Retrospective Analysis of Neonatal Surgery at Tottori University over the Past Ten Years.

Background: In recent years, the number of neonatal surgeries has been on the rise despite the decline in the number of births, and we examined the actual trends and problems at Tottori University Hospital located in the Sanin region. Methods: Medical records were retrospectively searched for patients who underwent major surgery during the neonatal period (within 30 days of age) at the Tottori University Hospital over the past 10 years (Jan. 2011 to Dec. 2020). Results: Sixty-five cases were included. Early birth infants (< 37 gestational weeks) comprised 15 cases (23%) and low birth weight (< 2500 g) infants involved 27 cases (42%). In the latter half (2016-2020), early birth and low birth weight infants were significantly less than in the first half (2011-2015). The common diseases were anorectal malformation (14 cases), esophageal atresia (10), duodenal atresia (10), and diaphragmatic hernia (9). Prenatal diagnosis was obtained in 26 cases (40%), with high diagnostic rate obtained in duodenal atresia (100%), abdominal wall defect (100%), ileal atresia (75%), meconium peritonitis (67%), and diaphragmatic hernia (67%). Fifty-five cases (85%) were operated on within 7 days of age. Other major malformations were associated in 23 cases (35%). There were 6 deaths (9%), of which 3 cases were low birth weight infants with gastrointestinal perforation, 2 cases with severe chromosomal abnormalities (esophageal atresia, omphalocele), and 1 case with diaphragmatic hernia with severe pulmonary hypertension. Home medical care has been required with gastrostomy tube in 2 cases. Conclusion: Neonatal surgery at Tottori University has been well performed as required with acceptable results along with the progression of other perinatal care. However, further investigation for improvements in premature delivery or organ hypoplasia may be required.

Erratum for Hasegawa et al., "Retrospective Analysis of Neonatal Surgery at Tottori University over the Past Ten Years".

[This corrects the article DOI: 10.33160/yam.2023.11.003.].

Reassessment of phenytoin for treatment of late stage progressive myoclonus epilepsy complicated with status epilepticus.

In order to find an effective treatment option for status epilepticus in progressive myoclonus epilepsy (PME), we reviewed the clinical course of 9 patients with PME. Initially, epilepsy was successfully treated with antiepileptics. However, it gradually became refractory to medication, and status epilepticus emerged 3-19 years after the onset of epilepsy. In these patients, status epilepticus in PME was classified into (1) myoclonic status epilepticus (MSE), (2) myoclonic-generalized status epilepticus (MGSE), and (3) generalized status epilepticus (GSE). MSE was common in patients with neuronal ceroid lipofuscinosis, and GSE was common in those with dentatorubral-pallidoluysian atrophy. MGSE was characterized by the mixture of escalating myoclonus and generalized seizures, and was observed in patients with Gaucher disease or unspecified PME. All patients were often refractory to infusion of benzodiazepines and barbiturates but phenytoin was able to terminate status epilepticus in 7 patients. Oral phenytoin administration as preventive therapy was effective in 6 patients. Aggravation of myoclonus was not provoked by these treatments. We propose that phenytoin should be considered as a treatment choice for PME patients at late stages to prevent the detrimental effects of prolonged or repeated status epilepticus on the brain tissues.