The Therapeutic Effect of Sclerocorneal Lens in Coexisting Corneal Ectasia and Stevens-Johnson Syndrome

PURPOSE: We report short-term treatment effects of a mini-scleral lens in patients with Stevens-Johnson syndrome and corneal ectasia. CASE SUMMARY: A 54-year-old female who had been diagnosed with Stevens-Johnson syndrome and keratoconus presented with persistent right eye pain and decreased visual acuity. Therapeutic lenses, topical antibiotic eye drops, and topical steroid eye drops were used; however the symptoms were not controlled, then the patient was treated with a mini-scleral contact lens in the right eye. At the time of the first visit to our hospital 17 years ago, and the best-corrected visual acuity (BCVA) of the right eye was logMAR 0.22. However, Stevens-Johnson syndrome and corneal ectasia were severe and cataract surgery was considered because of worsening cataracts, but the patients refused this surgery. At the time, the BCVA of the right eye was then reduced to logMAR 1.10. On ophthalmic examination, there was conjunctival fibrosis and corneal neovascularization of the right eye. The symptoms were not managed with a using a therapeutic lens, and then the patient was prescribed a mini-scleral contact lens. After wearing the mini-scleral contact lens, the corneal and conjunctival neovascularization was reduced. After 6 months, the BCVA of the right eye improved to logMAR 0.60. CONCLUSIONS: In patients with Stevens-Johnson syndrome and corneal ecstasia, mini-scleral contact lens can be considered as a useful treatment option for visual improvement and symptom control.

Aerofagia patológica: uma causa rara de distensão abdominal crônica / Pathologic aerophagia: a rare cause of chronic abdominal distension

Objetivo:Descrever o caso de uma adolescente com aerofagia patológica, uma doença rara causada pela deglutição excessiva e inapropriada de ar, e revisar o tratamento e os diagnósticos diferenciais.Descrição do caso:Menina de 11 anos portadora de retardo mental e cegueira, apresentava problemas comportamentais associados a retardo do desenvolvimento, foi consultada por distensão abdominal persistente por oito meses. Sua história pregressa incluía uma fundoplicatura à Nissen. Tomografia e radiografias abdominais mostravam distensão difusa do trato digestivo por ar, incluindo cólon e delgado. Doença de Hirschsprung foi excluída. A distensão persistiu mesmo após o controle da constipação e era mínima de manhã e máxima à noite. Ruídos audíveis e repetitivos de deglutição de ar foram observados e auscultados. A criança foi tratada farmacologicamente com o diagnóstico de aerofagia patológica associado a distúrbio obsessivo compulsivo, sem sucesso. A paciente foi submetida a gastrostomia descompressiva endoscopia e manteve nutrição oral.Comentários:A aerofagia patológica é uma doença rara e autolimitada em crianças, mas pode ser um problema grave e persistente naquelas com problemas neuropsiquiátricos, nas quais pode causar complicações sérias. Os tratamentos comportamentais e farmacológicos têm pouco sucesso nesse grupo. Casos graves podem precisar de tratamento cirúrgico, principalmente gastrostomia descompressiva.

Objective:To describe an adolescent with pathologic aerophagia, a rare condition caused by excessive and inappropriate swallowing of air and to review its treatment and differential diagnoses.Case description:An 11-year-old mentally impaired blind girl presenting serious behavior problems and severe developmental delay with abdominal distension from the last 8 months. Her past history included a Nissen fundoplication. Abdominal CT and abdominal radiographs showed diffuse gas distension of the small bowel and colon. Hirschsprung's disease was excluded. The distention was minimal at the moment the child awoke and maximal at evening, and persisted after control of constipation. Audible repetitive and frequent movements of air swallowing were observed. The diagnosis of pathologic aerophagia associated to obsessive-compulsive disorder and developmental delay was made, but pharmacological treatment was unsuccessful. The patient was submitted to an endoscopic gastrostomy, permanently opened and elevated relative to the stomach. The distention was resolved, while maintaining oral nutrition.Comments:Pathologic aerophagia is a rare self-limiting condition in normal children exposed to high levels of stress and may be a persisting problem in children with psychiatric or neurologic disease. In this last group, the disease may cause serious complications. Pharmacological and behavioral treatments are ill-defined. Severe cases may demand surgical strategies, mainly decompressive gastrostomy.

[Pathologic aerophagia: a rare cause of chronic abdominal distension]. / Aerofagia patológica: uma causa rara de distensão abdominal crônica.

OBJECTIVE: To describe an adolescent with pathologic aerophagia, a rare condition caused by excessive and inappropriate swallowing of air and to review its treatment and differential diagnoses. CASE DESCRIPTION: An 11 year-old mentally impaired blind girl presenting serious behavior problems and severe developmental delay with abdominal distension from the last 8 months. Her past history included a Nissen fundoplication. Abdominal CT and abdominal radiographs showed diffuse gas distension of the small bowel and colon. Hirschsprung's disease was excluded. The distention was minimal at the moment the child awoke and maximal at evening, and persisted after control of constipation. Audible repetitive and frequent movements of air swallowing were observed. The diagnosis of pathologic aerophagia associated to obsessive compulsive disorder and developmental delay was made, but pharmacological treatment was unsuccessful. The patient was submitted to an endoscopic gastrostomy, permanently opened and elevated relative to the stomach. The distention was resolved, while maintaining oral nutrition COMMENTS: Pathologic aerophagia is a rare self-limiting condition in normal children exposed to high levels of stress and may be a persisting problem in children with psychiatric or neurologic disease. In this last group, the disease may cause serious complications. Pharmacological and behavioral treatments are ill-defined. Severe cases may demand surgical strategies, mainly decompressive gastrostomy.