Congenital Cystic Adenomatoid Malformation

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Congenital Cystic Adenomatoid Malformation

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= Abstract =

An Intra abdominal Pulmonary Sequestration Containing Congenital Cystic Adenomatoid Malformation

Suk-koo Lee, M.D., Woo Yong Lee, M.D., Hyun Hahk Kim, M.D., FACS, FAAP.

Division of Pediatric Surgery, Department of Surgery, Samsung Medical Center Seoul, Korea

Pulmonary sequestration is a complex anomaly involving the pulmonary paren- chymal tissue and its vasculature. It presents as a cystic mass of· nonfunctional lung tissue without communication with the tracheobronchial system. Usually, it receives blood supply from anomalous systemic vessels. Therefore, preoperative diagnosis of the pulmonary sequestration is difficult, especially when it is located in the abdomen and combined with congenital cystic adenomatoid malformation (CCAM). We encountered such a mass(CCAM type 2) detected prenatally by ultrasonography. It was a kidney bean shaped, pinkish mass straddling the thorax and abdomen on the right side. Because of the sonographic appearance, neu- roblastoma was diagnosed preoperatively. The mass was completely extirpated without difficulty.

Index Words: Pulmonary sequestration, Congenital cystic adenomatoid malformation, Intraabdominal.

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Fig. 1. Lateral X-ray film shows the mass strad·

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Fig. 3. Photomicroscopic findings showing "back to back" cystic space( A) lined by cilliated colum·

nar epithelium, typical of CCAM type 2(B).

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Table 1. Clinical Features of Congenital Cystic Adenomatoid Malformation by Types'6

Type 1 Type 2 Type 3

Frequency( % ) 65 25 10

Age at diagnosis Usually first week to month of Exclusively first year Exclusively first days life, but rarely in adults of life to months of life

Other anomalies present( % ) 8 56 0

Cyst size(cm) 3-10 0.5-3 <0.5

Predominant epithelium Respiratory ( ciliated Ciliated columnar Cuboidal with some pseudostratified columnar) and cuboidal ciliation Other microscopic elements ( %)

mucous cells 32

cartilage 10

striated muscle 0

Presence of intervening

pulmonary parenchyma Yes

Prognosis Good

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1. Weinbaum PJ, Bors-Koefoed R, Green K:

Antenatal sonographic findings in a case of intra abdominal pulmonary sequestrations. Obstet Gynecol 73: 860-861, 1989 2. Aulicino MR, Reis ED, Dolgin SE, Unger

PO, Shah KD: Intra abdominal pulmonary sequestration exhibiting' congenital cystic adenomatoid malformation. Arch Pathol Lab Med 118: 1034-1037, 1994

3. Pryce OM: Lower accessory pulmonary artery with intralobar sequestration of lung; A report of seven cases. J Pathol 58: 457-467, 1946

4. Nikaidoh H, Swenson 0: The ectopic ori- gin of the right main bronchus from the esophagus; A case of pneumonectomy in a neonate. J Thorac Cardiovasc Surg 62:

151-160, 1971

5. Gebauer PW, Mason CB: Intralobar pulmonary sequestrations associated with anomalous pulmonary vessels; A nonentity.

Dis Chest 30: 282-288, 1959

6. Buntain WB, Woolley MM, Mahour GH: Pulmonary sequestration in children; A twenty-five year experience. Surgery

81:

413-420, 1977

7. Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R: Lung sequestration: Report of seven cases and reveiw of 540 published cases. Thorax 34:96-101, 1979

8. O'mara CS, Baker RR, Jeyasingham K:

Pulmonary sequestration. Surg GynecQI Obstet 147:609-616, 1978

9. Carter R: Pulmonary sequestration (collec- tive Review). Ann Thorac Surg 7:66-70, 1969

10. Stocker JT: Sequestrations of the lung.

Semin Diagn Pathol 3:106-121, 1986 11. Tonkin ILD, Allen RG, Riggs W, Satter-

white C: Double aortic arch associated with pulmonary sequestration. Cardiovasc Interventional Radiol 4:264-267, 1981 12. Mariona F, McAlpin G, Zadar I: Sono-

graphic detection of fetal extraihoracic pulmonary sequestration. J Ultrasound Med5:283-285, 1986

13. Black MD, Bass J, Martin DJ, Carpenter BF: Intraabdominal pulmonary sequestration. J Pediatr Surg 26:1381-1383,1991 14. Dibden LJ, Fischer JD, Zubervuhler PC:

Pulmonary sequestration and congenital cystic adenomatoid malformation in an in- fant. J Pediatr Surg 21: 731-733, 1986 15. Hirose R, Suita S, Taguchi T, Koyanagi

T: EXlralobar pulmonary sequestration mi- micking cystic adenomatoid malformation in prenatal sonographic appearance and histological findings. J Pediatr Surg 30:

(5)

1390-1393, 1995

16. Stocker JT, Madwell JE, Drake RM: Con- genital .cystic

,

adenomatoid malformation of the lung; classification and morphologic spectrum. Hum Pathol 8: 155-171, 1977 17. Stocker JT: The respiratory tract, in

Stocker JT, Dehner LP(eds): Pediatric pa-

thology. Philadelphia, P A, JB Lippincott 1992, Pp505-531

18. Zangwill BC, Stocker JT: Congenital cystic adenomatoid malformation within an ex- tralobar pulmonary sequestration. Pediatr Pathol 13:309-315, 1993

Congenital Cystic Adenomatoid Malformation