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Spontaneous Pneumothorax in Cystic Adenomatoid Malformation
1987; 42:681-88 4 Smith BAC, Hopkinson RB . Tracheal mpture during anaesthesia. Anaesthesia 1984; 39:894-98 5 Guernelli N, Bragalia RB, Bricolli A, Mastrorilli M, Vecchi R. Tracheobronchial ruptures due to cuffed Carlens tubes. Ann Thorac Surg 1979; 28:66-8 6 Freiberger JJ. An unusual presentation of an airway tear. Anesthesiology 1984; 61 :204-()6 7 Amauchi W, Birolini D, Bran(.~> PD. De Oliveira MR. Injuries to the tracheobronchial tree in closed tmuma. Thorax 1983: 38:923-28 8 Rollins RJ, Tocino I. Early radiographic signs of tracheal rupture. AJR 1987; 148:695-98 9 Kelly JP. Webb WR, Moulder PV. Everson C, Burch BH, Lindsey ES. Management of airway trauma: I. tracheobronchial injuries. Ann Thorac Surg 1985: 40:551-55 10 De La Rocha AG, Kayler D. Traumatic mpture of the tracheobronchial tree. Can J Surg 1985: 28:68-71 ll Scott Jones W, Mavroudis C, Richardson D, Gray LA, HoweR . Management oftmcheobronchial dismption resulting from hlunt trauma. Surgery 1984; 95:319-22 12 Petterson C, Deslauriers J, McClish A. A classic image of (.•>mplete right bronchus avulsion. Chest 1989; 96:1415-17
Spontaneous Pneumothorax in Cystic Adenomatoid Malformation* Unusual Clinical and Histologic Features
FIGURE I. Lateral chest roentgenogmm showing multiloculated cyst occupying the right middle lohe.
surrounding lung parenchyma. In older children, it may be associated with recurrent respiratory infections or it may be an incidental radiologic finding. 2 A spontaneous pneumothorax is a rare, but recognized, presentation ofCCAM in older children, but to our knowledge, it has been reported only in two newborns, and in both of these cases it was related to resuscitative measures.'' Presented herein is the case of a newborn with a congenital cystic lesion of the lung who presented with tension pneumothorax in the absence of any resuscitative measures. The unusual clinical and histologic features of this patient are discussed.
L£ah Bentur, M.D.; Gerald Canny, M.D., F.C.C.P.; lbul Thorner, M.D ., Ph .D.; Ricardo Superina, M. D.; lbul Babyn, M.D.; and Henry L£vison, M.D .
Pneumothorax is a rare presentation of congenital cystic adenomatoid malformation (CCAM) in the newborn period and is presumed to be due to resuscitative measures. A previously well three-week-old baby presented with spontaneous tension pneumothorax due to CCAM. In the lung resection specimen, a malformation was seen, which in addition to the histologic changes of CCAM, showed diffuse vascular proliferation in the interstitium and lining of air space by type 2 pneumocytes. We propose that this is a new variant of CCAM rather than one of the classic three types. The unusual clinical manifestation may be related to the unusual histologic features. (Chest 1991; 99:1292-93) CCAM =congenital cystic adenomatoid malformation
C
ongenital cystic adenomatoid malformation (CCAM) of the lung is a rare form of lung disease that has been classified into three types based on clinical and histologic criteria of Stocker et al.' In the neonatal period, this condition presents as progressive respiratory distress as a result of expansion of the cysts and compression of the *From the Division of Chest Medicine (Drs. Bentur, Canny, and Levison), Departments of Pathology (Dr. Thorner), Surgery (Dr. Superina), and Radiology (Dr. Bahyn), The Hospital for Sick Children, Toronto, Canada. Reprint requests: Dr. L£vison, Hospital for Sick Chilclnm, Tcmmto, Ontario, Ganado MSG LX.8
FIGtiRE 2. Microst'lpic section of lung showing partially collapsed cystic air spaces separated by thickened septa (hematoxylin-eosin. original magnifieation x 65).
1292
Spontaneous
Pneumothorax in Cystic Adenomatoid Malformation (Bentur et sl)
CASE REPORT
A three-week-old baby girl presented to the Emergt•ncy Department with acute respiratory distress of 12-hours' duration. She was the product of a normal pregnancy, without a history of hydramnios, and was delivered by elective cesarean section. Birth weight was 3 kg and Apgar St'Ores were 9 at 1 minute, and 9 at 5 minutes. She was discharged home 72 hours after delivery, and did not have any respiratory problems. Twelve hours prior to hospital admission, rapid and labored breathing developed that was associated with grunting, poor appetite, and a weak cry. On physical examination, her temperature was 37.2°C and respiratory rate was 72 breaths per minute; she was cyanosed, gnmting, and using her accessory muscles. Breath sounds were diminished over the right hemithorax. Findings from the rest of the physical examination were unremarkable. Initial chest roentgenogram showed a right tension pneumothorax. A chest tube was inserted; a repeated chest roentgenogram showed that the right lung had reexpanded but now a multiloculated cystic lesion was visualized in the right middle lobe (Fig 1). A computed tomographic scan of the chest revealed variable-sized cystic lesions in the right middle lobe with thin septation; the largest cyst measured 3 em in diameier. During the examination, the chest tube was inadvertently removed and a right tension pneumothorax developed with compression of the right upper and lower lobes. The clinical and radiologic picture supported the diagnosis of a mngenital cystic lesion, most probably a CCAM of the lung. At thorat•>tc>my, the lesion appeared to be unilocular and t•mfined to the right middle lobe. Except for compression by the mass, the upper and lower lobes appeared normal. A right middle lobectomy was performed. Pathologic examinations revealed a 3.5 X 3 X 2-cm thin-walled cystic stmcture filled with hemorrhagic tissue. Microsmpic sections showed a diffusely abnormal architecture with numerous large, partially mllapsed air spaces lined by a single flattened layer of nonciliated epithelium (Fig 2). Within the air spaces, there were t•>llections of macruphages, some t•mtaining hemosiderin, and occasional multinucleated giant cells. Occasional normal limits. The bronchioles were seen that appeared "~thin interstitial portion of the lung was occupied by a proliferation of small vascular channels lined by plump benign endothelium, which VIII-positive by immunoperoxidase staining. In some was l~tctor areas. these channels had the appearance of a capillary hemangioma while, in other areas, they more closely resembled organizing granulation tissue. None of the large cystic spaces was lined by endothelium. The pleura showed no abnormality. The lining epithelial cells were determined to be type 2 pneumocytes by elf'ctron mk-roscopy (Fig 3). The patient was discharged home af"tt,r a short and tuwventful recovery and has re mained clinically wPil during a six-month period of follow-up.
DISCliSSI0!\1
Congenital cystic adenomatoid mallimnation is charach·rizt•d hy anomalous fetal developnwnt of h•rminal respiratory structures, resulting in an adt•nomatoid proliferation of bronchiolar eleme nts and cyst formation .' The radiologic pictun• and macroscopic appearance in our patient demonstrated features of CCAM . The microscopic findings showed sonw features of CCAM as described in tlw literature. Normal pulmonary architecture was replaced by a disorganizt>d overgrowth of cysts lined by nonciliated epithelium. Two ft•atures were unusual for CCAM . First, the air spaces there was were lined with type 2 pneumocytes and st·<.~md, a difl'use vascular prolife ration in the interstitial region resembling a capillary hemangioma. Tlw endothelium was positivt> for factor VIII indicating these were blood vessels
FIGURE 3. Electron micrograph showing spaces lined by type 2 pneumocytes, containing surfactant bodies (arrows). A= air space; C =capillary (original magnification X 6,600).
rather than lymphatic channels. In contrast to pulmonary hemangiomatosis,' the vessels had not infiltrated the peribronchovascular connective tissue, interlobular septa, or pleura. In addition, the benign course in the follow-up period makes this diagnosis unlikely. The patient described herein is believed to be the first newborn with CCAM to present with pneumothorax without a history of previous resuscitative measures. The unusual presentation may be the result of the histologic features or an unrelated phenomenon. We suggest that this is a new variant of a CCAM rather than one of the classic three types. ACKNOWLEDGMENTS: Drs. T. Stocker and E. Popek at the Armed Fort·es Institute of Patlmlof..')", Washington, DC, revit•wt•d tht• pathologic findings of this case. REFERENCES
• 1 Stocker JT, Madewell JE, Drake RM . Congenital t·ystic adenomatoid malformation of the lung. Hum Patholl977; 8:155-71 2 Wolf SA, llertlzer Jll, Philippart AI. Cystic adenomatoid dysplasia of the lung. J Pediatr Surg 1979; 15:925-30 3 Gaisie G. Sang Oh K. Spontaneous pneumothorax in cystic adenomatoid malformation. Pediatr Radiol1983; 13:281-&'3 4 Magee F. Write JL. Kay JM. Pen•tz D. Donovan R, Churg A. Pulmonary capillary hemangiomatosis. Am Rev Respir Dis 1985; 132:922-25 CHEST I 99 I 5 I MAY, 1991
1293
Spontaneous Pneumothorax in Cystic Adenomatoid Malformation* Unusual Clinical and Histologic Features
FIGURE I. Lateral chest roentgenogmm showing multiloculated cyst occupying the right middle lohe.
surrounding lung parenchyma. In older children, it may be associated with recurrent respiratory infections or it may be an incidental radiologic finding. 2 A spontaneous pneumothorax is a rare, but recognized, presentation ofCCAM in older children, but to our knowledge, it has been reported only in two newborns, and in both of these cases it was related to resuscitative measures.'' Presented herein is the case of a newborn with a congenital cystic lesion of the lung who presented with tension pneumothorax in the absence of any resuscitative measures. The unusual clinical and histologic features of this patient are discussed.
L£ah Bentur, M.D.; Gerald Canny, M.D., F.C.C.P.; lbul Thorner, M.D ., Ph .D.; Ricardo Superina, M. D.; lbul Babyn, M.D.; and Henry L£vison, M.D .
Pneumothorax is a rare presentation of congenital cystic adenomatoid malformation (CCAM) in the newborn period and is presumed to be due to resuscitative measures. A previously well three-week-old baby presented with spontaneous tension pneumothorax due to CCAM. In the lung resection specimen, a malformation was seen, which in addition to the histologic changes of CCAM, showed diffuse vascular proliferation in the interstitium and lining of air space by type 2 pneumocytes. We propose that this is a new variant of CCAM rather than one of the classic three types. The unusual clinical manifestation may be related to the unusual histologic features. (Chest 1991; 99:1292-93) CCAM =congenital cystic adenomatoid malformation
C
ongenital cystic adenomatoid malformation (CCAM) of the lung is a rare form of lung disease that has been classified into three types based on clinical and histologic criteria of Stocker et al.' In the neonatal period, this condition presents as progressive respiratory distress as a result of expansion of the cysts and compression of the *From the Division of Chest Medicine (Drs. Bentur, Canny, and Levison), Departments of Pathology (Dr. Thorner), Surgery (Dr. Superina), and Radiology (Dr. Bahyn), The Hospital for Sick Children, Toronto, Canada. Reprint requests: Dr. L£vison, Hospital for Sick Chilclnm, Tcmmto, Ontario, Ganado MSG LX.8
FIGtiRE 2. Microst'lpic section of lung showing partially collapsed cystic air spaces separated by thickened septa (hematoxylin-eosin. original magnifieation x 65).
1292
Spontaneous
Pneumothorax in Cystic Adenomatoid Malformation (Bentur et sl)
CASE REPORT
A three-week-old baby girl presented to the Emergt•ncy Department with acute respiratory distress of 12-hours' duration. She was the product of a normal pregnancy, without a history of hydramnios, and was delivered by elective cesarean section. Birth weight was 3 kg and Apgar St'Ores were 9 at 1 minute, and 9 at 5 minutes. She was discharged home 72 hours after delivery, and did not have any respiratory problems. Twelve hours prior to hospital admission, rapid and labored breathing developed that was associated with grunting, poor appetite, and a weak cry. On physical examination, her temperature was 37.2°C and respiratory rate was 72 breaths per minute; she was cyanosed, gnmting, and using her accessory muscles. Breath sounds were diminished over the right hemithorax. Findings from the rest of the physical examination were unremarkable. Initial chest roentgenogram showed a right tension pneumothorax. A chest tube was inserted; a repeated chest roentgenogram showed that the right lung had reexpanded but now a multiloculated cystic lesion was visualized in the right middle lobe (Fig 1). A computed tomographic scan of the chest revealed variable-sized cystic lesions in the right middle lobe with thin septation; the largest cyst measured 3 em in diameier. During the examination, the chest tube was inadvertently removed and a right tension pneumothorax developed with compression of the right upper and lower lobes. The clinical and radiologic picture supported the diagnosis of a mngenital cystic lesion, most probably a CCAM of the lung. At thorat•>tc>my, the lesion appeared to be unilocular and t•mfined to the right middle lobe. Except for compression by the mass, the upper and lower lobes appeared normal. A right middle lobectomy was performed. Pathologic examinations revealed a 3.5 X 3 X 2-cm thin-walled cystic stmcture filled with hemorrhagic tissue. Microsmpic sections showed a diffusely abnormal architecture with numerous large, partially mllapsed air spaces lined by a single flattened layer of nonciliated epithelium (Fig 2). Within the air spaces, there were t•>llections of macruphages, some t•mtaining hemosiderin, and occasional multinucleated giant cells. Occasional normal limits. The bronchioles were seen that appeared "~thin interstitial portion of the lung was occupied by a proliferation of small vascular channels lined by plump benign endothelium, which VIII-positive by immunoperoxidase staining. In some was l~tctor areas. these channels had the appearance of a capillary hemangioma while, in other areas, they more closely resembled organizing granulation tissue. None of the large cystic spaces was lined by endothelium. The pleura showed no abnormality. The lining epithelial cells were determined to be type 2 pneumocytes by elf'ctron mk-roscopy (Fig 3). The patient was discharged home af"tt,r a short and tuwventful recovery and has re mained clinically wPil during a six-month period of follow-up.
DISCliSSI0!\1
Congenital cystic adenomatoid mallimnation is charach·rizt•d hy anomalous fetal developnwnt of h•rminal respiratory structures, resulting in an adt•nomatoid proliferation of bronchiolar eleme nts and cyst formation .' The radiologic pictun• and macroscopic appearance in our patient demonstrated features of CCAM . The microscopic findings showed sonw features of CCAM as described in tlw literature. Normal pulmonary architecture was replaced by a disorganizt>d overgrowth of cysts lined by nonciliated epithelium. Two ft•atures were unusual for CCAM . First, the air spaces there was were lined with type 2 pneumocytes and st·<.~md, a difl'use vascular prolife ration in the interstitial region resembling a capillary hemangioma. Tlw endothelium was positivt> for factor VIII indicating these were blood vessels
FIGURE 3. Electron micrograph showing spaces lined by type 2 pneumocytes, containing surfactant bodies (arrows). A= air space; C =capillary (original magnification X 6,600).
rather than lymphatic channels. In contrast to pulmonary hemangiomatosis,' the vessels had not infiltrated the peribronchovascular connective tissue, interlobular septa, or pleura. In addition, the benign course in the follow-up period makes this diagnosis unlikely. The patient described herein is believed to be the first newborn with CCAM to present with pneumothorax without a history of previous resuscitative measures. The unusual presentation may be the result of the histologic features or an unrelated phenomenon. We suggest that this is a new variant of a CCAM rather than one of the classic three types. ACKNOWLEDGMENTS: Drs. T. Stocker and E. Popek at the Armed Fort·es Institute of Patlmlof..')", Washington, DC, revit•wt•d tht• pathologic findings of this case. REFERENCES
• 1 Stocker JT, Madewell JE, Drake RM . Congenital t·ystic adenomatoid malformation of the lung. Hum Patholl977; 8:155-71 2 Wolf SA, llertlzer Jll, Philippart AI. Cystic adenomatoid dysplasia of the lung. J Pediatr Surg 1979; 15:925-30 3 Gaisie G. Sang Oh K. Spontaneous pneumothorax in cystic adenomatoid malformation. Pediatr Radiol1983; 13:281-&'3 4 Magee F. Write JL. Kay JM. Pen•tz D. Donovan R, Churg A. Pulmonary capillary hemangiomatosis. Am Rev Respir Dis 1985; 132:922-25 CHEST I 99 I 5 I MAY, 1991
1293