Your browser doesn't support javascript.
loading
Peutz-Jeghers syndrome: management for recurrent intussusceptions.
Verma, Anju; Kanneganti, Pujana; Kumar, Basant; Upadhyaya, Vijai Datta; Mandelia, Ankur; Naik, Prathibha B; Kumar, Tarun; Agarwal, Nishant.
Afiliación
  • Verma A; Department of Paediatric Surgery, SGPGIMS, Lucknow, 226014, India. anju_v2006@rediffmail.com.
  • Kanneganti P; Department of Paediatric Surgery, SGPGIMS, Lucknow, 226014, India.
  • Kumar B; Department of Paediatric Surgery, SGPGIMS, Lucknow, 226014, India.
  • Upadhyaya VD; Department of Paediatric Surgery, SGPGIMS, Lucknow, 226014, India.
  • Mandelia A; Department of Paediatric Surgery, SGPGIMS, Lucknow, 226014, India.
  • Naik PB; Department of Paediatric Surgery, SGPGIMS, Lucknow, 226014, India.
  • Kumar T; Department of Paediatric Surgery, SGPGIMS, Lucknow, 226014, India.
  • Agarwal N; Department of Paediatric Surgery, SGPGIMS, Lucknow, 226014, India.
Pediatr Surg Int ; 40(1): 148, 2024 Jun 02.
Article en En | MEDLINE | ID: mdl-38825635
ABSTRACT

BACKGROUND:

Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by hamartomatous gastrointestinal polyps along with the characteristic mucocutaneous freckling. Multiple surgeries for recurrent intussusception in these children may lead to short bowel syndrome. Here we present our experience of management in such patients.

METHODS:

From January 2015 to December 2023, we reviewed children of PJS, presented with recurrent intussusceptions. Data were collected regarding presentation, management, and follow-up with attention on management dilemma. Diagnosis of PJS was based on criteria laid by World Health Organization (WHO).

RESULTS:

A total of nine patients were presented with age ranging from 4 to 17 years (median 9 years). A total of eighteen laparotomies were performed (7 outside, 11 at our centre). Among 11 laparotomies done at our centre, resection and anastomosis of bowel was done 3 times while 8 times enterotomy and polypectomy was done after reduction of intussusception. Upper and lower gastrointestinal endoscopy (UGIE & LGIE) was done in all cases while intraoperative enteroscopy (IOE) performed when required. Follow-up ranged from 2 months to 7 years.

CONCLUSION:

Children with PJS have a high risk of multiple laparotomies due to polyps' complications. Considering the diffuse involvement of the gut, early decision of surgery and extensive bowel resection should not be done. Conservative treatment must be tried under close observation whenever there is surgical dilemma. The treatment should be directed in the form of limited resection or polypectomy after reduction of intussusception.
Asunto(s)
Palabras clave
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Recurrencia / Síndrome de Peutz-Jeghers / Intususcepción Límite: Adolescent / Child / Child, preschool / Female / Humans / Male Idioma: En Revista: Pediatr Surg Int Asunto de la revista: PEDIATRIA Año: 2024 Tipo del documento: Article País de afiliación: India Pais de publicación: Alemania
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Recurrencia / Síndrome de Peutz-Jeghers / Intususcepción Límite: Adolescent / Child / Child, preschool / Female / Humans / Male Idioma: En Revista: Pediatr Surg Int Asunto de la revista: PEDIATRIA Año: 2024 Tipo del documento: Article País de afiliación: India Pais de publicación: Alemania