Annals of Thoracic Medicine
CASE REPORT
Year
: 2006  |  Volume : 1  |  Issue : 2  |  Page : 81--83

Pneumonectomy in a child with congenital bronchiectasis: A case report and review of literature


Gowri Shankar1, Paras Kothari1, Dinesh Sarda1, Bharati Kulkarni1, Alka Kalgutkar2,  
1 Departments of Pediatric Surgery, LTMGH, Sion, Mumbai, India
2 Departments of Pathology, LTMGH, Sion, Mumbai, India

Correspondence Address:
Paras Kothari
Department of Pediatric Surgery, L.T.M.M. College and General Hospital, Sion, Mumbai - 400 022
India

Abstract

Williams-Campbell syndrome was first described in 1960 as a rare form of bronchiectasis. Its pathogenesis is characterized by the absence of or markedly diminished bronchial cartilage. We describe a case of a 4-year-old male child in whom respiratory symptoms developed in neonatal period and were found to have histopathological changes consistent with Williams-Campbell syndrome. A brief review of literature has been discussed.



How to cite this article:
Shankar G, Kothari P, Sarda D, Kulkarni B, Kalgutkar A. Pneumonectomy in a child with congenital bronchiectasis: A case report and review of literature.Ann Thorac Med 2006;1:81-83


How to cite this URL:
Shankar G, Kothari P, Sarda D, Kulkarni B, Kalgutkar A. Pneumonectomy in a child with congenital bronchiectasis: A case report and review of literature. Ann Thorac Med [serial online] 2006 [cited 2020 Sep 21 ];1:81-83
Available from: http://www.thoracicmedicine.org/text.asp?2006/1/2/81/27107


Full Text

Williams-Campbell syndrome is a rare form of congenital bronchiectasis.[1] It is associated with congenital deficiency of bronchial cartilage involving 2nd or 3rd through 6th generation bronchi.[2] The structural integrity of the trachea, main and more proximal bronchi is maintained. This leads to distal airway collapse and bronchiectasis.[3]

 Case Report



A 4-year-old male child presented with complaints of chronic cough since birth. There was history of being admitted for pneumonia at day six of life. Since then, he had recurrent episodes of cough with low-grade fever, each episode lasting for a period of 10-15 days. There was no history of breathlessness, hemoptysis and chest pain. The child was full term normal delivery birth with Apgar score of 10. Developmental milestones were normal.

On examination, the child had clubbing. Respiratory examination revealed no chest wall abnormality. There were crepitations with rhonchi in left lung field, with decreased air entry on the same side.

His routine investigations were normal. Sputum was negative for acid-fast bacilli.

Chest roentgenogram revealed right hyper-lucent lung with herniation of the right lobe. High-resolution computerized tomography (HRCT) revealed cystic spaces in the left hemi-thorax, suggestive of bronchiectasis with collapse of left lung and herniation of the right upper lobe. The right lung parenchyma was normal [Figure 1].

He underwent left pneumonectomy. Gross specimen revealed a small nonexpansile lung measuring 9 x 7 x 3 cm and weighing 150 g. The pleural surface was normal. There was no evidence of fibrosis. The cut specimen revealed a dilated bronchial system through the entire length of the lung extending from hilum to the terminal bronchi. The bronchial mucosa was normal. Pulmonary vasculature was normal [Figure 2]. Postoperative recovery was normal. Patient was discharged on 12th postoperative day.

Histopathological examination revealed markedly dilated airways, from the bronchial branches till the bronchioles. They were lined by ciliated epithelium with focal squamous metaplasia. Cartilage plates were seen only in the main branches of bronchus. There was absence of cartilage plates or deficient cartilage in the remaining segmental bronchi. The intervening lung parenchyma was normal [Figure 3].

The child has improved symptomatically and is on regular follow-up. He was totally asymptomatic in his last 2 years of follow-up.

 Discussion



In 1960, Williams and Campbell reported five patients with a very unusual pattern of generalized bronchiectasis, the outstanding feature being soft, compliant bronchi, which dilated and collapsed during inspiration and expiration. Necropsy studies in one of them showed that there was extensive deficiency of the cartilage from the 4th to 6th or 8th generation bronchi.[1]

While the etiology and pathology of this disorder is not completely understood, following is probably the sequence of events. It is postulated that these children were born with deficient cartilage in the bronchial tree. In infancy, viral respiratory infection resulted in small airway obstruction. The highly compliant bronchi would collapse during coughing. Poor drainage resulted in progressive obstructive disease, leading to pulmonary hyperinflation and segmental or lobar collapse.[4]

The cause of the cartilage deficiency is uncertain. There is no familial incidence of the disorder and no observable deficits in the cartilage outside the bronchi.[5]

The differential diagnosis of bronchiectasis is also extensive and includes congenital causes such as Young's syndrome, Mounier-Kuhn syndrome and Williams-Campbell syndrome. Acquired forms of bronchiectasis such as those due to cystic fibrosis, recurrent respiratory infection, aspergillosis, inhalation injuries and pulmonary fibrosis must also be considered.[6] Tracheobronchomegaly can be excluded on the basis of normal caliber of trachea and main bronchi.[7] The extent and distribution of the bronchial abnormalities is not consistent and regular in cystic fibrosis and aspergillosis.[8] The central location of the ectatic bronchi rules out acquired causes.

The clinical symptoms manifest by 3 years of age in 96% of patients and are characterized by intermittent cases of pneumonia and broncho-obstructive symptoms. The clinical course varies, with rapid clinical deterioration and death in some children and prolonged survival in others.[2] A decreased frequency of pneumonia has been observed in adults.[9]

HRCT findings include cystic bronchiectasis distal to the third generation bronchi, hyperinflation of lung and central cystic bronchiectasis. Due to the lack of cartilaginous support, the overtly compliant bronchial wall collapses during expiration with distal air trapping. Computed tomography demonstrated characteristic finding of this syndrome, i.e., ballooning of bronchi on inspiration and collapse on expiration.[7],[8]

The long-term prognosis is variable with rapid clinical deterioration and death in some children and prolonged survival in others.[2]

In our case described here, there was involvement of only the left lung, with the right lung being normal. The review of literature showed two similar cases with unilateral lung involvement.[1] The timing of onset of symptoms in the case reported suggests a congenital nature of the disease condition. Because of the frequent respiratory infections occurring since birth and given the good anatomical condition of the right lung, we recommended left pneumonectomy. There has been a report of similar case requiring pneumonectomy.[9] On follow-up, the operation showed good results with regard to respiratory function, control of infection and quality of life.

References

1Williams H, Campbell P. Generalized bronchiectasis associated with deficiency of cartilage in bronchial tree. Arch Dis Child 1960;35:182-91.
2Jones VF, Eid NS, Franco SM, Badgett JT, Buchino JJ. Familial congenital bronchiectasis: Williams-Campbell syndrome: Pediat Pulmonol 1993;16:263-7.
3Wayne KS, Laussig LM. Probable familial congenital bronchiectasis due to cartilage deficiency. Am Rev Respir Dis 1976;114:15-22.
4Williams HE, Landau LI, Phelan PD. Generalized bronchiectasis due to extensive deficiency of bronchial cartilage. Arch Dis Child 1972;47:432-8.
5Mead J, Turner JM, Macklem PT, Little JB. Significance of the relationship between lung recoil and maximum expiratory flow. J Appl Physiol 1967;22:95-108.
6Cole P. Clinical and imaging aspects of bronchiectasis. In : Potchen EJ, editor, Pulmonary Radiology: The Fleischer Society. W.B. Saunders: Philadelphia; 1993. p. 242-58.
7Watanabe Y, Nishiqama, Kanayama H, Enomoto K, Kato K, Takeichi M. Congenital deficiency due to bronchial deficiency: CT demonstration. J Comput Assist Tomogr 1987;11:701-3.
8Hartman TE, Primack SL, Lee KS, Swensen SJ, Muller NL. CT of bronchial and bronchiolar diseases. Radiographics 1994;14:991-1003.
9Tucciarone l, Tomassini A, Anacierio S, Colasanati A, Papandrea S. Left pneumonectomy in a child with congenital bronchiectasis. Pediatr Med Chir 1996;18:323-5.