Cantrell ji pentad: akpata, ọgwụgwọ, prognosis

E nwere ọtụtụ nke malformations guzobere utero. Ha pụrụ imetụta ọ bụla ngwa usoro. The kasị dị ize ndụ na-obi ntụpọ, akpa ume, digestive usoro, akụrụ, na ụbụrụ. Ụfọdụ n'ime ha bụ na-emegide ndụ. Ọzọ amenable ka ịwa ahụ ọgwụgwọ. Otu n'ime ihe ndị kasị dị mgbagwoju malformations atụle pentalogy Cantrell. Nke a na ọrịa e ji a ọnụ ọgụgụ nke imebi nke ọtụtụ akụkụ. Mgbe ụfọdụ, i nwere ike ịhụ na-ezughị ezu pentad Cantrell. N'agbanyeghị ogo ntụpọ, mgbe ụfọdụ, ndị dọkịta na-enwe ike ịnagide ọrịa ma weghachite dị mkpa ọrụ nke ahu.

Cantrell ji pentad - ihe bụ nke a daa ọrịa?

Nke a daa ọrịa na-metụtara congenital ọrịa obi, na ndị ọzọ na akụkụ. Dị ka aha pentad Cantrell agụnye 5 ntụpọ. Otu n'ime ha bụ kasị dị ize ndụ mebiri nke obi usoro, nke gụnyere a mgbanwe hemodynamics. Ọzọkwa a nkwarụ na-agụnye egwu ịchọ mma mma. Mara na o nwere ike-ozugbo na nwa nwa, ka obi nke nwa ọhụrụ "ruo si". Cantrell si syndrome ọ bụghị mgbe nile iso nke 5 ntụpọ. Ọtụtụ mgbe na 1 ma ọ bụ 2 ọrịa na-anọghị (tetrad chi atọ n'ime). Ke adianade ọrịa obi, ọrịa na-agụnye ọzọ arọ mgbaàmà - omphalocele. Ọzọkwa enwere mgbanwe na ihu abdominal na obi mgbidi, diaphragm nkwarụ.

Cantrell ji pentad: akpata ọrịa

Ọ dị mkpa ịmara na ọrịa na-kpụrụ nke abụọ trimester nke afọ ime. N'oge a, na-ebikwasị niile dị oké mkpa akụkụ na usoro. Dị ka ọ na-mara, maka 14-18 izu nke afọ ime kpụrụ mesodamụ. N'okpuru nduzi nke gburugburu ebe obibi ihe dị iche iche na-efe efe na nne ụfọdụ imebi nke embryogenesis nwere ike ime mgbe oge a. Otu n'ime ndị a abnormalities nwere ike ịbụ pentalogy Cantrell. The kpọmkwem akpata ọrịa na a ike. Otú ọ dị, nke a ntụpọ jikọtara chromosomal abnormalities. Otu n'ime ha, 18 na trisomy 13, Turner syndrome, X-jikọrọ ọrịa. Ọ maara na a nkwarụ bụ nnọọ obere. The ugboro ole nke ya omume bụ 3-5 ụmụ ọhụrụ kwa nde. Na ụwa nke na-erughị 100 ikpe kọwara. The ọrịa ọzọ na-ahụkarị ụmụ nwoke.

N'agbanyeghị eziokwu na iji chọpụta ihe kpatara nke ọrịa e mebeghị, mkpa ka ị mara banyere ihe ize ndụ ihe ndị na-eduga dị iche iche ọrịa nke nwa ebu n'afọ na mmepe. N'etiti ha:

1. Mmetụta nke na-emerụ na nsi (salts nke arọ ọla, ọgwụ ọjọọ, na ọgwụ).
2. Radieshon ikpughe.
3. Physical metụtara (mmerụ).
4. -Akpata nrụgide.
5. Ise siga na-aba n'anya.

Usoro nke hemodynamic ọrịa

N'ihi na eziokwu na pentad Cantrell nwere ike na-enwe dị iche iche obi ntụpọ, hemodynamic disturbances otú si dị iche na onye ọ bụla ọzọ. Ọtụtụ mgbe a na ọrịa na-esonyere ventricular septal nkwarụ. Mmepe nke hemodynamic ọrịa na-adabere na osote. Na ịrịba ntụpọ ime na nnukwu shunt obere gburugburu, si otú na-eduga na akpa ume ọbara mgbali. Ekpe ventricle na-ṅụbigara na compensatory hypertrophy. N'ihi ya, e nwere onye na-abawanye ma RV na LV.

Naa nkịtị atrial septal nkwarụ. Hemodynamic disturbances mgbe ọ na-adabere na size nke ntụpọ. Na a nnukwu nkwurịta okwu n'etiti ndị atria orùrù-ọbara n'ime nri iche na-budata ụba, dapụtara na hypertrophy na dilatation nke eyi. The ekpe atrial bufee emee.

Isi ngosipụta nke Cantrell ji pentad

Dị ka a maara, ndị oge gboo Cantrell ọrịa na-agụnye 5 ntụpọ. N'etiti ha bụ ndị na-esonụ imebi:

• Omphalocele nwa ebu n'afọ. Ụzọ ọzọ a na-akpọ elu-alọ abdominal mgbidi nkwarụ. Nke a mebiri e ji eziokwu na esịtidem akụkụ-ejikarị dị na afo, na-anakọtara na alọ hainia akpa na-aga ná mpụga.
• Obi mgbidi nkwarụ. Ọ na-akacha ji enweghị ma ọ bụ a gbawara n'etiti xiphoid. N'ihi ya, obi na-aga n'ihu.
• Enweghị nke pericardium, nke bụ na-n'akụkụ aka diaphragm.
• Gbasara obi anomalies development. Na fọrọ nke nta ka ihe nile ikpe na ectopia - ekwesịghị ịdị na a pụrụ isi chọta gị n'ụlọnga. Mgbe a na obi ntụpọ nwere ike ịbụ na abdominal ma ọ bụ n'olu. Ke adianade ectopia, na e nwere ndị dị iche iche ntụpọ. Na mgbakwunye na VSD na ASD emee atresia nke tricuspid valvụ, na akpa ume akwara. Na obere okwu, ndị zuru ezu enweghị obi-ulo.
• Diaphragmatic hainia.

Ke adianade Cantrell ọrịa nwere ike ịbụ na-ezughị ezu, ọ na-ahụ kwukwara na Nchikota ndị ọzọ anomalies. Ọtụtụ mgbe, a na ọrịa nke CNS (ụbụrụ hainia, hydrocephalus), eriri afọ (daa ọrịa nke ńsí eriri afo na splin), na-anọghị nke mkpịsị aka ma ọ bụ ụkwụ.

The adakarị picture nke ọrịa

The mgbaàmà nke ọrịa apụta ìhè si oge ọmụmụ. The mbụ na-eme ka ya onwe ya mara ịrịba ịchọ mma nkwarụ nke obi na abdominal akụkụ "riputasi" n'èzí. Ke adianade do, ke akpa awa nke ndụ ma sonyere ndị ọzọ egwu mgbaàmà. N'ihi na nke congenital obi ntụpọ mụrụ ọhụrụ na-akawanye obere oxygen. N'ihi ya, o nwere iku ume ọkụ ọkụ, cyanosis, indrawing nke intercostal oghere na ala akụkụ nke sternum. Ọtụtụ mgbe, ndị a ụmụ ọhụrụ ga-ejikọrịrị a ventilator. Chee ọrịa, ọ dị mkpa iji chọpụta ebe obi iji gosi na otu n'ime ndị isi atụmatụ - ectopia ahu.

Gịnị bụ nchoputa nke Cantrell ji pentad?

Cantrell si syndrome nchoputa dabeere isi na mpụga ule nke nwa amụrụ ọhụrụ. First, dọkịta anya n'ihi na ọnụnọ nke omphalocele - a ntụpọ nke abdominal na thoracic mgbidi, na mgbe ahụ na-ekpebi ebe obi. N'elu nkwenye nke ectopic, aghara nke akụkụ okuku ume na hemodynamic kwa, nchoputa bụ fọrọ ụfọdụ. Iji chọpụta na ọnụnọ nke obi ntụpọ, rụrụ auscultation na echocardioscopy. Ultrasound nke afo omume impractical n'ihi mbụ niile ntụpọ ga-tutu amama.

Ọgwụgwọ pentad Cantrell

N'agbanyeghị eziokwu na ndị pentad Cantrell - Nke a na-achọkwa na egwu osote, mgbe ụfọdụ ọ bụ omume na-esi die ya. Nke a na-emetụta ọrịa na hemodynamic abnormalities dakọtara na ndụ. The isi mmerụ ọgwụgwọ - a ịwa ahụ aka. The ọrụ mejupụtara ọtụtụ nkebi nke na a na-ewere otu n'ime toughest. The mbepụ na-rụrụ ke midline thoracic na abdominal mgbidi. N'otu oge ahụ, dọkịta na-awa kwesịrị ime mgbazi nke obi ntụpọ na Mbelata nke digestive usoro. Ectopia nke obi na-emekarị hapụrụ. Mgbe ụfọdụ, ahụ n'ihi obere VSD na ASD dina a ọzọ kwesịrị ekwesị afo (3 afọ).

Prevention malformation

Isi usoro nke mgbochi dị ka prenatal nchoputa. Abụkarị chọpụtara pentalogy Cantrell na nwa ebu n'afọ nwere ike napụta ugbua na 20-24 izu nke development. Ọtụtụ mgbe, ndị dị otú ahụ a ọgwụgwụ bụ ihe mere ndị nchupu nke ime. N'ihi na isi mgbochi na-agụnye:

• Iji zere mmetụta ọjọọ.
• Ịnọgide na-enwe a ike ndụ.
• Zere na-akpata nchekasị, karịsịa ke akpa ọkara nke afọ ime.

The prognosis nke ọrịa a bụ ambiguous, dị ka ọ na-adabere na ogo mmebi nke obi. Mgbe ụfọdụ, ndị dọkịta jikwaa ịzọpụta a ndụ nwa. O sina dị, ndị a ụmụaka mkpa mgbe nile na-elekọta ma na-elekọta nke ọkachamara.