Persistent cyanosis after birth

Introduction In children with spleen syndrome, there is persistent cyanosis, difficulty in breathing, difficulty in feeding, growth retardation, tachycardia and hypoxia. It looks like a congenital heart disease. Spleen-free syndrome, also known as Ivemark syndrome, without spleen with congenital heart disease syndrome, spleen hypoplasia syndrome, congenital spleen deficiency, such as atrioventricular and visceral transposition syndrome, is characterized by congenital spleen development Incomplete or no spleen, accompanied by cardiac vascular malformation with cavity, pulmonary venous reflux abnormalities, combined with diseases of abnormal chest and abdominal visceral location.

Cause

The cause of this disease is unknown. Pathogenesis: This abnormality occurs in the embryo 4 to 5 weeks. Due to the role of some teratogenic factors, such as the mother's pregnancy virus infection, resulting in a variety of malformations caused by the suspension of embryonic development, mainly due to cardiovascular malformations, some factors in the developmental stage of embryos 24 to 27 weeks At this stage, it is the spleen, the development of the lungs, the development of the pulmonary veins, and the period of the final fixation of the stomach and duodenum, which causes the development to be suspended and constitutes a congenital symptom of various organ deformities. The intrinsic family has reported that many compatriots suffer from this sign, and its genetic pattern is autosomal recessive.

an examination

1. Continuous cyanosis: The patient has persistent cyanosis after birth, difficulty breathing, difficulty feeding, growth retardation, tachycardia and hypoxic symptoms, which resembles cyanotic congenital heart disease.

2. Symptoms and signs of cardiovascular abnormalities: older people may have clubbing (toe, anterior region of the anterior region), and about half of the cases have diffuse systolic murmurs along the left sternal border.

3. No spleen: Abdominal B-ultrasound found that the spleen is extremely small or even absent.

4. Poor immune function: low resistance. Various infections often occur. Severe infections are often the cause of death.

5. Multiple malformations: except for spleen and cardiovascular malformations. There are often abnormalities such as gastrointestinal transposition.

6. Hairpin: The hair strand persists after birth.

7. Digestive system: visceral translocation, bilaterally symmetric liver is transverse liver, spleen is absent or minimal, and splenic artery is absent.

8. Cardiovascular system: the superior vena cava remains on both sides, and the inferior vena cava and descending aorta are parallel.

9. Blood test: The presence of the Holely-Jolly body of the surrounding red blood cells has a definite diagnosis.

Differential diagnosis

When examining the presence or absence of bruising in newborns, it is necessary to correctly distinguish between peripheral cyanosis and central cyanosis, lips and oral mucosa, which is the most reliable and sensitive part of true cyanosis. Significant local cyanosis is still required in neonates to be differentiated from certain skin pigmentation such as viviparous. Newborns often have a common skin pattern that is more pronounced in cold environments and is caused by autoregulation instability of skin vasomotor. Infants exposed to the first exposure can be seen on the head and face due to pressure caused by congestion, edema, local skin and even lips can be blue-violet, should be distinguished from central cyanosis.

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