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Hydranencephaly is a rare and fatal central nervous system (CNS) disorder, occurring in less than 1 per 10,000 births [1]. Cerebral hemispheres are almost absent and replaced with cerebrospinal fluid, but there is typically intact falx cerebri, thalamus, and cerebellum. Facial features and midline structures generally appear normal, distinguishing it from other CNS anomalies [1]. Most patients are stillborn or die within a few hours to months after birth, though long-term survival has been reported in rare cases [2]. Etiologies may be related to infections, toxins, genetics, or occlusive vascular lesion, but there are still many doubts about exact etiopathogenic mechanisms [1]. A previous case report briefly described the survival of a patient with hydranencephaly born as one of monochorionic-diamniotic (MCDA) twins [3]. However, there have been few reports on the neurological manifestations after survival especially as an extremely low birth weight (ELBW) infant of MCDA twins. Here, we report the clinical manifestations in a survived ELBW case of hydranencephaly, in MCDA twins. The male patient was born at 33 weeks and 2 days of gestation. The birth weight was 840 g, height 33 cm, and head circumference (HC) 20.8 cm. His identical twin was born without any congenital anomalies and his birth weight was 1.7 kg (20th percentile). Prenatal ultrasound revealed the presence of fetal hydrops and brain ischemic insult. There was no maternal history of infections or exposure to toxins during pregnancy. Termination of pregnancy was avoided due to the possibility of intrauterine fetal death of the co-twin. The patient’s Apgar score was 7 at 1 minute and 9 at 5 minutes. Physical examination at birth showed microcephaly, corneal opacities, anonychia of feet, but normal facial appearance (Fig. 1). He had stable spontaneous breathing and actively responded to external stimuli. Diagnostic tests were delayed due to his parents’ initial refusal. As he was still alive with minimal care, further tests were performed. Brain ultrasonography performed at 18 days of age showed that supratentorial brain structures were almost absent, with the presence of only a small remnant of frontal lobe-like areas. At 28 days of age, 37 weeks of postmenstrual age (PMA), he started showing some sucking movements, and echocardiography was normal. Glaucoma on both eyes was detected by eye consultation at 38 days of age. At 41 weeks of PMA, a full dose of bottle feeding was possible, and automated auditory brainstem response test was normal. At 46 weeks of PMA, brain magnetic resonance imaging revealed the presence of the bilateral frontal lobes, small in size and displaced. A large cystic

Clinical Manifestations of Hydranencephaly: A Case in Monochorionic-Diamniotic Twin