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Thứ Hai, 23 tháng 7, 2018

Adult Intrathoracic Kidney


Bochdalek’s hernia (posterolateral defect, pleuroperitoneal hernia), firstly described by Bochdalek in 1848 [7], is a congenital posterior lateral diaphragmatic defect that allows abdominal viscera to herniate into the thorax, resulting from failed closure at 8 weeks of gestation of the pleuroperitoneal ducts, primitive communications between the pleural and abdominal cavities [13]. It is more common in infants (90%) with an incidence of 1/2500 live births; however, the literature on Bochdalek hernia in adulthood is rather limited, with approximately 100 cases reported [2814] even if asymptomatic prevalence in the general population may be as high as 0.17% [1015]. It occurs most frequently on the left side with approximately 80% being left-sided and 20% right-sided [16]. This is presumably due to the pleuroperitoneal canal closes earlier on the right side [17], or to narrowing of the right pleuroperitoneal canal by the caudate lobe of the liver [18].
Bilateral Bochdalek’s hernias are rare [1617]. These hernias are usually congenital and may cause severe life-threatening respiratory distress in the first hours or days of life. Herniated organs are frequently the omentum, bowel, spleen, stomach, kidney, and pancreas on the left, and part of the liver on the right. Because of the pulmonary hypoplasia due to the compression of the lungs by the adjacent hernia, these patients are frequently symptomatic at birth.
Although this condition usually presents in the neonatal period with severe respiratory distress, a few cases being asymptomatic until adult life have also been reported in literature and are usually associated with a better outcome [1921].
In childhood, they are often misdiagnosed as pleuritis, pulmonary tuberculosis, or pneumothorax, and this can result in significant morbidity.
In adults, like infants, most occur on the left side (85%), usually causing gastrointestinal symptoms. In contrast to the acute presentation by infants with these hernias, most adults present with more chronic abdominal symptoms [22], such as recurrent pain, vomiting, and postprandial fullness [23]. Chronic dyspnea, pleural effusion, and chest pain are the most common chest symptoms and signs that are present in this condition [8].
Diagnosis requires a high suspicious index and needs to be confirmed with image studies. In adults, Bochdalek’s hernias are diagnosed incidentally but most cases become surgical emergencies when an abdominal organ is strangled [3]. While urgent surgery is frequently needed for the treatment of the symptomatic Bochdalek hernia, the surgical treatment of asymptomatic Bochdalek hernias may be performed days to years later according to the patient’s status. Larger hernias should be operated because of potential complications.
Renal ectopia describes a kidney that is not located in its usual position. Ectopic kidneys are thought to occur in approximately 1 in 1,000 births, but only about 1 in 10 of these are ever diagnosed [6].
Some of these are discovered incidentally, such as when a child or adult is having surgery or an X-ray for a medical condition unrelated to the renal ectopia.
The complex embryological development of the kidneys can lead to renal anomalies, such as renal ectopia. Most ectopic kidneys are found in the lower lumbar or pelvic region secondary to failure to ascend during fetal life [24].
With a prevalence of less than 0.01%, intra-thoracic kidneys represent less than 5% of all renal ectopias with the least frequency of all renal ectopias [46].
Wolfromm [25] reported the first case of clinically diagnosed intra-thoracic kidney in 1940. In 1988, S. M. Donat and P. E. Donat [4] reviewed cases reported in the literature between 1922 and 1986, and found the abnormality to occur more commonly on the left (62%) than on the right side (36%); 2% of the patients had bilateral intra-thoracic kidney. In addition, this anomaly is observed with higher frequency in males (63%) than in females (37%) [26].
Pfister-Goedeke and Burnier [27] classified the thoracic kidneys into 4 groups: thoracic renal ectopia with closed diaphragm, eventration of the diaphragm, diaphragmatic hernia (congenital diaphragmatic defects or acquired hernia such as Bochdalek hernia), and traumatic rupture of the diaphragm with renal ectopia.
The incidence of intra-thoracic kidney with Bochdalek hernia is reported to be less than 0.25% [4], and the relationship between them remains uncertain. The embryological origin is debatable: various authors have proposed that there exists either an abnormality in the pleuroperitoneal membrane fusion or an abnormality in the high migration of the kidney due to delayed mesonephric involution [28].
Intra-thoracic kidney associated with Bochdalek hernia differs from other intra-thoracic renal ectopias as it tends to be mobile and easily reduced from the thorax to the abdominal cavity with other organs. [26] Commensurate herniation of abdominal viscera is common.
In all cases, the kidney is located within the thoracic cavity and not in the pleural space; the renal vasculature and ureter on the affected side typically exit the pleural cavity through the foramen of Bochdalek and are usually significantly longer than those in the normally positioned kidney [29]. Most intra-thoracic kidneys remain asymptomatic and have a benign course [30].
Anatomically, the features of intra-thoracic kidney are rotational anomalies such as the hilus facing posteriorly, long ureter, high origin of the renal vessels, and occasionally medial deviation of the lower pole of the kidney [263132].
In spite of these abnormalities, it is usually fully functional and does not exhibit dysplasia, contralateral hypertrophy, or obstruction of the lower urinary tract [425273335].
Treatment for the ectopic kidney is only necessary if obstruction or vesicoureteral reflux (VUR) is present. There is an increased incidence of ureteropelvic junction obstruction, VUR, and multicystic renal dysplasia in ectopic kidney [629].
If the kidney is not severely damaged by the time the abnormality is discovered, the obstruction can be relieved or the VUR corrected with an operation. However, if the kidney is badly scarred and not working well, removing it may be the best choice [629].
Our patient had an elongated ureter, medially deviated lower pole, and rotational abnormality in which the hilum was posterior. The left intra-thoracic kidney and the left Bochdalek hernia in our patient has been visualized by CT and MR imaging.
Intra-thoracic kidneys are rare clinical entities that pose many diagnostic and management dilemmas for clinicians. The association of a Bochdalek hernia and an intra-thoracic renal ectopia is very rare. It is emphasized that this condition should be considered in the differential diagnosis of a lower intra-thoracic mass. A high index of suspicion can result in early diagnosis and prompt intervention with reduced morbidity and mortality.

Thứ Bảy, 21 tháng 7, 2018

Ectopic Intrathoracic Kidney



ABSTRACT: Ectopic intrathoracic kidney is a rare entity in literature. But ultrasound could reveal easily an intrathoracic mass while chest X-rays evaluate a chest mass with difficulty.

Bệnh nhân : Nữ 61 tuổi ở Bình Dương đến khám  kiểm tra sức khỏe vì 2 tháng trước chị gái chết vì K phổi.
X QUANG PHỔI: thấy tổn thương dạng khối choán chỗ, kích thước : 11x 5cm,
hình bầu dục, giới hạn rõ, ở vùng sau dưới phổi trái.

SIÊU ÂM: Thận lạc chỗ nằm ở cực trên lách, khó xác định trên hoành hay dưới hoành.

CT SCAN: Thấy thận trái lạc chỗ kèm thoát vị hoành trái lên khoang ngực trái.

CT ANGIO:Từ xuất phát ở vị trí bình thường động mạch thận trái đi lên nuôi thận T lạc chỗ. Chức năng bài tiết thận trái bình thường.

Siêu âm Medic Cần thơ phát hiện cấu trúc thận lạc chỗ trên lách
Với CT scan  CT angiocuống thận  vị trí trong lồng ngực được 
xác định   thuyết phục hơn
Đây  ca thận lạc chỗ trong lồng ngực hiếm gặp cần phối hợp các 
phương tiện chẩn đoán hình ảnh để có chẩn đoán đúng.
như X-Quang, CT  siêu âm.KẾT  LUẬN
1/ Ectopic intrathoracic kidney: A case report and literature review: 
Amit Gupta, Ravishankar, Pillenahalli Maheshwarappa, Hemant Jangid ,
Mangi Lal Meena, Hong Kong Journal of  Nephrology (2013) 15, 48e50
 ECTOPIC KIDNEY: Wydawnictwo  Aluna Agnieszka Szmigielska ,
 Aleksandra Księżopolska , Maria Roszkowska-Blaim , 
Michał  Brzewski , Grażyna Krzemień,  Developmental Period
 Medicine, 2015;XIX,2 © IMiD.