Cataratta Congenita

Date
Mar, 01, 2021
cataratta

 

La cataratta congenitarappresenta la più comune causa di perdita della funzione visiva curabile in età pediatrica. La sua incidenza, diversa a seconda delle statistiche, e pari a 8-9/10000 neonati, aumentando a 16/10000 bambini di 7 anni. Si stima che circa 200.000 bambini nel mondo siano ciechi a causa della cataratta. Può interessare uno o entrambi gli occhi (in circa i 2/3 dei casi).

CAUSE

fattori responsabili dell’opacizzazione del cristallino possono essere molteplici. I più comuni comprendono le infezioni intrauterine, i disordini metabolici e le sindromi geneticamente trasmesse. Un terzo delle cataratte pediatriche sono sporadiche e non sono associate con eventuali malattie sistemiche o oculari. La modalità di trasmissione più frequente è l’autosomica dominante a penetranza completa.

  • Idiopatiche (non derivante da cause note)
  • Associata ad anomalie cromosomiche del neonato come la sindrome di down, sindrome di turner,…
  • Infezioni intrauterine (soprattutto se contratte nel primo trimestre): morbillo, rosolia, varicella, citomegalovirus, herpes simplex, herpes zoster, poliomielite, influenza, virus di Epstein-Barr, sifilide e toxoplasmosi.
  • Disordini metabolici (soprattutto se contratti in gravidanza): galattosemia, diabete, ipocalcemia, ipoglicemia, mannosidasi, deficienza galatochinasi, ipoparatiroidismo o gravi carenze alimentari
  • Assunzione di farmaci, soprattutto in gravidanza, come tetracicline, sulfamidici, cortisone, viatamina D
  • Esposizione ai raggi X durante la gravidanza (maggior rischio nel primo trimestre).
  • Associato a malattie oculari: aniridia, persistenza del vitreo primitivo iperplastico, microftalmo, retinopatia del prematuro (ROP), anomalia di Peter, cornea guttata, endoftlamiti
  • La prematurità e la sofferenza feto-neonatale;
  • Malattie sistemiche del neonato (artrite reumatoide, sindrome di Marfan, sindrome di Weill-Marchesani e malformazioni cranio-facciali)
  • Traumi

TIPI di OPACITA’

  1. singole
  2. multiple
  3. piccole (che interferiscono poco con la funzione visiva)
  4. estese e molto dense

SEGNI e SINTOMI

  1. Ambliopia più o meno grave, tipica delle cataratte estese e dense del cristallino
  2. riflesso pupillare biancastro (leucocoria) o l’assenza del classico riflesso rosso in fotografia, spesso segnalato dagli stessi genitori
  3. strabismo, se la cataratta è monolaterale
  4. nistagmo che insorge intorno ai 3 mesi di età, in presenza di cataratta bilaterale evoluta

TRATTAMENTO

Il trattamento consiste nell’asportazione del cristallino

Sebbene l’argomento sia ancora controverso, è ampiamente condivisa l’opinione che in caso di cataratta completa e densa l’intervento è inevitabile. Quando la cataratta è incompleta l’intervento è indicato quando la funzione visiva è compromessa (AV < 4/10).

Se l’acuità visiva non è ottenibile con certezza bisogna tener presente che quanto più posteriore e centrale è la cataratta tanto più determina ambliopia e che una cataratta che oscura il riflesso rosso per più di 3 mm al centro della pupilla può incidere in modo significativo sulla funzione visiva.

Generalmente si preferisce inserire la lente artificiale se il bambino ha più di 18 mesi anche se in alcuni casi si può inserire nella stessa seduta chirurgica.

TIMING

La cataratta interferisce con il normale sviluppo della funzione visiva.

In generale va operata tra la quarta e la sesta settimana di vita (max la decima settimana), cercando di ridurre al minimo l’intervallo tra i due occhi (1-2 settimane) in caso di cataratta bilaterale.

RIABILITAZIONE e Follow-up

Fino a quando la IOL non viene inserita è opportuno proseguire un trattamento riabilitativo sia con l’uso di occhiali sia con lenti a contatto, in quanto l’occhio senza cristallino vede ovviamente male. E’ possibile che si renda necessario un trattamento occlusivo per l’ambliopia e per un  eventuale strabismo.

Visite periodiche sono poi necessarie per monitorare lo stato della retina e del disco ottico.

Referenze

  1. Chan WHBiswas SAshworth JLLloyd IC; Congenital and infantile cataract: aetiology and management; Eur J Pediatr. 2012 Apr;171(4):625-30.
  2. Nucci P., Serafino M., Oftalmologia pediatrica e strabismo. Fabiano gruppo editore. 2012; cap 13: 317-332
  3. Chan WH, Biswas S, Ashworth JL, Lloyd IC. Congenital and infantile cataract: aetiology and management. Eur J Pediatr. 2012 Apr;171(4):625-30.
  4. Lindsay RG, Chi JT. Contact lens management of infantile aphakia. Clin Exp Optom. 2010 Jan;93(1):3-14
  5. Hussin HMMarkham R; Long-term visual function outcomes of congenital cataract surgery with intraocular lens implantation in children under 5 years of age; Eur J Ophthalmol. 2009 Sep-Oct;19(5):754-61.
  6. Krishnamurthy R, Vanderveen DK. Infantile cataracts. Int Ophthalmol Clin. 2008 Spring;48(2):175-92
  7. Lloyd IC, Ashworth J, Biswas S, Abadi RV. Advances in the management of congenital and infantile cataract. Eye (Lond). 2007 Oct;21(10):1301-9
  8. Zetterström CLundvall AKugelberg M.J Cataract Refract Surg. 2005 Apr;31(4):824-40. Cataracts in children.
  9. Lambert SR. Management of monocular congenital cataracts. Eye (Lond). 1999 Jun;13 ( Pt 3b):474-9
  10. Basti S, Greenwald MJ. Principles and paradigms of pediatric cataract management. Indian J Ophthalmol. 1995 Dec;43(4):159-76
  11. Morgan KS. Pediatric cataract and lens implantation. Curr Opin Ophthalmol. 1995 Feb;6(1):9-13
  12. Arkin M, Azar D, Fraioli A. Infantile cataracts. Int Ophthalmol Clin. 1992 Winter;32(1):107-20
  13. Keech RV, Tongue AC, Scott WE. Complications after surgery for congenital and  infantile cataracts. Am J Ophthalmol. 1989 Aug 15;108(2):136-41.

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