Winson s.Ho, md,and john a.Jane jr., md.Severe cranial deformity following cerebrospinal fluid diversion in an adolescent with osteogenesis imperfecta.Journal of neurosurgery pediatrics 22 (2018).Doi: 10.3171/2018.4.Peds18109.Osteogenesis imperfecta (oi) is an inherited connective tissue disorder that causes bone fragility and deformity.Neurological manifestations, including macrocephaly and hydrocephalus, have been reported.Increased vascular fragility or bleeding diathesis also predisposes oi patients to intracranial hemorrhage.The development of chronic subdural fluid collections or hydrocephalus may require csf diversion.The authors report a previously unrecognized complication of csf diversion in a patient with oi, that is, a delayed severe cranial deformity, presumably due to over-shunting.In addition to the cosmetic concern, the deformity caused severe headaches and tenderness.The patient underwent craniectomy and titanium mesh cranioplasty, which resulted in the complete resolution of symptoms.This report raises the possibility that over-shunting in patients with oi could predispose to the formation of cranial deformity requiring surgical intervention.Reported event: this (b)(6) female with a history of oi had been adopted from (b)(6) as a child.She first presented to the university of (b)(6) emergency room when she was (b)(6) with complaints of headache following a minor head trauma several weeks earlier.Head ct demonstrated a chronic left-sided subdural hematoma and an incidental right middle cranial fossa cyst, which was managed conservatively and resolved on follow-up.At that time, it was noted that she had mild ventriculomegaly, which was thought to represent cerebral atrophy due to a history of traumatic brain injury or malnutrition in infancy.Clinically, she fared well for the next few years.She presented to us again at the age of (b)(6) with severe headache after having suffered a ground-level fall 5 days earlier.Mri demonstrated bilateral subdural hygromas presumably related to the rupture of the middle cranial fossa cyst.As a temporizing measure, bilateral burr holes were made for drainage of hygroma fluid, which was under high pressure.An external subdural drain was placed, but she could not be weaned from it over the next few days.She then underwent placement of a right subdural peritoneal shunt (sps) with a programmable strata valve (medtronic) set at 0.5.She initially did well; however, 3 weeks later she presented with a (b)(6) shunt infection requiring explantation of the shunt and placement of an external subdural drain.After clearance of the csf, she underwent reimplantation of the sps with the strata valve set at 0.5.Unfortunately, she continued to be symptomatic with headache and emesis, requiring revision and replacement of the valve with a fixed ultra-low pressure valve.For 7 days, she was observed with an icp monitor and was subsequently discharged with normal icp measurements.About 2 weeks later, she presented again with headache and emesis.Mri demonstrated a resolved subdural collection but increased ventriculomegaly.Given her clinical symptoms and worsened radiographic findings, we believed that she had developed ¿internal hydrocephalus¿ and recommended placement of a left frontal ventriculoperitoneal shunt (vps) with a strata valve set at 2.0.She did well after shunt placement and was followed up in clinic.At the 4-month follow-up, mri demonstrated decreased ventricle size and resolution of the subdural hygroma.At the 2-year follow-up after vps placement, she was noted to have progressive ¿band-like¿ collapse in the occipital area.She was asymptomatic at that time; therefore, we recommended continued observation.However, over the following 6 months, she became increasingly symptomatic.She had progressive daily headaches that affected her sleep and increasing tenderness to touch in the affected areas.A ct venogram was obtained to confirm patency of the sagittal sinus.Three-dimensional reconstruction of head ct demonstrated collapse of her skull in the region of her lambdoid suture where she had significant wormian bone formation.Given that her symptoms were significantly affecting her quality of life, we recommended a cranioplasty using a custom titanium mesh.Surgery was performed with a bilateral parietal-occipital craniectomy encompassing the deformed area.Pairs of burr holes next to the sagittal sinus, superior and inferior to the deformity, were made.We then performed bilateral parietal-occipital craniectomies of the lateral margins of the wormian bone.We left the wormian bone over the sagittal sinus because of its adherence to the superior sagittal sinus in order to avoid any vascular injury.The custom titanium mesh was then placed to cover the defect.Postoperative head ct demonstrated an excellent cosmetic outcome.The patient had an uneventful postoperative course and was discharged on postoperative day 5.By the 3-month follow-up in clinic, her headaches had completely resolved and she was otherwise doing well clinically.At the 18-month follow-up, she continued to do well.The deformity was well corrected.Mri of the brain demonstrated stable ventricular size and continued resolved subdural collection.
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