Intra-abdominal extralobar pulmonary sequestration presenting antenatally as a suprarenal mass

Extralobar pulmonary sequestration is a rare pulmonary parenchymal anomaly which rarely may be present in an intra-abdominal location. The authors report a case of intra-abdominal extralobar pulmonary sequestration which presented to us as an antenatally diagnosed suprarenal mass and was worked up as such. The diagnosis was revealed only at laparotomy. Intra-abdominal extralobar pulmonary sequestration should also be kept in differential diagnosis in cases of masses in the suprarenal location, especially on the left side.     

Laparoscopic resection of intra-abdominal extralobar pulmonary sequestration

The intra-abdominal localization of extralobar pulmonary sequestration (EPS) is an uncommon entity, although there are an increasing number of publications in literature on EPS over recent years. There seems to be a predominance of left-sided suprarenal positioning of the sequester and so far resection has been undertaken by way of laparotomy. This paper describes the laparoscopic resection of EPS in two patients. In both instances the procedure was successful and the postoperative course was uneventful. It is concluded that EPS should be included in the differential diagnosis of suprarenal masses, particularly on the left side. Laparoscopic resection is the method of choice for EPS.     

Infradiaphragmatic extralobar pulmonary sequestration in an infant

A rare case of infradiaphragmatic pulmonary sequestration with an antenatally diagnosed abdominal tumor is reported. Extirpation of the tumor was performed 2 months after delivery; the histological examination showed alveolar and bronchial tissue. Offprint requests to: M. Yagi     

Torsion and infarction of an extralobar pulmonary sequestration

An 8-year-old girl presented with rapid onset of haemorrhagic pleural effusion due to torsion and infarction of an extralobar sequestration of the lung. A literature survey has failed to reveal a similar case.     

Extrathoracic pulmonary sequestration detected by antenatal ultrasonography

Extralobar pulmonary sequestration (ELS) is an uncommon congenital anomaly in which the sequestered tissue is found outside the normal lung and is invested with its own pleural covering. We describe an unusual case of ELS presenting as an abdominal mass detected incidentally during antenatal ultrasonography. At birth, the otherwise healthy 3050 gram fullterm female had an asymptomatic ELS located posteriorly between the right hemidiaphragm and the liver. Extensive investigation culminating in exploratory surgery and resection of the mass yielded the diagnosis. This condition might be more common than is presently recognized, and familiarity with its appearance on antenatal ultrasound should lead to refinement in its diagnosis and management. Correspondence to: G. J. Gross     

Sclerosing haemangioma arising within extralobar pulmonary sequestration

Extralobar pulmonary sequestration is a rare anomaly of abnormal pulmonary tissue without any communication to the bronchial tree. Sclerosing haemangioma is a rare lung tumour, generally seen in middle-aged women. The combination of these two rare pathologies has not been previously reported. We describe the CT and CT angiographic findings of sclerosing haemangioma arising within an extralobar pulmonary sequestration in a 2-year-old girl.     

Infradiaphragmatic extralobar pulmonary sequestration masquerading as an intra-abdominal, suprarenal mass

Three infants were found to have infradiaphragmatic masses by prenatal ultrasound. Postnatal imaging studies confirmed the presence of these masses, which were suspected of being intra-abdominal malignancies (neuroblastoma). The other principal differential diagnosis was extralobar pulmonary sequestration (EPS). Intraoperative findings were consistent with EPS, which was confirmed by histologic examination. We present these three infants, review the literature, and discuss the evaluation and treatment of infradiaphragmatic EPS.     

Non-immune hydrops fetalis and bilateral pulmonary hypoplasia in a newborn infant with extralobar pulmonary sequestration

Extralobar pulmonary sequestration was found in a newborn premature infait that presented with non-immune hydrops fetalis, massive bilateral hydrothorax and polyhydramnios in utero. The baby died of severe respiratory insufficiency 15 h after birth. Postmortem examination revealed distended lymphatic vessels in the sequestered lung tissue probably due to impeded lymph drainage. We suggest that not extralobar pulmonary sequestration itself but a subsequent massive unilateral hydrothorax due to severe obstruction of lymph drainage was the cause of the non-immune hydrops fetalis, pulmonary hypoplasia and polyhydramnios. If these symptoms are diagnosed before delivery, a search for extralobar pulmonary sequestration is indicated.     

Congenital pulmonary extralobar intra-abdominal sequestration

Pulmonary sequestration is a rare congenital anomaly characterized by nonfunctioning embryonic pulmonary tissue that has no communication with the normal tracheobronchial tree. Pulmonary sequestration comprises 0.15% to 6.4% of all congenital pulmonary malformations and is divided into intralobar and extralobar types. The extralobar sequestration is rare, and comprises only 25% of all sequestrations. It is frequently associated with diaphragmatic hernia, eventration and other congenital anomalies. The authors report a case of extralobar retroperitoneal pulmonary sequestration which presented as adrenal tumour. The diagnosis was done by anatomo-pathological examination and no associated malformation was detected.     

儿童肺隔离症的介入治疗

目的用心导管介入方法治疗肺隔离症。方法10例平均年龄(9.9±5.8)岁(1~13岁),临床出现反复呼吸道感染、咯血症状,术前经X线胸片、胸部CT和磁共振基本明确诊断为肺隔离症的儿童,经股动脉插管至主动脉弓降部行造影,了解异常血管的起始、直径、数量及回流的静脉,选择大于血管直径30%~40%的蘑菇伞片或金属弹簧圈在降主动脉起始部行异常血管栓塞。结果介入治疗全部成功,无残余瘘。咯血症状当天消失,原有的肺部感染3~5d内控制;粗大血管堵塞后出现一过性胸痛症状,生命体征稳定,持续2周左右恢复。1~6个月胸片复查,肺部阴影8例完全消退,2例明显缩小仅残留微小淡密度影,且无临床症状。结论肺隔离症介入治疗有效、安全、无手术疤痕,恢复快,可免除开胸肺叶切除手术的损伤。     

Cystic sonographic appearance of extralobar pulmonary sequestration

A cystic fetal chest mass showing spontaneous improvement in utero is described. This mass was shown to be an extralobar pulmonary sequestration with associated cystic adenomatoid malformation type 2.     

Intralobar pulmonary sequestration occurring without alteration of pulmonary parenchyma

Typical changes of lung sequestration, including the scimitar sign, were seen on plain films in the left lower lobe. At aortography vasculature of a pulmonary sequestration was seen located paravertebrally in the left posterobasal segment. The arterial supply arose from a division of the thoracic aorta above the diaphragm; venous drainage was to the pulmonary veins. No pulmonary parenchymal changes were detected in the sequestration after resection, nor was there an extrapleural component of the sequestration.     

Intralobar pulmonary sequestration with congenital cystic adematous malformation and rhabdomyomatous dysplasia

Pulmonary sequestration and congenital cystic adenomatous malformations (CCAM) are well known but still uncommon anomalies of the lung. Extralobar sequestrations are well described in association with CCAM, but fewer intralobar lesions are found with this association. The existence of striated muscle within CCAM is described, and we have evidence of dysplastic changes within the various cellular components occurring, with rhabdomyomatous dysplasia being one of these. A literature review shows no previous evidence of a reported intralobar sequestration associated with CCAM and rhabdomyomatous dysplastic changes.     

Congenital cystic adenomatoid malformation and extralobar sequestration occurring independently in the ipsilateral hemithorax

We report a case of congenital cystic adenomatoid malformation (CCAM) and extralobar pulmonary sequestration (EPS) occurring independently in the ipsilateral hemithorax. A literature search using Medline, Winspirs 2.0, found 14 previously reported cases of CCAM within an EPS. There are no reports of each form of congenital pulmonary abnormality occurring separately in the same patient. Accepted: 17 December 1998     

Pulmonary sequestrations detected by antenatal ultrasound

We present three cases of abdominal and abdomino-thoracic extra-lobar pulmonary sequestrations detected by antenatal ultrasound (US) over a 12-month period. In addition, we briefly refer to a case previously described. A cystic mass was demonstrated in the left upper abdomen of the fetus in all four cases, with two extending into the thorax. The diagnosis was confirmed postnatally by fine-needle biopsy and later excision of the mass. In all four cases the lesion did not interfere with the pregnancy, and only one child demonstrated transient effects of mild pulmonary hypoplasia. Review of the antenatal scans showed that in each case the sequestration did not appear to grow during the pregnancy, with a relative decrease in size. Extra-lobar sequestration should always be considered in the differential diagnosis of an abdominal mass diagnosed on antenatal US, especially if cystic and extending from the abdomen through the diaphragm into the mediastinum. Although abdominal sequestrations are rare in comparison to thoracic lesions at postnatal diagnosis, they represent 21 of the 40 published series of pulmonary sequestrations diagnosed on antenatal US.     

Extralobar pulmonary sequestration presenting with torsion

The current report describes the case of a 13-year-old girl with an extralobar pulmonary sequestration who presented with chest and back pains without evidence of infection. Her symptoms were discovered to be secondary to torsion and infarction of the pulmonary sequestration. Although pulmonary sequestration is not an uncommon differential diagnosis for chest masses, it is rare for it to present in this manner.     

Intralobar pulmonary sequestration diagnosed by MR angiography

A definitive diagnosis of pulmonary sequestration necessitates evidence of a systemic arterial supply to the sequestered lung segment by aortography. We report a case of intralobar pulmonary sequestration in an 11-month-old girl in whom the diagnosis was made by contrast-enhanced magnetic resonance (MR) angiography. Contrast-enhanced MR angiography is the most reliable noninvasive method for the definitive diagnosis of pulmonary sequestration in children.     

Coincidence of congenital cystic adenomatoid malformation and extralobar sequestration of the lung in a newborn

We present a rare case of coincidence of an extralobar sequestration with a congenital cystic adenomatoid malformation of the lung in a newborn. The symptoms, diagnostic features, and therapy are described and the etiology and classification are briefly discussed.