can be myriad varying from asymptomatic cases to gross neurological deficits. We report the cases of 2 infants who presented to us with repeated episodes of generalized tonic clonic seizures since birth. No etiology could be established for either of them. Subsequent imaging revealed chronic subdural hematoma, MCA infarct and cortical atrophy in both the cases. Both the cases improved following evacuation of the chronic subdural hematoma. One of the infants had secondary craniosynostosis for which additional coronal suturectomy had to be done. The management of such cases and a brief review of literature are discussed. [Indian J Pediatr 2007; 74 (11) : 1046-1048] E-mail: rajkumar@sgpgi.ac.in Key words : Chronic subdural hematoma; Cortical atrophy; MCA infarction; Status epilepticus Chronic subdural hematoma of known etiology in neonates and infants is very rare and only limited cases have been reported in literature. Commonly associated factors include trauma during pregnancy, maternal coagulopathy, intrauterine infections, use of warfarin, metabolic disorders etc. However, spontaneous chronic subdural hematoma (CSH) without known etiology is extremely rare. Our literature search by electronic media revealed only 5 cases of chronic subdural hematoma without known etiology.1-5 Unrecognized in-utero trauma and a complex interaction of intracranial and intrauterine pressures seem to be a convincing explanation for such cases. Subtle degree of birth asphyxia could also be a possible etiological factor. This is stressed by presence of cortical infarct and atrophy in our two cases, the association of which has not been described in earlier case reports of idiopathic infantile chronic subdural hematoma. CASE REPORTS Case 1 A 4-month-old female child (6 Kg weight, born of normal vaginal delivery) was referred to us for complaints of Correspondence and Reprint requests : Dr. Raj Kumar, Professor, Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow -226014, U.P., India; Phone : 05222668700, 2668800, Fax : 91(522)2668129 and 2668017 [Received February 13, 2007; Accepted July 24, 2007] 1046 repeated episodes of generalized tonic clonic seizures since birth. The frequency of seizures increased over past 15 days to result into status epilepticus. She was put on phenobarbitone and phenytoin sodium (according to her body weight) by a pediatric physician, but the seizures remained uncontrolled. The antenatal history was insignificant and there was no history suggestive of birth trauma. However, the child did not cry for almost 8 minutes following birth and as such a diagnosis of birth hypoxia was made. The social, motor and language developmental milestones of the child were delayed. Physical examination revealed brachycephaly with overriding of parietal bones over frontal bones suggestive of secondary craniosynostosis. The head circumference was 37 cm and anterior fontenalle was fused. There was no apparent cranial nerve paresis. On gross examination, the motor power and bulk of all limbs were normal. Preliminary investigations revealed a low hemoglobin concentration (7g/dl), normal platelet counts, normal prothrombin time and a normal partial thromboplastin time. Skull X-rays with anterior-posterior, lateral, and Water views revealed prematurely fused coronal sutures and associated ridging of the suture line. The plain and contrast CT of head showed diffuse atrophy of brain parenchyma with prominent cisterns, suspected right middle cerebral territory infarction and bifrontal acute on chronic subdural hematoma, significant on left side in comparison to right causing a mass effect on left side (Fig. 1). A diagnosis of birth hypoxia, diffuse cortical atrophy, with left middle cerebral territory infarct and secondary craniosynostosis, mainly affecting the coronal sutures was made. Indian Journal of Pediatrics, Volume 74—November, 2007 Idiopathic Chronic Subdural Hematoma, MCA Infarct and Cortical Atrophy with Status Epilepticus Fig. 1. Plain CT head showing diffuse cortical atrophy with prominent cisterns and bifrontal acute on chronic subdural hematoma in the first infant (left more than right). Sodium valporate was added to the previous treatment, but seizures could not be controlled. Bleeding and clotting parameters were repeated and the child was taken up for surgery. A bifrontal wide craniotomy extending upto the frontal base with bicoronal suturectomy was done and the hematoma was evacuated. Postoperatively, the seizures were controlled on antiepileptic treatment. Case 2 A 3-month-old male child presented with complaints of repeated episodes of generalized tonic clonic seizures and right sided paucity of movements since birth. The seizures were controlled on phenytoin sodium started by a pediatric physician. He was born of a normal full term vaginal delivery with a birth weight of 3.2 Kg. The antenatal history was insignificant and there was no history suggestive of birth trauma or asphyxia. No resuscitation was required for the child immediately after birth. There was no history of parental coagulopathy. His head circumference was in the 90th percentile and the anterior fontanelle was full. Neurological examination showed paucity of movements with increased tone in the right sided limbs. Biochemical investigations revealed normal hemoglobin concentration, normal platelet counts, normal international normalised ratio and a normal partial thromboplastin time. CT scan was suggestive of bilateral acute on chronic subdural hematoma more on left side as compared to right side. There was presence of cortical atrophy along with chronic infarct in left MCA distribution (Fig. 2). The child was successfully treated with a simple burr-hole evacuation and external drainage. At the time of discharge from the hospital, the seizures were well controlled on same doses of antiepileptic drugs and weakness on the right side has improved. On follow up after 3 month, the subdural hematoma did not recur and a repeat drainage was not required. Indian Journal of Pediatrics, Volume 74—November, 2007 Fig. 2. Plain CT scan of the second infant showing bilateral acute on chronic subdural haematoma with cortical atrophy and chronic infarct in left MCA distribution DISCUSSION Spontaneous chronic subdural hematoma (CSH) without known etiology is extremely rare presentation in infants. There are only 5 case reports in the literature which have described this entity in new born babies.1-5 The diagnosis is established only after excluding known causes of chronic subdural hematoma in infants, most common being birth trauma, hemorrhagic diathesis and metabolic disorders.6, 7 Various possible etiological factors can be hypothesized for development of such entity in infants. Unrecognized in utero trauma and a complex interaction of intracranial and intrauterine pressures seem to be the most convincing explanation for such cases.2, 4, 5, 8 This is multifactorial in origin. Fragile cerebral vasculature of the infantile brain coupled with enlarged subarachnoid spaces, makes it prone to minor and unrecollected maternal abdominal trauma. Evidence of these factors coming into play is further stressed by associated cortical infarcts and atrophy in the two cases presented by us. Although, cerebral infarction has been reported in neonates with traumatic acute subdural hematoma, its association with infantile chronic subdural hematoma has not been described. Occlusion of major vessels does occur in head injury leading to cortical infarction. 6 The occlusion may follow rupture of the vessel, deranged microcirculation or arterial dissection. The cortical atrophy in our cases could be a result of either cerebral infarction or to accommodate an acute subdural hematoma, occurring at some stage on account of elevation of pressure at local site, without manifesting as raised intracranial pressure because of opened sutures. Cerebral atrophy of the ipsilateral hemisphere after acute subdural hematoma has been reported in infants.9 Other factors associated with the development of idiopathic chronic subdural hematoma in infants include 1047 R. Kumar et al sudden resolution of infantile hydrocephalus in cases with intermittent aqueductal stenosis and hydrocephalus,10 maternal use of anticoagulants, parental history of coagulopathy1, cryptic vascular malformations, subtle bleeding diathesis11, asymptomatic intrauterine infections, hypovitaminosis, premature closure of fetal foramen ovale causing cardiac and hepatic decompensation with increase in central venous pressure and secondary coagulopathy12 and associated arachnoid and neuroepithelial cysts.13, 14 The clinical presentation of infantile chronic SDH varies from asymptomatic cases to infants with gross neurological deficits. The presentation could be subtle manifesting as anemia only. Although seizures are common, presentation with status epilepticus has not been reported in literature. Secondary craniosynostosis, another rare manifestation of the disease, can be presumed to be because of atrophic brain parenchyma. It was present in the first infant resulting in clinically apparent cosmetic deformity. Management varies from simple burr hole evacuation to craniotomy and subdural- peritoneal shunt in recurrent cases. Prognosis is generally fair in these cases, provided early management is ensued. Acknowledgements The authors are extremely grateful to Mr. A. P. Dhar Dwivedi, Department of Neurosurgery, SGPGIMS, Lucknow, for secretarial assistance. REFERENCES Chronic subdural hematoma in infants is most commonly due to non accidental trauma, while acute SDH in neonates is most commonly due to birth related trauma. In contrast, idiopathic chronic SDH in neonates is a rare entity. Unrecognized in utero trauma seems to be the most common causative factor in such cases. Review of literature suggests that etiology in these cases is multifactorial, although the final common pathway is tearing of veins and accumulation of blood in subdural space. These infants must be thoroughly investigated to rule out known causative factors. Clinical presentation can be variable from asymptomatic cases to infants with gross neurological deficits, including rare presentation of secondary craniosynostosis due to cerebral atrophy. 1. Alturu VL, Kumar IR. Intrauterine chronic subdural hematoma with postoperative tension pneumocephalus. Pediatr Neurol 1987; 3: 306-309. 2. Breningstall B, Patterson RJ. Intrauterine subdural hematoma. Pediatr Radiol 2000; 30 : 630-631. 3. Diaz A, Taha S, Vinikoff L, Andriamamonjy C, Leriche B, Bintner M. Chronic subdural hematoma in utero. Case report with literature review. Neurochirurgie 1998; 44 : 124-126. 4. Gunn TR, Mok PM, Becrott DM. Subdural hemorrhage in utero. Pediatrics 1985; 76 : 605-610. 5. Hadzikaric N, Al-Habib H, Al-Ahmad I. Idiopathic chronic subdural hematoma in the newborn. Childs Nerv Syst 2006; 22 : 740-742. 6. Kumar R, Mahapatra AK. Head injury in children. In AK Mahapatra, Raj Kamal, eds. Head Injury 3rd ed. New Delhi; Modern Publisher, 2005; 253-270. 7. Miura T, Tanaka H, Yoshinari M et al. A case of scurvy with subdural hematoma. Rinsho Ketsueki 1982; 23 : 1235-1240. 8. Franklin J. Posterior fossa chronic subdural hematoma in the neonate. AJNR Am J Neuroradiol 1986; 7 : 1099-1100. 9. Tomita H, Ito U, Tone O, Masaoka H, Tominaga B. Atrophy and maldevelopment of the ipsilateral cerebral hemisphere after acute subdural hematoma in infants. Surg Neurol 1994; 42 : 498-504. 10. Robinson MJ. Fetal subdural hemorrhages presenting as hydrocephalus. Br Med J 1980; 281: 35. 11. Cheung PY, Obaid L, Rajani H. Spontaneous subdural hemorrhage in newborn babies. The Lancet 2004; 363 : 20012002. 12. Hanigan WC, Ali MB, Cusack TJ, Miller TC, Shah JJ. Diagnosis of subdural hemorrhage in utero. J Neurosurg 1985; 63 : 977979. 13. Parsch CS, Krauss J, Hofmann E, Meixensberger J, Roosen K. Arachnoid cysts associated with subdural hematomas and hygromas: analysis of 16 cases, long-term follow-up, and review of the literature. Neurosurgery 1997; 40 : 483-490. 14. Matsuyama T, Aoyama N, Sakaki T. A rare case of middle fossa neuroepithelial cyst accompanied by chronic subdural hematoma. No Shinkei Geka 1993; 21 : 931-933. 15. Misra M, Salazar JL, Bloom DM. Subdural-peritoneal shunt: treatment for bilateral chronic subdural hematoma. Surg Neurol 1996; 46 : 378-383. 1048 Indian Journal of Pediatrics, Volume 74—November, 2007 Infantile chronic subdural hematomas are often managed successfully with simple burr hole evacuation and external drainage. Other surgical options include craniotomy with excision of membrane and subduralperitoneal shunt in recurrent cases.15 Another indication of craniotomy is the presence of secondary craniosynostosis, as in our case, where necessary suturectomy has to be done to allow for the growth of cranial vault. CONCLUSION