Temporal arachnoid cysts: are they congenital?

Childs Nerv Syst https://doi.org/10.1007/s00381-017-3613-9

CASE REPORT

Temporal arachnoid cysts: are they congenital? Hamilton Matushita 1 & Daniel Dante Cardeal 1 & Fernanda Gonçalves de Andrade 1 & Manoel Jacobsen Teixeira 1

Received: 10 July 2017 / Accepted: 4 October 2017 # Springer-Verlag GmbH Germany 2017

Abstract Case report The authors report two cases of arachnoid cysts (ACs) neither detected during pregnancy nor shortly after birth when newborns underwent CT scan evaluation after birth head trauma. ACs were diagnosed at 10 months and 6 years, respectively. The first one becomes symptomatic, and the other one was incidentally found during a head trauma investigation. Discussion These cases give support for the postnatal pathogenesis for some of the assumed congenital ACs. We collected data from the literature that supports the acquired hypothesis for ACs. Keywords Intracranial arachnoid cyst . Pathogenesis . Congenital . Acquired

Introduction Congenital malformations comprise a diverse group of disorders that often present at birth, either as the result of genetic disturbances, infections, errors of morphogenesis, or abnormalities in the intrauterine environment. Congenital disorders * Hamilton Matushita [email protected]; [email protected] Daniel Dante Cardeal [email protected] Fernanda Gonçalves de Andrade [email protected] Manoel Jacobsen Teixeira [email protected] 1

Department of Neurosurgery, Division of Pediatric Neurosurgery, University of São Paulo, Avenida Dr. Eneas Carvalho de Aguiar 255, São Paulo 05403900, Brasil

affecting the brain are now often recognized before delivery with the use of prenatal ultrasonography. There has been much debate about the etiology and pathogenesis of ACs. The cause of most ACs is unknown; the term Bprimary arachnoid cysts^ is suggested for this idiopathic group, and Bsecondary arachnoid cysts^ is suggested for those that can be attributed to injury or inflammation and for those which are associated with tumors [1]. ACs, although considered a congenital anomaly, are rarely reported either in prenatal or in the perinatal study [2–7]). Studies on the prenatal ultrasound have demonstrated a significantly lower prevalence of the ACs than the prevalence of these cysts on the MRI studies performed in asymptomatic populations. These data from the literature indicate that probably, most of the intracranial arachnoid cysts are not congenital. We present two children in which middle fossa ACs were not detected on prenatal ultrasonography nor in the CT images obtained after birth and in late follow-up to explore lesions due to traumatic labor.

Case report Case 1 A male baby was born by vaginal delivery after 40 weeks of an uneventful pregnancy. He had a traumatic birth with a prolonged labor and forceps-assisted delivery. There was bruising and swelling at the right frontal area of the head, including his nose and eyelids. He weighed 3680 g, was 50.0 cm long, had a head circumference of 34 cm, and Apgar scores of 7 and 9 at 1 and 5 min, respectively. In utero ultrasounds at 19 and 32 weeks of gestation had been normal. On his first day of life, the fontanel was full and slightly tense although he was alert, with good muscle tone, positive Moro reflexes, strong sucking, and equal and reactive pupils. A head

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computed tomography (CT) was obtained on his first day of life. CT revealed diastasis of lambdoidal suture, bleeding at the falco-tentorial junction, and a doubtful opening of the Sylvian fissures (Fig. 1a, b). The child was discharged from the nursery on his 18th day of life and was referred to the outpatient follow-up. His early development was appropriate with the achievement of the expected developmental milestones. As there was no neurological dysfunction resulting from the birth trauma, the child was discharged from medical follow-up. When he was 5 years old, he was admitted to the emergency room of our hospital due to a minor head trauma followed by transient disorientation and vomiting. At admission, he was complaining of dizziness, but he was alert and oriented with appropriate responses and no focal neurological signs. He had

Fig. 1 Case 1. Axial non-enhanced CT scans: (a and b) obtained on Day 1 after births revealing diastasis of the lambdoidal sutures, bleeding at the falco-tentorial junction, and doubtful opening of the Sylvian fissures; (c and d) obtained when the patient was 5 years old, incidentally demonstrating an asymptomatic middle fossa arachnoid cyst; and (e and f) obtained when the patient was 9 years old, showing a stable arachnoid cyst

a small bruise on the left frontal-palpebral area. During the examination, it was observed that he had an asymmetric head with bulging of the right temporal bone. A CT scan revealed no consequences of the head trauma but discovered an arachnoid cyst at the right middle fossa (Fig. 1c, d). The child improved in less than 24 h, and as he was asymptomatic, he was delivered to an outpatient clinic for follow-up. He has developed with no specific symptoms and has achieved normal education levels. A follow-up CT at 9 years of age demonstrated a stable arachnoid cyst (Fig. 1e, f).

Case 2 A 3000-g male child, delivered after a 38-week gestation by the extended vaginal way, was the first child of a 28-year-old mother. The pregnancy was uneventful, during which four ultrasound examinations were carried out with no abnormalities. The last ultrasound was performed in the 32nd week of gestation. Apgar scores were 6 at the first minute and then 8 and 9 at the third and fifth minutes, respectively. The child presented a cephalohematoma at the top of his head and was sent to the intensive care unit. A non-invasive respiratory support was needed for only 24 h. On his second day of life, the newborn presented episodes of fixed gazes followed by perioral cyanosis and upward ocular deviation for approximately 2 min. A cranial ultrasound was performed on the same day and revealed a hemorrhagic focus in the posterior fossa. The CT scan showed a subdural hematoma over the occipital lobes and a posterior interhemispheric fissure extending to the posterior fossa on the left side (Fig. 2a). The newborn evolved uneventfully. A serial ultrasound demonstrated the gradual absorption of the subdural hematoma and no hydrocephalus. The CT scan obtained on day eight showed an almost complete absorption of the posterior fossa hematoma (Fig. 2b). The child was discharged from the hospital after 15 days of hospitalization and was referred to an outpatient clinic. The child developed progressive macrocephaly, and he was readmitted to the hospital at the age of 1 year and 3 months. Upon admission, he showed normal developmental milestones and an increased head circumference of 47.5 cm (> 97.5 percentile) with full and soft fontanelle. A head CT was performed and revealed a large arachnoid cyst filling the mesial portion of the right temporal fossa and laterally displacing the temporal lobe (Fig. 2c, d). We performed a cystic fenestration through a small temporal craniotomy. We observed during the procedure that the subarachnoid space next to the opto-chiasmatic cistern was very narrow and had many arachnoid trabeculae (Fig. 3). The post-op follow-up was free of complications, and the child was discharged 5 days later. During the follow-up, after 2 years, a subsequent CT scan showed a considerable reduction of the cyst size (Fig. 2e, f). He has developed very well.

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Fig. 3 Case 2. Intraoperative photographs showing a narrow subarachnoid space with a dense amount of delicate subarachnoid trabeculae dividing the cavity of the opto-chiasmatic cistern. The cistern was opened medial to the ACs during the fenestration of the temporal cyst

Fig. 2 Case 2. Axial non-enhanced CT scans: (a) obtained on the second day after birth, revealing a thick subdural hematoma in the left posterior fossa; (b) obtained on Day 8, showing almost complete absorption of the hematoma and accumulation of the subdural collection of fluid in both sides of the posterior fossa; (c and d) obtained after 1 year and 3 months, showing a large arachnoid cyst on the right middle fossa; and (e and f) obtained 2 years after the surgery, showing marked decrease of the arachnoid cyst

Discussion Congenital ACs may result from the maldevelopment of the arachnoid membrane during the early phase of subarachnoid space formation. Therefore, it would be expected that the cyst formation could be diagnosed during pregnancy or early in the post-natal period. There are few cases diagnosed during infancy, although most ACs are detected during the first two decades of life with peak prevalence at 1 and 5 years of age of 3.8 and 4.6%, respectively [8–11]. One possible reason why congenital ACs are rarely diagnosed during infancy is either because they do not cause symptoms or because the symptoms are misunderstood. When symptomatic, during infancy, macrocephaly related to hydrocephalus is the main clinical

finding [12]. Development of the ACs after birth may raise the question of congenital origin for some of these cysts or if the birth head trauma plays a role in the development of cysts. Our two cases illustrate with neuroimaging the development of middle temporal fossa cysts after birth, and it is noteworthy to emphasize that no lesion was detected prenatally or at birth. The observed prevalence of prenatal ACs, based on routine ultrasonography studies of pregnant women, varies from 0.003 to 0.008%, usually diagnosed in the last trimester of pregnancy [13, 14]. Additionally, as ACs are non-fatal malformations, they are not reported in perinatal autopsies of aborted fetuses [15–17], which makes the prevalence figures from prenatal ultrasounds more accurate. ACs may correspond to 1.7% of all central nervous system anomalies detected in routine workups among pregnant women [13]. Although the estimated accuracy of prenatal detection of central nervous system anomalies ranges between 92% and 99.7% [18, 19], ACs may be confused with other fluid-filled cysts [20, 21], which may inflate the estimations. On the other hand, postnatal populational prevalence rates based on studies of cranial MRIs show a prevalence ranging from 0.23 to 3.5% in healthy populations [12, 22–28]. A systematic review and meta-analysis of 16 studies involving 19,559 people without neurologic symptoms who underwent an MRI of the brain stated ACs have a specific prevalence of 0.5% [24]. In the healthy pediatric population, ACs as incidental findings of MRI studies occur in 0.88% of the cases [23]. An MRI study assessing applicants for military flying duties, that is, a healthy, young population, found a prevalence of ACs of 1.7% [28]. A multi-purpose health study conducted in Norway into 1006 participants between 50 and 66 years of age established a prevalence of intracranial ACs of 3.6%, and specifically of 0.5% at the middle cranial fossa [29].

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Considering all these statistical information, we may conclude that the prevalence of ACs is at least 100 times more common postnatal than antenatal, and their possibility increases with age. Another discrepancy between ACs in prenatal and postnatal populations relates to the topographic distribution of the arachnoid cysts. Most ACs diagnosed prenatally are placed supratentorial in the midline [20, 21, 30, 31], contrary to what is observed in postnatal diagnoses of ACs, where the most prevalent is in the middle cranial fossa [8, 11, 32, 33]. Different studies in the literature also suggest the acquired pathogenesis in most ACs. Okumura et al. [34] presented the case of a 7-year-old boy with a temporal fossa arachnoid cyst developing during infancy. His cyst was not found on a brain CT scan taken for the evaluation of seizures on the second day after his birth. Choi et al. [35] reported that 15.6% of arachnoid cysts are closely related to head injuries either during infancy or the perinatal period. Mainly, in the middle cranial fossa, in 11.6% of the cases, the trauma would be involved in the pathogenesis of the ACs. The mean latent period from head trauma to initial clinical manifestation was 2.2 years, ranging from 10 months to 6.2 years. Martinez-Lage et al. [10] described a case of the development of a middle fossa arachnoid cyst in a child of 22 months old who was followed due to an arrested hydrocephalus diagnosed when she was 7 months old. They attributed it to a cyst too small to be detected in the first CT scan. Peraud et al. [36] reported a large arachnoid cyst in the middle cranial fossa in a three-day-old infant that was not evident on the prenatal ultrasound at 32 weeks of gestation. Rao et al. [37] reported treating one child with a frontal hemispheric cyst that developed after birth and was not present on imaging studies performed during the perinatal period. A Japanese national survey conducted in 1999–2000 to study the etiology and associated conditions of prenatally and postnatally diagnosed congenital hydrocephalus found ACs to be three times more common in the postnatal period than in the fetal period [38]. On the other hand, other cystic anomalies like holoprosencephaly, porencephaly, schizencephaly, hydranencephaly, and agenesis of the corpus callosum were more prevalent with fetal hydrocephalus. The fact that ACs were exceptionally predominant in the fetal hydrocephalus group suggests the high likelihood of the postnatal development of the condition [38]. Congenital ACs are most likely result from the anomalous splitting of primitive mesenchyme, which forms the inner pia mater and the outer arachnoid and the subarachnoid space between them [1]. The pulse of CSF is the force that dissects this loose mesenchymal tissue. Faulty separation of the primitive mesenchyme will originate in an arachnoid pouch, which may develop progressively through life [10]. The interhemispheric and Sylvian fissures, the deepest of the brain, are more prone to the anomalous splitting of the primitive mesenchyme. These areas are where the mechanisms of the folding of the brain mantle are more intense

and profound. The congenital malformation of the arachnoid membrane can be the original event that can change during postnatal life, depending on impaired CSF hydrodynamics or the secondary injuries [39]. Birth trauma and traumatic postnatal events may contribute to the development of this prenatal propensity or initiate, by itself, a little disruption of the arachnoid membranes leading to the formation of ACs. Another causative factor independent of the congenital malformation is the occurrence of leptomeningeal adhesions following hemorrhage or infections. Probably, the acquired postnatal factors are more pronounced than the prenatal congenital predispositions.

Conclusions Based on documentation of these two cases, we suggest that some of the middle fossa arachnoid cysts may have an acquired pathogenesis and were not congenital, as would be assumed. Some ACs are not due to faulty embryological development but may represent the consequence of acquired perinatal insults. Acquired ACs may develop secondary to birth trauma. The arachnoid may have been disturbed during the traumatic labor or acquired in later life. Compliance with ethical standards Conflict of interest The authors declare no conflict of interest.

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