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Pulmonary Developmental Anomaly Associated with KlippeiFeil Syndrome and Anomalous Atrioventricular Conduction* Rnjesh Bhagat, M.D.; &ushal Pant, M.D.; Vyay K. Singh, M.B ., B.S., F.C .C.P.; Chandra.vhekar Pant, M.D.; Ajay Gupta, M.D. ; and Om Prakash }aggi, M.D.

We report the findings in a patient with Klippei-Feil syndrome and associated agenesis of right upper and middle lobes, hypoplasia of the right lower lobe of the lung, and Lown-Ganong-Levine syndrome. To our knowledge, such an association has not been previously described. (Chest 1992; 101:1157-58)

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ulmonary agenesis and hypoplasia may be one of many multisystem anomalies in primary embryologic defect syndrome or may result from decreased intrathoracic space due to other congenital thoracic or spinal defects. o.> KlippeiFeil syndrome, resulting from congenitally fused cervical vertebrae, has been cited to be rarely associated with pulmonary agenesis and hypoplasia. 2 Although many congenital cardiac anomalies have been identified with all of these conditions,'"" to our knowledge, an accessory atrioven*From the Clinical Research Centre {Drs. Bha~at, Pant, and Jaggi) and DST, CVM , ABC Foundation {Dr. Singh), Vallabhbhai Patel Chest Institute, University of Delhi, and the Institute of Nuclear Medicine and Allied Sciences (Drs. Pant and Gupta), Delhi, India.

opaque ri~-tht hemiFJGllRE 1. Bnmchogram of right lung showiu~-t lnn~-t and absence of ri~-tht thorax with herniation of l~>ntralateral upper and middle lobes and hypoplastic right lower lobe.

tricular conduction pathway has never heen demonstrated together with Klippei-Feil syndrom{~ and pulmonary lobar agenesis and hypoplasia. We report the findings in a patient with such an association. CASE REPORT

A 23-year-old woman was referred to us fc>r investigation of an abnormal chest roentgenogram , prompted three months ago by a bout of respiratory tract infection . \Vhen she presented to us. she had no complaint. The patient had never smoked and never suffered from any chronic pleuropulmonary disease. Her menstrual cycle was normal and regular. She had a low hairline and a short neck with painless restriction of movement , more so on the left side. Examination of the chest revealed signs of volume loss of the right side with ipsilateral shift of the mediastinum . Heart sounds we re parasternal reJ.:ion; no added sounds were best heard in the ri~-tht audible. The findinl-(s on mutine investigations of biplastic right lower lobe . Digital subtraction angioJ.:raphy demonstrated a tiny hyJX>plastic right pulmonary artery (Fig 2). Findings on CT and ultrasonographic examination of the al>

Pulmonary developmental anomaly associated with Klippel-Feil syndrome and anomalous atrioventricular conduction.

We report the findings in a patient with Klippel-Feil syndrome and associated agenesis of right upper and middle lobes, hypoplasia of the right lower ...
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