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What should I do if I have the extra "lungs"?
Written by | Skinny Egg Lean Week
Source | "Medical Community" Pediatric Channel
The lungs of the human body are divided into two sides, the left oblique fissure is divided into 2 lobes, and the right side is divided into 3 lobes, which bear the heavy responsibility of breathing. If "one lung" is longer, is the person's breathing ability stronger?
Recently, the Children's Hospital Affiliated to Zhejiang University School of Medicine received such a child: according to the child's mother, the child was suspected of having "isolated lungs" during the obstetric examination, and the child's physical fitness after birth was good, but it was easier to catch a cold than the same age, so follow-up treatment has not been taken. When the child is 6 years old, in order not to affect the child's future life, the mother takes the child to the hospital for treatment.
After examination, the child was diagnosed with lobular lung isolation and was surgically removed.
Pulmonary isolation is a rare congenital broncho-pulmonary dysplasia that accounts for 0.15% to 6.45% of all lung malformations[1], and is divided into intra-in-lobe and extra-lobed types according to the presence or absence of a complete pleura on the surface of the abnormal lung tissue and normal lung tissue. The extra lungs are actually cystic lesions, which not only do not strengthen the ability to breathe, but do not have any respiratory function.
In order to understand the key points of diagnosis and treatment of lung isolation disorder, the "Pediatric Channel of the Medical Community" talked to Tan Zheng, deputy director of the Department of Thoracic Surgery of the Children's Hospital Affiliated to Zhejiang University School of Medicine, let us take a look at it together, what should be done with this extra "lung"?
1
The symptoms of this disease are not significant, and it is easy to be confused by the eyes
Should the symptoms of pulmonary isolation disorders be dominated by lung symptoms? Deputy Director Tan Zheng introduced that the clinical symptoms of intra-lobe and extra-lobed lung isolation disorders are also very different due to the different locations of the isolated lungs [2].
Leaf appearance - "I am asymptomatic, you always don't recognize me." ”
Lobular pulmonary isolation is often asymptomatic or mild because the isolated lungs are separated from the normal lungs and do not communicate with the bronchi. Moreover, cystic lesions of the leaf type are mostly located between the lower left lobe and the diaphragm, or in the subdiaphragm or mediastinum, and are easily misdiasected as posterior mediastinal tumors and diaphragm tumors.
Once significant symptoms of this type occur, they are mostly caused by vascular torsion. The leaf profile provides an anatomical basis for torsion, and if the blood vessels are twisted, it can lead to isolation of pulmonary ischemia and infarction[3], and the clinical manifestations are mainly abdominal pain, and a small number of patients have dyspnea, chest pain, chest tightness, and vomiting.
Ye Nei type - "I have symptoms, but you still easily mistake me for someone else." ”
Cystic lesions of intra-lobular lung isolation can be directly or partially communicated with the bronchi, resulting in the isolation of the lungs prone to repeated infection, clinical symptoms are common, but non-specific, mostly fever, cough, chest pain, cough and even purulent blood sputum and repeated pneumonia attacks, etc., easy to misdiagnosis as lung tumors, lung abscesses and bronchiectasis.
Professor Tan Zheng mentioned: "Due to the isolation of the lung and the normal lung, the arterial blood supply is abundant, the probability of infection is very high, and it will also show some bleeding symptoms. We have had recurrent hemoptysis in 10-year-olds, which turned out to be intra-lobular isolated lungs. ”
The lack of specific manifestations of clinical manifestations, physical examination, and routine laboratory tests of pulmonary isolation disorders poses great challenges to the clinical diagnosis. This reminds clinicians to consider lung isolation disorder and make further diagnosis after excluding diseases such as pulmonary cysts, bronchiectasis, pneumonia, tuberculosis, lung tumors, and diaphragm hernias in children with no obvious precipitating recurrent episodes of focal lung infection and intermittent hemoptysis.
2
Can't distinguish it, how to use auxiliary inspection to refine the "golden eye of fire"?
According to clinical observations and literature reports, abnormal blood supply arteries are more common in the lower thoracic aorta, followed by the abdominal aorta and intercostal arteries [4]. Therefore, the discovery of abnormal blood supply is the key point in diagnosing the disease.
Chest x-ray: mainly used for initial screening. Abnormal shadows of the lungs and inflammatory manifestations of the lungs may be found, but they are not specific and are not preferred, and chest CT is supplemented to distinguish the types of pulmonary isolation disorders.
Chest CT: Chest spiral CT augmentation scan or CT 3D reconstructive vascular imaging may be preferred. Imaging is manifested as two or more manifestations, including solid mass shadow, cystic shadow, patch shadow, and cystic solid shadow [5]. In addition, CT can also determine the location, size, number of abnormal blood supply arteries and the relationship with the aorta.
Preoperative CT manifestations of lobular pulmonary isolation: A. "teardrop-like" lesions in the left thoracic cavity; B. Leaf-shaped isolated lung-like "crater-like" signs with clear margins at the edges of lesions; C. Isolate abnormal blood supply arteries originating from the abdominal aorta[3]
CT manifestations of intra-lobular isolation lung enhancement: A. Enhanced lesions in the lower lobe of the right lung; B. There is a aortic branch for blood supply, and the diameter of the blood tubes 3. 4 mm; C. Aortic vascular reconstruction, showing abnormal branches derived from the aorta[6]
Magnetic resonance imaging (MRI): can detect clearly defined masses in the chest and their internal structures, as well as the source, walking, and venous return of abnormal arteries.
Ultrasonography: If you suffer from pulmonary isolation disorder in the fetal period, the fetal lung tissue will be dysplasia, and there will be pleural effusion, mediastinal displacement, etc., and the probability of intrauterine death of the fetus is high. Therefore, early diagnosis has important clinical significance for the disease. Ultrasonography is the test of choice for diagnosing fetal pulmonary isolation disorder due to its ease of operation, reproducibility, and absence of radiation damage.
When the fetus presents with pulmonary isolation, ultrasound can see strong or slightly strong echoes of round or oval shapes with clear boundaries or slightly strong echo clumps, tracing for cystic areas of different sizes in the blood vessels, and small light point reflexes scattered in the presence of infection [7].
Ultrasound images of fetal pulmonary isolation at 16 weeks of gestation[7]
Deputy Director Tan Zheng reminded that if the fetus is suspected to have pulmonary isolation disease during the obstetric examination, then after the fetus is born, it can be transferred to the children's hospital for diagnosis.
3
Excess "lungs" are treated with surgical resection as soon as possible
For children who have developed lung infection and are treated, conservative treatment may still have the possibility of repeated secondary infections, and a few may become cancerous, so surgical resection should be considered once the lung isolation disorder is confirmed. Compared with traditional open chest, thoracoscopy has the advantages of less trauma, lighter postoperative pain, and faster recovery, and has become the mainstream treatment method.
Deputy Director Tan Zheng mentioned that for the leaf type and the intra-leaf type, the difficulty of surgical resection is also different, the intra-leaf type is mainly the resection of the lobe of the lung, or the resection of a lung segment, which is basically similar to the similar surgical method of adult lung cancer. At the same time, attention should also be paid to the treatment of blood vessels, which are one more step than normal lobectomy and lung segment resection, that is, the blood vessels that deal with systemic circulation. The leaf form is relatively simple to manage due to the separate vascular supply, including the treatment of circulating vessels and veins of return. Some lobular isolated lungs will grow in the diaphragm or even in the abdominal cavity, and this condition first requires finding the isolated lungs.
"We generally recommend that children within the age of 4 have resection surgery as soon as possible, at this time the child's lungs still have regenerative function, and the growth and function of the child's normal lung will not be affected after the operation." The current technology for lung tissue lesions can be performed day surgery, that is, 24-hour hospitalization, the entire operation process is very traumatic, to help the child recover as soon as possible after the operation, and grow up as healthy as a normal child. Deputy Director Tan Zheng said.
Expert Profiles
Tan Zheng
Deputy Chief Physician
Deputy Director of the Department of Pediatric Thoracic Surgery and Deputy Director of the Surgical Endoscopy Center of children's hospital affiliated to Zhejiang University School of Medicine
Member of the Thoracic Surgery Group of the Pediatric Surgery Branch of the Chinese Medical Association
Standing Member of the Pediatric Thoracic Minimally Invasive Professional Committee of the Maternal and Child Health Care Association
He is a national member of the Maternal and Child Minimally Invasive Professional Committee of the China Maternal and Child Health Care Association
Member of pediatric tumor professional committee of Zhejiang Anti-Cancer Association
Member of Thoracic Surgeons Branch of Zhejiang Medical Doctor Association
Member of Pediatric Surgery Branch of Zhejiang Medical Doctor Association
Youth Member of Pediatric Surgery Branch of Zhejiang Medical Association
Undertake and participate in 10 provincial and ministerial projects. He has participated in the compilation of 10 books and textbooks on pediatric thoracic surgery, published more than 20 academic papers, and included 2 papers in SCI. The first in China to obtain the qualification certificate of the main surgeon of the four-generation Da Vinci robot pediatric surgery. Pediatric surgeon who has achieved the milestone of 100 robot surgeries in the shortest time in China. The first pediatric thoracic surgeon in the world to receive the 100 robotics milestone.
bibliography:
Chen Gang,Chen Zhanggen,Jia Bing,Li Zhong. Clinical analysis of 22 cases of pulmonary isolation disorder in children[J].Fudan Journal (Medical Edition),2006,33(5):683-685.
Yang Jingjing,Li Yumei. Clinical analysis of 16 cases of pulmonary isolation[J].China Health and Nutrition,2021,31(33):29-30.
Li Xiao, Cai Chun, Sun Yuling, Du Mengwei, Zhang Gang, Yan Xiangang, Zhou Xiaotong, Yu Gang. Single-hole thoracoscopic technique for the treatment of leafy outer lung isolation in infants and young children[J].Chinese Journal of Minimally Invasive Surgery,2021,21(10):923-926.
Zhu Yufeng,Bai Yuan,Li Hu,Xue Wang,Qin Yongwen. Clinical study on imaging diagnosis and interventional therapy of lung isolation disorder[J].South China Journal of National Defense Medicine,2018,32(3):161-164,172..
[5] Tashtoush B，Memarpour R，Gonzalez J，et al. Pulmonary sequestration：
A 29 patient case series and review［J］.Journal of Clinical ＆Diagnostic Research Jcdr，2015，9（12）：AC05.
[6] Su Yun,Wu Yurui,Zhang Xiaolun,Liu Jing,Li Zexi. Endoscopic treatment of pediatric pulmonary isolation in 65 cases[J].Chinese Journal of Minimally Invasive Surgery,2021,21(10):899-903.
[7] XIONG Jing,CHEN Qian,CHEN Huayan,CHEN Lin. Analysis of the clinical application value of ultrasound and magnetic resonance examination on fetal lung isolation[J].Chinese Journal of CT and MRI,2021,19(6):77-78,102.
Source: Pediatrics Channel of the Medical Professions
Editor-in-charge: Ling Jun
Proofreader: Zang Hengjia
Plate making: Xue Jiao