l NI)IAN Vol. 40

JOUnNA L

OF P E D I A T R , ICS

December, 1973

No. 311

HYPOPLASIA OF THE LUNG IN THE NEWBORN* An Autopsy Study ZAKIA SULTANA, V.H. TALIB,S.D. PATIL, M.S. DEStIPANDE AND K.D. SHARMA

Aurangabad Hypoplasia of the lung denotes underdevelopment of the alveolar tissue with a normal bronchial, tree; it may be unilateral, lobar, segmental or rarely bilateral. T h e condition may be primary or secondary (Levine and Mascia 1966). Primary hypoplasia of the lung is a rare condition and is more often seen on the left side. It is associated with other defects such as cardiac and skeletal abnormalities, agenesis of kidneys and Potter's syndrome (Levine and Mascia 1966). Secondary hypoplasia of the lung is the term applied to subnormal lung volume which may result from any condition causing reduced capacity of the thoracic cage or reduced space for p u l m o n a r y growth in early foetal life. Thus it occurs in nearly all cases of congenital diaphragmatic hernia and hydrothorax, and in most of sketetal anomalies like achondroplasia, osteogenesis imperfecta, cardiomegaly, intrathoracic cysts, bronchial stenosis, compression bv an anomalous pulmonary artery and anencephaly. Polycystic kidneys may diminish the thoracic space but there is no correlation between the *From the Department of Pathology, Medical College, Aurzngabad. Received on October 22, 1973.

size of such kidneys and the degree o f pulmonary hypoplasia (Aherne 1964). In view of the rarity of hypoplasia of the lung, we describe 13 cases found at autopsy at the Medical College Hospital, Aurangabad.

Material and Methods Autopsies were carried out on 165 newborn infants dying in the perinatal period, fl'omJanuary 1970 to June 1972. Complete autopsy was performed in all the cases. Lungs from each necropsy were carefully removed, weighed, and evidence of respiration was carefully recorded. Lungs appearing very small and showing on microscopy, proportionately numerous bronchi and reduction in alveolar d'lcts and alveoli with increase in interstitial connective tissue were diagnosed as hypoplasia according to the criteria of Aherne (1964). Results Hypoplasia of the hmg was observed in 13 cases. Lungs were small in size (Fig. 1 Plate I) and weighed b e t w e e n S - 1 0 G . T h e lungs wereateleetatic, congested, solid and liver-like in consistency. O n microscopy the lungs showed areas of underdeveloped air sacs which showed incomplete expansion

420

VoL. 40, No. 311

INDIAN JOURNAL OF PEDIATRICS

or collapse. These air sacs were lined by cuboidal epithelium with vacuolated cytoplasm (Fig. 2 Plate I), with well developed br~nchl (Fig. 3 Plate 1I) and relative increase in the amount of connective tiss,ue. In most of the cases blood vessels showed congestion. The association with different anomalies is shown in Table 1. Discussion

Pulmonary hypoplasia has been shown to be associated with various anomalies which include absence of vagus nerve, absence of il~ilateral main branch of the pulmonary artery, microcephaly, anterior thoracic spina bifida, massive polycystic kidneys, skeletal malformations of the thorax, hydrothorax and renal agenesis (Levine and Mascia 1966). In the present study 5 cases (38.4 per cent) had associated anencephaly. This association has not been well explained but in most cases of anencephaly hypoplasia has been observed (Aherne 1964). Attention has mostly been given to congenital dia-

T a b l e 1.

phragmatic hernia because this is a remediable condition. Potter (1962) stated that with moderate degrees of hypoplasia the foetal lung is less mature than might no,-mally be expected, and that the bronchi are diq)roporti,mately nemerous, the alveoli and alveolar ducts being reduced in number. Areechan and Reid (1963) describe the necropsy findings in 5 cases of congenital diaphragmatic hernia in which there was hypoplasia of the lung, alld mention the same structural disparities as does Potter (1962). Pulmonary anoxia may occur in hypoplastic lungs which are most often associated with other congenital malformations especially diaphragmatic hernia (Areechan and Reid 1963). Presumably, herniation of viscera into tile chest interferes with lung growth on the affected side and the degree of hypoplasia depends on the degree of reduction in thoracic size (Potter 1962). W a h a l a n d Gupta (1967) reported the incidence of hypoplasia of the lung in diaphragmatic hernias as 1.7 per cent,

Associatedmalformations.

Associated anomalies Aneneephaly Diaphragmatic hernia Anophthalmitis with multiple skeletal anomalies Hydrocephalus Eventration of abdominal viscera with sacrococcygeal dermoid Renal agenesis

Number of cases

PLATE

I

INmAN JOVRNAI- OF PFI3I.X'rRIC

Fig. 2, Hypoplastic lung with atelectasis and alveoli lined by many layers of cuboidal cells with clear c vtopla,m .II & E x 4(10)

Fig. 1. Gross ahotograph of hypoplasia of the lung in a case of diaphragmatic hernia after reduction of herniated visera.

,-...

nt

i

rlr

Fig. 3. Microphotograph showing well formed bronchi. compressed Mveoli lined by cuboidal epithelium, septa showing congestion and increased connective tissue (H & E x 100,. SULTANA ET AL.--HYPOPI.ASIA ()F THI- LUNG IN TIlE NEV'.'B()RN.

INDIAN JOURNAL OF PEDIATRIGS

Fig. 1 Calcinosis in tissues of thoracic cage.

Fig. 2.

Calcium deposits in the popliteal fossa and calf.

SACHDEV ET AL.~DERMATOMYO$ITIS.

PLATE

II

SULTANA ET AL.--HYPOPI,ASIA OF THE LUNG IN THE NILXVBORN closely similar to the figure in the present study. H y p o p l a s i a o f the lung appears once ill every 2200 births and in 1 . 2 - 1 . 4 per cent of" all perinatal necropsies (Buttler and Claireaux 1962). T h e incidence in the present study was 7.8 per cent w h i c h is similar to the d a t a o f Driscoll and S m i t h (1962)--10.9 per cent. In the present study the significance of the association of h y p o p l a s i a with anophthahnitis, hydrocephaly, eventration of a b d o m i n a l viscera and renal agenesis is obscure. T h e association with renal agenesis has been described by A h e r n e (1964) a n d Levine a n d Mascia (1966) as a rare occurrence.

Summary H y p o p l a s i a o f the lungs was observed in 13 cases (7.8 per cent) out o f 165 autopsies p e r f o r m e d at M e d i c a l College IIospital, A u r a n g a b a d . The associated m a l f o r m a t i o n i n c l u d e d anencephaly, d i a p h r a g m a t i c hernia, h y d r o cephalus, skeletal defects a n d renal agenesis.

421

We are thankful to the Dean, Medical Co!lege, Aurangabad, for his permission to publish this paper.

References Aherne, W. (1964). Neonatal Pulmonary Diseases. In Recent Advances in Clinical Pathology. Ed. P.W. Dykes. Churchill, London. p. 284. Areechan, W. andReld, L. (1963). Hypoplasla of lung with congenital diaphragmatic hernia, Bri!. reed. J. 1,230. Buffer, N. and Glairaux, A.E. (1962). Congenital diaphragmatic hernia asa cause of perinatal mortality. Lancet, l, 659. Driscoll, S.G. and Smith, G.A. (1962). Neonatal pulmonary disorders. Pediat. Clin. de. Amer. 9, 325. Levine, H.I. and Maseia, A.V. (1966). Pulmonary diseasesand anomalies of infancy and childhood, tlarper & Row, New York, p. 6. Potter, E.L. (1962). Pathology of Foetus and Newborn. Ylar Book Publishers, Chicago. p. 301. Wahal, K.M. and Gupta, J.S. (1967). Pathology of lungs ia perinatal deaths. Indian o7. Med. Res. 55, 325.