Congenital bilobar emphysema

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Journal of Pediatric Surgery (2008) 43, E5–E7

Congenital bilobar emphysema Ali Ghribi a , Mongi Mekki a,⁎, Imed Krichene a , Riadh Jouini a , Kaies Maazoun a , Lassaad Sahnoun a , Mohamed Ben Brahim a , Mohsen Belghith a , Chiraz Hafsa b , Abdelfattah Zakhama c , Abdellatif Nouri a a

Department of Pediatric Surgery, Fattouma Bourguiba Hospital, 5000 Monastir, Tunisia Departement of Radiology, Fattouma Bourguiba Hospital, 5000 Monastir, Tunisia c Department of Pathology, Fattouma Bourguiba Hospital, 5000 Monastir, Tunisia b

Received 15 December 2007; revised 24 March 2008; accepted 25 March 2008

Key words: Bilateral congenital lobar emphysema; Congenital bilobar emphysema; Lobectomy

Abstract Congenital bilobar emphysema is reported to be extremely rare. We describe 3 cases, and we review the diagnosis problems and treatment methods of this childhood respiratory tract pathologic condition. © 2008 Elsevier Inc. All rights reserved.

Congenital lobar emphysema is an infrequent, but important, cause of respiratory distress in the newborn. Congenital emphysema affecting more than one lobe has been rarely reported. Surgical management of bilobar emphysema is controversial. We describe 3 cases of infantile bilobar emphysema, diagnosed preoperatively in one patient and during the follow-up period in the other 2 patients.

1. Case reports 1.1. Case 1 A male infant developed respiratory distress at 1.5 months of age. He was born at term by abdominal delivery.

⁎ Corresponding author. Tel.: +216 98229168; fax: +216 73460678. E-mail address: [email protected] (M. Mekki). 0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.03.069

Pregnancy had been normal. Birth weight was 2500 g. The Apgar score was 9 and 10 at 1 and 5 minutes, respectively. The patient was hemodynamically stable, tachypneic, but with no abnormal lung sounds. Breath sounds were present on the right side. Chest radiography demonstrated a hyperinflated left hemithorax with mediastinal shift to the right and mediastinal hernia (Fig. 1). Computed tomographic (CT) scanning showed marked hyperinflation of the left upper lobe (LUL) and compression of the left lower lobe with mediastinal shift to the right and atelectasis of the right upper lobe (Fig. 2). Radionuclide perfusion scintigraphy demonstrated a perfusion defect in the LUL and a hypoperfused right middle lobe (RML) (Fig. 3). Left upper lobectomy was performed. Postoperatively, the infant was asymptomatic. One month later, he developed respiratory distress of minor degree; CT scanning showed then marked RML emphysema leading to right middle lobectomy. Histologic examination demonstrated distended alveoli. He has been followed for 5 years and no complications have occurred since.


A. Ghribi et al.

Fig. 1 Chest radiography showing hyperinflated left hemithorax with mediastinal shift to the right and a mediastinal hernia.

1.2. Case 2 This male infant was born at full term after an uncomplicated pregnancy. Birth weight was 3300 g. The Apgar score was 9 and 10 at 1 and 5 minutes, respectively. The child had been well until 4 months of age when he developed respiratory distress. The patient was tachypneic with sternal and intercostal retraction. Breath and lung sounds were normal. Chest radiography demonstrated a hyperinflated right hemithorax with mediastinal shift to the left. The CT scanning showed hyperinflation of the right upper and middle lobes. A 1-stage bilobectomy was performed. The patient's postoperative recovery was uneventful. Histologic examination demonstrated distended alveoli. At present, 9 years after the operation, he is doing well with no further problems.

Fig. 3 Radionuclide perfusion scintigraphy showing perfusion defect in the LUL and a hypoperfused RML.

1.3. Case 3 A 3700-g, full-term 40-day-old boy was transferred to our department because of severe respiratory distress. Pregnancy and delivery had been normal. The Apgar score was 9 and 10 at 1 and 5 minutes, respectively. Chest radiography revealed a hyperinflated right hemithorax with herniation to the opposite side. Blood gas sample analysis demonstrated respiratory acidosis with carbon dioxide retention and severe hypoxia. Emergency thoracotomy was performed revealing a grossly hyperexpanded RML that was resected. Immediately after surgery, the child was successfully extubated. Twenty-four hours later, he developed severe respiratory distress. Chest radiograph revealed an emphysematous right upper lobe requiring a lobectomy. The patient was kept on mechanical ventilation for 10 days before dying of a nosocomial infection.

2. Discussion

Fig. 2 Tomography scanning showing hyperinflation of the LUL with mediastinal shift to the right.

Congenital lobar emphysema is an uncommon childhood respiratory tract disease characterized by overinflation of a pulmonary lobe. The exact cause of congenital lobar emphysema is difficult to determine and seems even nonexistent in 50% of cases, as in our 3 patients. The most commonly identified causes are congenital cartilage defect and bronchial obstruction [1]. Bilobar or multifocal involvement is extremely rare [2]. Bilateral localization was more often reported than unilateral localization. Only 9 cases of bilateral emphysema have been reported in the literature [1]. The most commonly affected parts of the lungs are the LUL and RML [1-3]. In our patients, bilobar emphysema was unilateral in 2 cases. In our case reports, the predominance of male patients, reported in the literature [1-3], was confirmed.

Congenital bilobar emphysema Most patients present with moderate respiratory distress. Their symptoms develop in the first few days of life. Severe life-threatening respiratory distress is the next common form of presentation that requires immediate surgical intervention. The least common form of presentation is mild respiratory distress. The patients show symptoms after the neonatal period and usually have recurrent respiratory tract infections and cough. In our cases, the symptoms started later than reported in the literature. The average delay was 70 days. Chest radiography often showed a hyperinflated hemithorax and mediastinal shift to the contralateral site. In the case presenting with a large emphysematous lobe, lung herniation to the contralateral hemithorax and atelectasis of the contralateral lung can be seen. The CT scanning of the chest may reveal bilobar involvement and may show bronchial obstruction. It may have negative results in patients with bilobar emphysema as demonstrated in case 1 because of mediastinal shift, atelectasis, or compressed near lobe [2]. Perfusion scintigraphy can show decreased perfusion secondary to the compression of surrounding blood vessels. It is more sensitive for bilobar involvement and seems appropriate to evaluate cases of congenital lobar emphysema for the possibility of multifocality such as in case 1. Radionuclide ventilation/perfusion scintigraphy is suggested as a complementary imaging method before initial treatment [3]. All reported bilateral cases except for one, required surgical correction, that is, unilateral or bilateral lobectomies [1]. Bilateral lobectomy as a 2-stage procedure was performed in 3 cases. Three other patients had successful 1-stage bilateral lobectomies [1]. In our study, bilateral lobectomies whether they were 1-stage (case2) or 2-stage operations (case 1) were successful in the treatment of bilateral emphysema.

E7 Lung expansion and blood gases immediately postoperatively could help to decide on either 1-stage resection of both lobes or a 2-stage resection [1]. Simultaneous bilateral thoracotomy may result in significant postoperative pain [1]. We strongly believe that an infant tolerates sequential thoracotomy better than simultaneous bilateral thoracotomy. In case of unilateral bilobar involvement, we opted for simultaneous bilobectomies. In cases requiring emergency procedures, recognizing other emphysematous locations is important as these may need resection. Sometimes it is difficult to find a second lobe involved because of nearby lobe compression as in case 3. Thus, postoperative evaluation of the rest of the lung is warranted, especially, as these patients need to be closely and regularly followed up.

3. Conclusion Congenital bilobar emphysema is a rare condition. Diagnosis of bilobar involvement may be difficult. Congenital bilobar emphysema may be managed by simultaneous or sequential bilobectomies. Surgical management should be guided by clinical and radiologic findings and evaluated peroperatively and postoperatively.

References [1] Maiya S, Clarke JR, More B, et al. Bilateral congenital lobar emphysema: how should we proceed? Pediatr Surg Int 2005;21:659-61. [2] Hugosson C, Rabeeah A, Al-Rawaf, et al. Congenital bilobar emphysema. Pediatr Radiol 1995;25:649-51. [3] Ekkelkamp S, Vos A. Successful surgical treatment of a newborn with bilateral congenital lobar emphysema. J Pediatr Surg 1987;22:1001-2.

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