|
Can sarica, cem yucetas, ali ozen, necati ucler, capan konca, selahattin akar.Management strategies for hydrocephalus in alobar hol oprosencephaly: a case report and discussion.Pediatric neurosurgery 2018.Doi: 10.1159/000489856 abstract holoprosencephaly is a rare congenital malformation resulting from an impaired midline division of the prosencephalon into distinct cerebral hemispheres.Hydrocephalus is a frequent problem among the few survivors with alobar holoprosencephaly (ahpe), its most severe form.The literature about neurosurgical management of hydrocephalus in this condition is limited and dispersed, and there are still some points that need to be resolved.We report the case of a newborn with ahpe, hydrocephalus, and central diabetes insipidus.We delineate the complexity of the management of these patients and emphasize the benefits of using an initial programmable shunt valve.Further discussion about management strategies includes reviewing previous reports and the benefits of shunting for hypothalamic osmoreceptor function.Reported event.A preterm neonate was born at (b)(6) weeks of gestation to a (b)(6)-year-old mother (g2p1a0l2) by cesarean section.The apgar scores at 1, 2, and 5 min were 6, 8, and 9, respectively.The neonate was admitted to the neonatal intensive care unit because of an antenatal diagnosis of hydrocephalus at (b)(6) weeks of gestation.The neonate weighed (b)(6) and had a head circumference of 35 cm (97th percentile) and a length of 50 cm.Vital signs were within the normal limits.On physical examination, the anterior fontanel was full but not tense and otherwise normal.Neurologically, the neonate had a good tonus with spontaneous movements.Her hematochemical routine tests and torch screen were normal.The mother had a healthy 4-year-old child, and her maternal history was unremarkable except for an unspecific urinary tract infection during pregnancy, which was self-limited.There was no history of trauma, drug abuse, diabetes mellitus, or any other chronic disease.She was anemic during her pregnancy but did not receive any treatment.She did not use any folic acid or vitamin supplement during her pregnancy.The parents were nonconsanguineous and had no significant family history.The mother¿s obstetric history was also not significant.At birth, head magnetic resonance imaging (mri) revealed a single midline monoventricle encircled by a cup-shaped cerebral cortex and a large fluid cavity that occupied the area above the dorsocaudal aspect of the diencephalon.Midline structures (corpus callosum, septum pellucidum, interhemisphericfissure, and falx cerebri) were absent.The basal ganglia and hypothalamic and thalamic nuclei were fused in the midline.The neonate was diagnosed with ahpe.A karyotype analysis showed no chromosomal abnormality (46xx).Further genetic testing was not performed because of financial reasons.Otolaryngology, ophthalmology, and neurosurgery consultations were requested.Craniofacial and ophthalmic anomalies were not detected.The neonate was asymptomatic and discharged from the hospital with recommendation for follow-up in 1 month.The parents returned with the infant at 3 months of age.Her head circumference had increased to 45 cm (> 95th percentile)and her weight had increased from (b)(6), increasing from the 90th percentile to the 30th percentile.The infant was breastfed during this period, but the mother reported poor feeding and started supplementary formula (aptamil bmf; milupa, friedrichsdorf, germany).The follow-up physical examination was normal except for a tense fontanel, and the tonus was normal with a lack of neck control.There were no signs of dehydration.Blood test results were normal except for hypernatremia (sodium: 151 mmol/l) and hyperchloremia.The infant was admitted to the neurosurgery ward, and a pediatric consultation was requested.The primary diagnosis was central diabetes insipidus.Hypotonic fluid (5% dextrose in half-normal saline) was administered for 24 h.The next morning, her sodium level was 149 mmol/l, and anterior pituitary axis-controlled hormone levels were normal.We implanted a ventriculoperitoneal shunt with a strata ii small valve (medtronic inc., minneapolis, mn, usa).The operation lasted 72 min from induction of anesthesia to waking up.No complications occurred in the perioperative period (fig.1d).The shunt valve was set at 2.0.The preoperative and postoperative examinations were similar.The postoperative sodium level was slightly higher than the preoperative level (154 mmol/l).Despite continuous hypotonic fluid administration, the sodium level increased to 160 mmol/l (table 1) and she was transferred to the pediatric intensive care unit.Nasal desmopressin acetate (20 ¿g/day) was started in addition to hypotonic fluid treatment.On day 2 of treatment, her sodium level was still high (156 mmol/l), and the desmopressin dose had doubled.The next day, a neurosurgery consultation was requested regarding fluid accumulation in the shunt pouch and suspicion of early shunt failure.Computed tomography (ct) confirmed fluid accumulation around the shunt valve and above her left eye.We tapped the shunt and found no signs of proximal catheter obstruction.Cerebrospinal fluid (csf) analysis and abdominal ultrasonography did not yield any significant data.We set the valve pressure at 0.5.In the following hours, the infant was anuric with significant generalized edema.Desmopressin was immediately discontinued because of an overdose.After continuous urosemide administration for 1 day and adjustment of her intravenous fluid support, the edema resolved on day 3 of the overdose, and the infant was transferred back to the ward without symptoms and with a sodium level of 151 mmol/l.We added tap water to her diet rather than administering desmopressin again, and the valve pressure was increased to 1.5.During follow-up, her hypernatremia gradually resolved to some extent and measured 146 mmol/l on day 7 of hospitalization.She was discharged without symptoms with that sodium level.Eighteen days after her discharge, she was admitted to the emergency department with fluid accumulation around the shunt valve.She was asymptomatic, and her valve was set to 1.0.Two days after this adjustment, the fluid around the valve had resorbed.The infant was 5 months of age at the time of writing.She was asymptomatic with a normal tonus and could control her neck.She had normal emotional functions (e.G., laughing, crying, watching).At the time of reporting, she was being followed up by a pediatric endocrinologist and had improved feeding with breast milk, formula, and tap water.She was not being administered desmopressin.
|
