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Malformazione Adenomatoide Cistica Congenita del Polmone fetale (CCAM)

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Malformazione Cistica Adenomatoide Congenita del Polmone (CCAM): rara lesione amartomatosa cistica, di solito monolaterale, che interessa il polmone di 1/4.000 embrioni descritta per la prima volta nel 1949 da  Ch’in e Tan (1). E’ dovuta ad una eccessiva crescita dei bronchioli (cisti bronchiale) e mancato sviluppo degli alveoli terminali. Provoca, oltre a ipoplasia polmonare, spostamento del mediastino, inversione diaframmatica, polidramnios da ostacolata deglutizione da parte dell’esofago compresso. L’ascite e l’idrope fetale possono comparire in caso di lesioni gravi interessanti i 2/3 dell’emitorace. Frequentemente si associano agenesie e disgenesie renali, atresia digiunale, tetralogia di Fallot, ernia diaframmatica, e anomalie scheletriche (2,3).

Classificazione sec. Stocker:

Tipo 1: macrocisti: cisti con diametro 2-10 cm

Tipo 2: cisti con diametro <2 cm

Tipo 3: con diametro <0.5 cm

Diagnostica USGEcograficamente, dalla 16a w, si visualizzano una o più aree rotondeggianti prevalentemente anecogene (tipo 1), ipoecogene (tipo 2) o iperecogene (tipo 3). La diagnosi differenziale va fatta con tutte le lesioni cistiche intratoraciche: ernia diaframmatica per la presenza di anse intestinali, igroma cistico, cisti broncogene, sequestro polmonare, enfisema lobare congenito e amartoma della parete toracica.

Outlook: la prognosi  correlata al volume (calcolato secondo la formula delle ellissi: Altezza x Larghezza x Profondità x 0.52) della lesione e la contemporaneità di idrope e polidramnios ed è quasi sempre buona in caso di lesione unica senza idrope e polidramnios con una percentuale di sopravvivenza dell’80%. Il ischio di insorgenza dell’idrope fetale è calcolato con il rapporto fra volume della lesione e la circonferenza cranica. Un valore >1.6 indica alta probabilità di insorgenza di idrope (3).

Terapia:

  1. Monitoraggio seriale della lesione e nessun trattamento. Frequentemente si assiste ad una risoluzione spontanea della patologia (4)
  2. beta-metasone: con scarsi risultati (5-7).
  3. IVG
  4. shunt toraco-amniotico in caso di cisti unica dominante
  5. EXIT: resezione chirurgica della cisti o resezione segmentaria o  lobare con una procedura particolare chiamata  Ex Utero intrapartum Therapy (EXIT). Si tratta di un parto cesareo in cui il ginecologo consegna il feto ai chirurghi i quali eseguono l’intervento mentre il bambino è ancora attaccato alla placenta. Quando l’intervento polmonare è completato e il bambino è stabile, si taglia il cordone ombelicale  e il bambino viene consegnato ai pediatri della TIN (8-10). La percentuale di sopravvivenza all’intervento è del 40-60% circa (10). Il neonato sarà sottoposto alla respirazione assisita possibilmente con ventilazione ad alta frequenza oscillatoria (HFOV) detta anche ventilazione jet. A differenza della ventilazione tradizionale, con cui il neonato viene fatto respirare ad un ritmo di 30-60 respiri al minuto, con questa tecnica l’aria viene pompata continuamente nei polmoni che oscillano ad una frequenza di 600-900 oscillazioni al minuto. La ventilazione oscillatoria ad alta frequenza (HFOV) rilascia volumi molto piccoli (spesso più piccoli dello spazio morto) a frequenze estremamente elevate. La ventilazione oscillatoria è unica perché l’espirazione è generata attivamente, in contrapposizione ad altre forme di ventilazione assistita in cui l’espirazione è passiva. La HFOV riduce: i rischi di un intervento chirurgico per chiudere il dotto di Botallo le forme gravi di retinopatia 
  6. il danno polmonare indotto dalla ventilazione meccanica (VILI). La ventilazione non convenzionale riduce il volutrauma (eccessivo volume corrente erogato) e l’atelettrauma (bassa o inadeguata pressione di fine espirazione).

References list:
  1. Ch’in KY, Tang MIA: “Congenital adenomatoid malformation of one lobe of a lung with general anasarca”. Arch Pathol. 1949; 48 :221–229.
  2. Gornall AS, Budd JL, Draper ES, et al.: “Congenital cystic adenomatoid malformation: accuracy of prenatal diagnosis, prevalence and outcome in a general population. Prenatal Diagn. 2003; 23 :997–1002.
  3. Gornall AS, Budd JL, Draper ES, et al.: “Congenital cystic adenomatoid malformation: accuracy of prenatal diagnosis, prevalence and outcome in a general population. Prenatal Diagn. 2003; 23 :997–1002.
  4. Ehrenberg-Buchner, S; Stapf, AM; Berman, DR; Drongowski, RA; Mychaliska, GB; Treadwell, MC; Kunisaki, SM: “Fetal lung lesions: can we start to breathe easier?”. Am. J. of obstetrics and gynecology ; 2012;208 (2): 151.e1–7
  5. Butterworth SA, Blair GK.: “Postnatal spontaneous resolution of congenital cystic adenomatoid malformations”. J Pediatr Surg. 2005; 40 :832–834
  6. Curran PF, Jelin EB, Rand L, Hirose S, Feldstein VA, Goldstein RB, Lee H (2010). “Prenatal steroids for microcystic congenital cystic adenomatoid malformations”J Pediatr Surg. 45 (1): 145–50.
  7. Peranteau WH, Wilson RD, Liechty KW, Johnson MP, Bebbington MW, Hedrick HL, Flake AW, Adzick NS (2007). “Effect of maternal betamethasone administration on prenatal congenital cystic adenomatoid malformation growth and fetal survival”Fetal Diagn Ther 22 (5): 365–371.  
  8. Shanmugam G, K MacArthur, Pollock J: “Congenital lung malformations – antenatal and postnatal evaluation and management. Eur J Cardiothoracic Surg. 2005; 27 :45–52.
  9. Hedrick HL: “Ex utero intrapartum therapy”. Sem Pediatr Surg. 2003; 10 (3):190–195. 
  10. Hedrick HL, Flake AW, Crombleholme TM, et al.: “The ex utero intrapartum therapy procedure for high-risk fetal lung lesions. J Pediatr Surg. 2005; 40 :1038–1043. 


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