Management of Esophageal Strictures in Children With Recessive Dystrophic Epidermolysis Bullosa

Castillo, Ricardo; Davies, Yinka; Lin, Yuan‐Chi; Garcia, M. C. Gimenez; Young, Harvey

doi:10.1097/00005176-200205000-00012

Cited by 50 publications

(65 citation statements)

References 9 publications

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“…This frequency increases with age, with a cumulative risk of almost 95% by 45 years of age (Fig 6). 44,45,51,52 Progressive scarring and narrowing of the esophagus limit the food textures that can be ingested, presenting initially with dysphagia to hard or bulky foods, then to softer foods, and, eventually, to liquids. If left untreated, patients ultimately may be unable to swallow even their own saliva.…”

Section: Pharynx and Esophagusmentioning

confidence: 99%

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Fine

Mellerio

2009

Journal of the American Academy of Dermatology

244

168

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Section: Pharynx and Esophagusmentioning

confidence: 99%

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Fine

Mellerio

2009

Journal of the American Academy of Dermatology

244

168

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“…In the esophagus and oropharynx, repeated blistering often leads to scar formation and ultimately, esophageal strictures [5][6][7][8]. Patients generally develop progressive microstomia, and by age 12, more than 50% have significant ankyloglossia [9].…”

Section: Introductionmentioning

confidence: 99%

Non-endoscopic percutaneous gastrostomy placement in children with recessive dystrophic epidermolysis bullosa

et al. 2007

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Recessive dystrophic epidermolysis bullosa (RDEB) is associated with high nutritional demands, esophageal strictures and dysphagia. About one quarter of the patients require gastrostomy tube placement to maintain adequate nutritional status. To minimize trauma to the skin and pharyngoesophageal mucosa caused by commonly used gastrostomy tube insertion techniques, we used a non-endoscopic, percutaneous, image-guided approach. This approach combines the use of ultrasound for mapping of the liver and spleen, water-soluble contrast enema to visualize the colon, and gastric insufflation to define the stomach. The gastrostomy tube is replaced by a low-profile button gastrostomy tube 10-12 weeks postoperatively. The five female patients reported in this series ranged in age from 6 to 9 years. They all tolerated the procedure well and no perioperative complications were encountered. All were able to tolerate feedings on postoperative day 1 and all underwent successful replacement of gastrostomy tubes by low-profile button tubes. Our experience suggests that a non-endoscopic, image-guided approach to gastrostomy tube placement offers a safe, effective, and minimally traumatic alternative to more commonly used approaches. It minimizes the risk of procedure-related morbidity and leads to overall improvement in the quality of life. As such, we strongly recommend that it be included in the treatment armamentarium for patients with epidermolysis bullosa and nutritional failure.

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“…Esophageal blisters usually evolve to scar tissue and stenosis and may secondarily lead to dysphagia, one of the biggest problems in patients with recessive EB dystrophica7. Complications such as esophageal perforation, pneumomediastinum, pneumoperitonium, and mediastinitis must be diagnosed as early as possible, as they may be fatal conditions 12 . Teeth are often compromised in EB patients 7,14,16 .…”

Section: Discussionmentioning

confidence: 99%

“…Such manifestations are related to the appearance of mucosal blisters (mainly in the oropharynx and esophagus), followed by rupture and hypertrophic scarring, leading to frenulum shortening, microstomia, esophageal stenosis, laryngeal stenosis, and nasal vestibule stenosis. Esophageal stenosis is a complication usually found in advanced cases, and remains as one of the main challenges in treating this condition 12 . Descriptive studies analyzing this infirmity have not yet been published in Brazil.…”

Section: Introductionmentioning

confidence: 99%

Manifestações otorrinolaringológicas e esofágicas da epidermólise bolhosa

Fantauzzi¹,

Maia²,

Cunha³

et al. 2008

Rev. Bras. Otorrinolaringol.

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Epidermólise bolhosa (EB) é um conjunto de afecções bolhosas, de caráter hereditário, com diferentes quadros clínicos e diferentes modos de transmissão genética. Os indivíduos evoluem com bolhas na pele e mucosas, que surgem espontaneamente ou após mínimos traumatismos. OBJETIVO: Descrever as manifestações otorrinolaringológicas, as complicações esofágicas relacionadas à EB e a experiência na conduta de pacientes com estenose esofágica decorrente da EB. CASUÍSTICA E MÉTODO: Estudo descritivo de 60 pacientes com EB, atendidos de 1999 a 2006, no serviço de Otorrinolaringologia e Cirurgia de Cabeça e Pescoço do Hospital X, centro de referência para EB. RESULTADOS: Dos 60 pacientes com idade média de 14,5 anos, 28 (46,6%) eram mulheres e 32 (53,4%) homens. Oito (13,4%) tinham o diagnóstico de EB simples, 51 (85%) EB distrófica e um (1,6%) caso de EB adquirida. Lábios, boca, língua e pavilhão auricular foram os locais mais acometidos (32 pacientes - 53,3%). Disfagia foi encontrada em 28 pacientes (46,6%). Após dilatação do esôfago todos apresentaram remissão do sintoma. CONCLUSÃO: EB é uma doença rara e os pacientes devem ser encaminhados para tratamento em centros de referência. Portanto, é fundamental que os médicos envolvidos com os cuidados de pacientes com EB conheçam as condutas necessárias para melhorar a qualidade do tratamento sem prejuízos adicionais.

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Management of Esophageal Strictures in Children With Recessive Dystrophic Epidermolysis Bullosa

Cited by 50 publications

References 9 publications

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Extracutaneous manifestations and complications of inherited epidermolysis bullosa

Non-endoscopic percutaneous gastrostomy placement in children with recessive dystrophic epidermolysis bullosa

Manifestações otorrinolaringológicas e esofágicas da epidermólise bolhosa

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