Primary 25-gauge sutureless vitrectomy with oblique sclerotomies in pseudophakic retinal detachment.

PURPOSE: To evaluate the anatomic and functional outcomes of 25-gauge sutureless vitrectomy in primary treatment of noncomplex pseudophakic rhegmatogenous retinal detachments (RRD). METHODS: Prospective interventional institutional case series. Twenty-two eyes with pseudophakic RRD with proliferative vitreoretinopathy grade A or B underwent primary 25-gauge vitrectomy with oblique sclerotomies and gas endotamponade. Eyes with minimum follow-up of 6 months were evaluated. Main outcome measures were reattachment rate with single surgery, reoperation, complication rates, and changes in visual acuity (VA). RESULTS: Mean duration of visual loss was 14.68 +/- 12.87 days. Seventeen (77.27%) eyes had macular detachment. In all eyes 25-gauge sutureless vitrectomy was completed without complications. The mean follow-up period was 10.40 +/- 5.77 months. Retinal attachment was achieved in 21 (95.45%) eyes with single surgery and in all (100%) eyes with second vitrectomy. Mean preoperative VA of 1.61 +/- 1.18 improved to 0.50 +/- 0.53 at the last visit (P < 0.001). Transient hypotony was detected in 2 (9.09%) eyes with spontaneous resolution. No other postoperative complication was observed. CONCLUSIONS: Twenty-five-gauge sutureless vitrectomy with oblique sclerotomies in primary treatment of noncomplex pseudophakic RRDs resulted in reattachment in 95.45% with single surgery, and in 100% with reoperation in one eye, accompanied by an increase in visual acuity in 86% of eyes.

Vigabatrin caused rapidly progressive deterioration in two cases with early myoclonic encephalopathy associated with nonketotic hyperglycinemia.

Vigabatrin, a structural analogue of gamma-aminobutyric acid (GABA), is used for the treatment of generalized and partial seizures in infants. The drug inhibits the GABA transaminase and elevates the GABA concentration in the brain. Here we present the vigabatrin experience in two patients with early myoclonic encephalopathy owing to nonketotic hyperglycinemia (glycine encephalopathy). Both patients had early infantile seizures characterized by fragmentary myoclonic jerks associated with burst-suppression pattern on electroencephalography. Nonketotic hyperglycinemia was diagnosed with elevated cerebrospinal fluid and plasma glycine levels. The seizures were initially thought to be infantile spasms, and vigabatrin (50 mg /kg/day) was started for the treatment of seizures. Rapidly progressive deterioration was noticed after a few days. Acute encephalopathy associated with sleepiness and respiratory failure developed. Vigabatrin produced acute encephalopathy, which regressed in a few days after vigabatrin was stopped in the first patient. However, in the second case, despite the discontinuation of vigabatrin, there was no recovery of general conditions. Our observations in two cases indicate the risk of using vigabatrin in patients with nonketotic hyperglycinemia. The elevated GABA concentration in the brain can enhance the encephalopathy, together with the elevated levels of glycine. (J Child Neurol 2006;21:82-84).

Our experience with fulminant hepatic failure in Turkish children: etiology and outcome.

Fulminant hepatic failure is a rare and devastating event during childhood. The etiology of liver failure is reported to change according to age and geographical location. We aimed to investigate, retrospectively, causes and outcome of fulminant hepatic failure in Turkish children. Thirty-four children with fulminant hepatic failure were analysed by means of etiology and outcome. Etiological factor, clinical presentation, encephalopathy stage and biochemical parameters were correlated with outcome. Acute viral hepatitis was detected in 12 cases (35.2 per cent) and hepatitis A was the most commonly detected cause among cases with fulminant hepatic failure (n = 9, 26.4 per cent). Hepatitis B and non A-E infection were diagnosed in two (5.8 per cent) and one (2.9 per cent) cases, respectively. Wilson's disease was defined in four patients (12.5 per cent). Budd-Chiari syndrome (2.9 per cent), autoimmune hepatitis (2.9 per cent) and mushroom poisoning (2.9 per cent) were other detected causes of fulminant hepatic failure in this group. No viral, metabolic, toxic or anatomic reason could be detected in the remaining 15 (44.1 per cent) patients and they were evaluated as cryptogenic. Mortality was 67.6 per cent (23 cases). Encephalopathy grade, total and indirect bilirubin levels were found to be significantly higher in patients who died (p = 0.004, p = 0.03, p = 0.04). Seven patients could have been transplanted (two cadavaric, five living related) and the mortality of this group was 28.5 per cent (n = 2). It was concluded that fulminant hepatitis A virus (HAV) infection is the most common detectable cause of fulminant hepatic failure in Turkish children.

Dissection of aorta: a pediatric case report.

We present a 15-year-old boy who developed sudden walking disability and sensory loss. He could not stand up on his feet and had no feeling following a sudden fall while playing basketball. He had been referred to a local hospital with these symptoms. In his physical examination absence of deep tendon reflexes and sensory loss were noted. His arterial blood pressure was 210/160 mmHg. He was transferred to our hospital with these findings and diagnosis of Guillain-Barré syndrome and hypertensive encephalopathy. There was sudden onset of sensory loss, walking disability and history of trauma. In the following hours hematuria, back pain and lower extremity ischemia developed. We suspected spinal artery injury based on the findings. Dissection of descending aorta was established with the help of magnetic resonance imaging of spinal region and contrasted aortography. The patient went to surgery immediately. He was lost on the second day after operation because of malperfusion. We report this case because dissecting aorta is very rare in the pediatric age group. High index of suspicion and early aortography are needed to diagnose aorta dissection.