A
bstract
Amniotic band syndrome (ABS) is a rare congenital disease with variable manifestations ranging from simple constriction rings at the extremities to major defects such as exencephaly. Here we report the case of a female baby born full term (39 weeks) from a 35-year-old primiparous mother by cesarean section. In addition to the constriction rings at the extremities (fingers), the newborn presented facial malformations and a cranial anomaly suggestive of exencephaly. Supportive treatment was chosen because of the poor prognosis, and the child died 5 months later. Depending on the anomaly associated with ABS and its complexity, as in our case, genetic studies should be performed whenever possible, and the parents should be informed about the possibility of recurrences and incompatibility with life.
A
BSTRACT
Amniotic band syndrome is a rare condition that is associated with various malformations. Its etiology is controversial. The neuroimage shown here is of a newborn with placenta–encephalocele adhesion and other malformations that suggest amniotic band syndrome.
Background:
Amniotic band syndrome (ABS) is a rare condition of controversial etiology that is associated with varying degrees of anomalies. This study reports a case of a newborn with ABS associated with double encephalocele in the frontal region.
Case Description:
A 29-year-old primiparous woman with no history of prenatal infection or consanguineous marriage had a cesarean section at gestational week 38, giving birth to a newborn who was well but had limb anomalies (constriction rings, amputations, and syndactyly) and craniofacial anomalies, mainly double frontal encephalocele. The patient underwent surgical repair and subsequent placement of a ventriculoperitoneal shunt.
Conclusion:
Studies clarifying this uncommon association with double encephalocele are limited. ABS associated with double encephalocele is rare and even more complex when associated with other anomalies. Thus, the conditions in such children are severe and require multidisciplinary monitoring.
A 4-year-old female child presented to an emergency hospital with headache and episodes of seizures. Computed tomography and magnetic resonance imaging of the skull (Figs. 1a, b and c) were performed; they showed large suprasellar cystic lesions with right temporal extension and mild hydrocephalus. Subsequently, an endoscopic ventriculocystocysternotomy was performed (Figs. 1d, e, and f), which showed good results.
Suprasellar arachnoid cysts account for 9%–21% of the pediatric arachnoid cysts [1,2]. According to the morphology and characteristics, there are three types of suprasellar arachnoid cysts, as follows: 1. Diencephalic leaflet dilatation of the Liliequist membrane with the formation of purely suprasellar cysts, which presents with hydrocephalus; 2. The defect of the mesencephalic leaflet of the Liliequist membrane with dilatation of the interpeduncular cistern, which presents without hydrocephalus; 3. Asymmetrical form that extends to other subarachnoid spaces, which presents with macrocrania and mild or no hydrocephalus [1], similar to the present case. The expansion could be due to an osmotic gradient, a slit valve mechanism, tissue debris transudation from the choroid plexus, or ectopic glial cells [3]. The first treatment option for suprasellar arachnoid cysts should be endoscopic ventriculocystocysternotomy; ventriculoperitoneal shunt may be considered the second treatment option if endoscopic ventriculocystocysternotomy fails [2].
Figure Caption
Magnetic resonance imaging of the brain: (a) T1 axial and (b) T2 axial showing a large suprasellar (black arrows) cyst with expansion to the right temporal pole, having a mass effect and mild ventricular dilatation(white arrows) without ependymal transudation; (c) Sagittal FIESTA-T2 with compressive effect on the brain stem(white arrow) and basilar artery(black arrow) displacement; Endoscopic view: (d) ventriculocystotomy; (e) the third ventricular floor with pituitary stalk(black arrow) and gland(white arrow), dorsum of the sella turcica(black curved arrow), posterior communicating artery(black arrowhead), and oculomotor nerve(white arrowhead); (f) cyststocisternotomy was conducted with visualization of the basilar artery((black arrow) and dorsum of the saddle.
Hydrocephalus caused by optic pathway glioma (OPG) is most often treated with a ventriculoperitoneal shunt (VPS), but this treatment may present complications such as ascites. Hence, ventriculocisternal shunt is an option worth considering. This article reports a case in which it was decided to place a ventriculocisternal or Torkildsen shunt in a patient with hydrocephalus caused by OPG. Case Report: A 12-year-old girl, amaurotic, with a VPS due to hydrocephalus caused by an OPG, reported to the emergency room with abdominal distension and pain and difficulty in walking. Computed tomography of the abdomen was performed, and a large amount of fluid was evidenced in the peritoneal cavity (ascites). Liver and kidney functions were normal. In the surgical procedure, the distal extremity of the ventriculoperitoneal catheter was exposed, and 3 L of ascites fluid of cerebrospinal origin was drained. After 10 days of antibiotic therapy, a ventriculocisternal or Torkildsen shunt was placed. After 4 years of follow-up, the patient has no complaints. Discussion: Arne Torkildsen was the first to perform a ventriculocisternal shunt in 1937; it is indicated in cases of hydrocephalus with obstruction of the aqueduct or third ventricle. In OPG, increased protein levels in CSF and the use of platinum-based chemotherapy agents would explain the development of ascites after VPS placement. In these cases of hydrocephalus with third ventricle tumor infiltrating the hypothalamus, the ventriculocisternal shunt can be used as an option in specific cases.
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