A Case of Middle Lobe Syndrome Occurring in Two Sisters

Hiroshi TAKAHASHI, Shigeru KIMURA, Isao NAGAI, Masayuki KUBOTA and Kouji AONO

ABSTRACT: We report herein a rare case of middle lobe syndrome, occurring in two sisters. Since selective aspirations of sputum under bronchoscopic guidance proved ineffective, a middle lobectomy was per- formed in both cases. Histological findings of the resected specimens showed overgrowth of the bronchial glands, lymphocytic infiltration around the alveoli and glandular metaplasia of the alveoli in both cases. Based on the allergic diathesis, we suggest that in these 2 sisters, middle lobe syndrome was caused by chronic inflammation.

KEY WORDS: middle lobe syndrome, bronchiectasia

INTRODUCTION CASE REPORTS

The tel-m "middle lobe syndrome" was coined by Graham et al. in 1948,1 since when a number of cases have been reported. However, cases of siblings, especially chil- dren, have rarely been encountered. We recently operated on two sisters with middle lobe syndrome, aged 8 and 3 years old, respectively. Both had a history of atopic dermatitis and asthmatic bronchitis and an allergic diathesis of these cases suggested allergy to be one of the causative factors of their middle lobe syndrome. Both sisters showed atelectasis of the lingula after middle lobectomy, which was improved by medical therapy and bronchoscopic suction of spu- tum. The appropriate management of chil- dren with middle lobe syndrome is also discussed herein.

The Second Department of Surgery, School of Medi- dne, Ehime University, Ehime, Japan

Reprint requests to: Hiroshi Takahashi, MD, The Second Department of Surgery, School of Medicine, Ehime University, Shitsukawa, Shigenobu, Onsen-gun, Ehime 791-02, Japan

Case I (the elder sister) An 8 year old girl was admitted to our

surgical department in March, 1988 for re- current pneumonia. Although her father had a history of atopic dermatitis, the family history was otherwise unremarkable. At the age of 1 year she had received medical treatment for asthmatic bronchitis and atopic dermatitis and experienced repeated epi- sodes of pneumonia thereafter. At the age of 6 years, atelectasis of the right middle lobe was noted on a chest X-ray and, since it was refractory to medial therapy, she was re- ferred to our department of surgery. On admission, her skin was dry and breathing sounds in her right lower chest diminished. Another chest X-ray confirmed atelectasis of the right middle lobe (Fig. 1). Allergy testing for egg-white, milk, and house dust proved negative. Bronchoscopic e~tamination dem- onstrated obstruction of the orifice of the middle lobe bronchus by viscous secretions and a bronchogram revealed cylindrical ectasia of the bronchial tree in the right middle lobe (Fig. 2). Because the atelectasis

JAPANESE JOURNAL OF SURGERY, VOL. 20, No. 5 pp. 597-601, 1990

Jpn. J. Surg. 598 Takahashi et al. September 1990

Fig. 1. Case 1. A, Posteroanterior; and B, lateral chest X-ray showing atelectasis of the right middle lobe.

Fig. 2. Case 1. Bronchogram showing cylindrical ectasia of the bronchial tree in the right middle lobe.

was refractory to medical therapy and the selective suction of sputum under broncho- scopic guidance, a right middle lobectomy was per formed in April, 1988. The gross findings revealed nei ther any incomplete inteflobar fissures nor peribronchial lymph node swelling. The middle lobe was dark red a n d showed diffuse a te lectas is and the lumen of the middle lobe bronchus was filled with yellow, viscous sputum. Micro-

Fig. 3. Case 1. Photomicrograph of the resected speci- men showing A, overgrowth of the bron- chial glands; and B, lymphocytic infiltra- tion around the alveoli and glandular metaplasia of the alveoli.

Volume 20 Middle lobe syndrome occurring in two sisters 599 Number 5

Fig. 4. Case 1. Posteroanterior chest X-ray showing ate- lectasis of the lingula 6 months after the operation.

Fig. 5. Case 2. Bronchogram showing slightly cylindri- cal ectasia and a narrowed orifice of the right middl e lobe bronchus.

scopic findings revealed overgrowth of the bronchial glands, lymphocytic infiltration around the alveoli and glandular metaplasia of the alveoli (Fig. 3). Her immediate post- operative course was uneventful, however, 6 months after operation, atelectasis of the lingula appeared (Fig. 4), which was resolved by medical therapy and bronchoscopic aspi- ration of the sputum.

Case 2 (the younger sister) This patient was a 3 year old girl, who

developed coughing and wheezing at the age of 2 years and also had a history of atopic dermatitis. A roentgenographic examination of the chest prior to an inguinal hernia operation incidentally revealed atelectasis of the right middle lobe. She was transferred to our department together with her sister. On admission, her skin was dry and the breath- ing sounds in her right lower chest dimin- ished. The chest X-ray and bronchoscopic findings were similar to those of her sister and a bronchogram showed a slightly nar- rowed orifice of the middle lobe bronchus and bronchiectasia (Fig. 5). Right middle lobectomy was performed on the same day as

her sister's operation. The interlobar fissures were complete and per ibronchiat lymph node swelling was not seen. The atelectatic change was obvious. Histological findings revealed similar signs of chronic inflamma- tion as those seen in her sister and glandular metaplasia of the alveoli was also partially seen. Six months after the operation, atelec- tasis of the lingula similarly sppeared as in her sister, which was also improved by medi- cal therapy.

DISCUSSION

The incidence of middle lobe syndrome is lower in children than in adults, accounting for only about 15 per cent of the total cases2 The diagnosis of middle lobe syladrome in children may be delayed or missed because of non specific symptoms and signs, how- ever, with the increasingly widespread use of bronchoscopy in children, the identification of middle lobe syndrome is expected to increase. This syndrome may actually not be so uncommon in children and is frequently seen in pediatric allergy patients, 3 the ma-

600 Takahashi et al.

Table 1. Sibling Occurrences of Middle Lobe Syndrome

Jpn. J. Surg. September 1990

Year Author Cases Associated Disease

1966 Dees 5 2 Sisters IgA deficiency 1967 Danielson 4 4 of 5 Siblings 1969 Vidal 6 2 Female Members al-antitrypsin deficiency 1987 Livingston 2 2 Brothers Retinitis pigmentosa

jority of whom have asthma or a family history o f atopic disorders, as in the two patients presented herein?, 3 In a survey of the literature, we were only able to find 5 cases of sibling occurrence, including the present case (Table 1). 4-8

Although the etiology of middle lobe syn- drome in children is not well understood, there are two theories now recognized as explanations for its occurrence, la The first view implicates extrinsic bronchial obstruc- tion by pefibronchial lymph node swelling, 1 while more recently, attention has been drawn to the relative anatomical isolation of the middle lobe with resultant poor collateral vent i la t ionY The impairment of collateral ventilation is presently the most accepted theory in cases without evidence of anatomi- cal bronchia l obstruction3 In both our cases, no morpholog ica l abnormal i t ies o f the bronchial cartilage or pulmonary vascular system were present and there were nei ther incomplete inter lobar fissures nor peribron- chial lymph node swelling. For these re- asons, the theory of impaired collateral ven- tilation seems most applicable to our case of siblings. In patients with an atopic back- ground who are susceptible to upper respira- tory infections, once b r o n c h o p n e u m o n i a de- velops, expectoration may become very diffi- cult. Atelectasis may then develop, firstly because of the anatomical features of the middle lobe, such as the narrowness of the middle lobe bronchus and its sharp angle f r o m the i n t e r m e d i a t e b r o n c h u s , a n d secondly, because of the poo r collateral ventilation. I f p roper t reatment is not given, the persisting inflammation might cause or- ganic changes leading to bronchiectasia.

It is difficult to decide the operative indica-

tions for middle lobe syndrome. Billig et al. p roposed that findings on bronchogram are

�9 useful in the decision of therapeutic ap- proach. 8 They r ecommended one month of medical therapy prior to bronchoscopy and b r o n c h o g r a p h y in c h i l d r e n and if the b ronchogram demonstrated abnormalities, then they r ecommended middle lobectomy should be pe r fo rmed within two weeks. If it was normal, however, they recommended medical managemen t be continued for at least ano the r month. Recently, Livingston et al. reported that bronchoscopy produced therapeutic benefits in two-thirds of their cases and that middle lobectomy was curative in the remain ing patients refractory to treat- ment? Since bronchoscopic suction was not effective in our cases, we proceeded to per- form middle lobectomy.

The postoperative course of these patients should be observed very carefully and when atelectasis o f the contralateral lobe appears, appropriate medical m a n a g e m e n t should be performed. It is our impression that early diagnosis and treatment is extremely impor- tant in this clinical entity.

(Received for publication on Dec. 11, 1989)

REFERENCES

1. Graham EA, Burford TH, Mayer JH. Middle lobe syndrome. Postgrad Med 1948; 4: 29-34.

2. Livingston GL, Holinger LD, Luck SR. Right middle lobe syndrome in children. International Journal of Pediatric Ot0rhinolaryngology 1987; 13: 11-23.

3. Thatcher H, Kaplan A. Middle lobe syndrome in asthmatic children. J Maine Med Assoc 1972; 63: 46-50.

4. Danielson GK, Haanson CW, Cooper ED. Middle lobe bronchiectasis. Report of an unusual familial

Volume 20 Middle lobe syndrome occurring in two sisters 601 Number 5

occurrence. J Am Med Assoc 1967; 201: 605-608. 5. Dees SC, Spock A. Right middle lobe syndrome in

children.JAMA 1966; 197: 78-84. 6. Vidal J, Robinet-Levy M, Michel FB. Familial form

of condensation and retraction of the middle lobe with a deficit of alpha-l-antitrypsin. J Fr Med Chir

Thorac 1969; 22: 469. 7. Culiner MM. The right middle lobe syndrome; a

non-obstructive complex. Dis Chest 1966; 50: 50-57.

8. Billig DM, Darling DB. Middle lobe atelectasis in children. AmerJ Dis Child 1972; 123: 96-98.