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256 DSJUOG Review Article DSJUOG Radu Vladareanu et al 10.5005/jp-journals-10009-1473 REVIEW ARTICLE Neck 1Radu Vladareanu, 2Simona Vladareanu, 3Costin Berceanu ABSTRACT craniocaudal succession: 1st arch on day 22nd; 2nd and 1 Cystic hygroma (CH) is the most frequently seen fetal neck mass 3rd arches sequentially on day 24th; 4th arch on day 29th. on the first-trimester ultrasound (US). Overall prognosis is poor with Pharyngeal arches consist of a mesenchymal core – a high association with chromosomal and structural anomalies. mesoderm and neural crest cells – that is covered on When diagnosed prenatally, fetal karyotyping and detailed US the outside with ectoderm and lined on the inside with evaluation should be offered. Prenatal and postnatal surgical or endoderm.1 nonsurgical treatment options are available. Fetal goiter (FG) and fetal thyroid masses are rare fetal conditions and may occur as part Each arch contains a central cartilaginous skeletal of a hypothyroid, hyperthyroid, or euthyroid state. Screening for element, striated muscle rudiments, innervated by an FGs should be carried out in pregnancies of mothers with thyroid arch-specific cranial nerve, and an aortic arch artery.1 disease. If a FG is detected, a detailed US examination should Arterial blood reaches the head via paired vertebral be performed. Congenital high airway obstruction syndrome arteries that form from anastomoses among intersegmen- (CHAOS) is characterized by bilaterally enlarged lungs, flat or inverted diaphragms, dilated tracheobronchial tree, and massive tal arteries and through the common carotid arteries. The ascites. It is usually a lethal abnormality. Fetuses with suspected common carotid arteries branch to form the internal and CHAOS should be referred to a fetal medicine center able to external carotid arteries. The common carotids and the perform ex utero intrapartum treatment (EXIT) delivery. Neck roots of the internal carotids are derived from the 3rd arch teratomas are associated with high mortality rates. Prenatal US arteries, whereas distal portions of the internal carotids diagnosis of cervical teratoma can be made at 15 and 16 weeks of gestation. Planning of delivery in a tertiary center allows the are derived from the cranial extensions of the paired performance of EXIT. Lymphangioma of the neck usually diag- dorsal aortae. The endothelium of the head vasculature nosed in late pregnancy could be traditionally referred to as CH, and aortic arch arteries is derived from the mesoderm.1,2 but there is a different prenatal history and outcome. The external pharyngeal clefts or grooves between Keywords: Congenital high airway obstruction syndrome, the arches remain separated from the apposed, internal Cystic hygroma, Goiter, Lymphangioma, Teratoma. pharyngeal pouches by thin pharyngeal membranes. How to cite this article: Vladareanu R, Vladareanu S, Berceanu C. These membranes are initially two-layered, consisting Neck. Donald School J Ultrasound Obstet Gynecol 2016;10(3): of ectoderm and endoderm, later being infiltrated by 256-270. mesenchymal cells.1,2 Source of support: Nil All of the pharyngeal pouches give rise to adult struc- tures. These are the tubotympanic recess, the palatine None Conflict of interest: tonsils, the inferior parathyroid glands, and the ultimo- branchial body. The parathyroids, thymus primordia, and EMBRYOLOGY AND DEVELOPMENT ultimobranchial bodies separate from the lining of the OF THE NECK STRUCTURES pharynx and migrate to their definitive locations within the neck and thorax.1 Human embryos include four pairs of well-defined The parathyroid glands and the ultimobranchial pharyngeal arches. The pharyngeal arches form in bodies migrate inferiorly to become embedded in the posterior wall of the thyroid gland. The two para thyroids 1Professor, 2,3Lecturer exchange position as they migrate: Parathyroid III 1 becomes the inferior parathyroid, whereas parathyroid Department of Obstetrics and Gynecology, Carol Davila 1-3 University of Medicine and Pharmacy, Elias Emergency IV becomes the superior parathyroid. University Hospital, Bucharest, Romania The thyroid primordium first forms late in the 4th 2Department of Neonatology, Elias Emergency University week and appears as a small, solid mass of endoderm Hospital, Bucharest, Romania proliferating at the apex of the foramen cecum on the developing tongue. The thyroid primordium descends 3Department of Obstetrics and Gynecology, The University of Medicine and Pharmacy, Craiova, Romania through the tissues of the neck at the end of a slender thyroglossal duct. The thyroglossal duct breaks down Corresponding Author: Radu Vladareanu, Professor Department of Obstetrics and Gynecology, Carol Davila by the end of the 5th week, and the isolated thyroid, University of Medicine and Pharmacy, Elias Emergency consisting of lateral lobes connected by a well-defined University Hospital, Bucharest, Romania, Phone: +40722351081 isthmus, continues to descend reaching its final position e-mail: [email protected] just inferior to the cricoid cartilage by the 7th week.1,2 256 DSJUOG Neck Lymphatic channels arise by vasculogenesis and Table 1: Cystic hygroma incidence angiogenesis from venous precursor cells. Lymphangio- Author/study Incidence genesis begins with the formation of bilateral sprouts Arigita M, Bennasar M, Bienvenido 1:285 from the anterior cardinal veins at about day 42 of P. In: Copel J, D’Alton M, Gratacos FASTER trial E, Platt L, Tutschek B, Feltovich H, development. These sprouts eventually form a pair of Odibo A, editors. Obstetric imaging; enlargements, the jugular lymphatic sacs, which will 2012 collect fluid from the lymphatic vessels of the upper Bianchi DW, Crombleholme TM, 1:285 limbs, upper trunk, head, and neck. In the 6th week, four D’Alton ME, Malone FD. Fetology- Unselected pregnancies diagnosis and management of the in the United States additional lymphatic sacs develop to collect lymph from fetal patient. 2nd ed. 2010 First-trimester the trunk and the lower extremities: The retroperitoneal ultrasonography lymphatic sac, cisterna chili, and paired posterior lym- Breathnach FM, Malone FD. In: 1:285 phatic sacs associated with the junctions of the external Rodeck CH, Whittle MJ, editors. Unselected population 1,4,5 Fetal medicine – basic science Aneuploidy confirmed in and internal iliac veins. and clinical practice. 2nd ed. 200983 50% of the cases 40% trisomy 21 NECK ANOMALIES Chen CP, Liu FF, Jan SW, et al. 1:750 199684 Spontaneous abortions Cystic Hygroma (CH) Cystic hygroma is a congenital lymphatic malformation. This hypothesis is supported by the observation It is the most frequently observed fetal neck pathology that reversed or absent ductus venosus flow during 6 on prenatal ultrasound (US). atrial contraction is observed in these fetuses. Eventual Cystic hygroma refers to the finding of marked skin recanalization or formation of collaterals may explain the thickening extending along the entire length of the fetus transient nature of abnormal ductus venosus waveforms at early US examination.7 and nuchal edema.13 Cystic hygroma represents not a tumor, but an anomaly of the lymphatic drainage into the venous Condition, Etiology, and Pathophysiology system, which leads to the accumulation of lymph in the Cystic hygroma is seen frequently in the first trimester, jugular lymphatic sacs of the cervical region.8 affecting about one in 300 pregnancies.7 Based on the presence of septations, it can be classified Cystic hygroma is also frequently associated with 6 into septated or nonseptated. other malformations and chromosomal abnormalities in However, with the improving resolution and the approximately 75% of the cases6 (Table 2). increasing quality of the image offered by advances of US Cystic hygroma has been related to the inherited dis- machines, it has become clear that all nuchal translucen- orders and malformation syndromes in euploid fetuses 9 cies have visible septations. and to drug intake, including alcohol, aminopterin, and trimethadione.6,14 Prevalence, Incidence, and Epidemiology There are studies suggesting that septations predict an increased likelihood of aneuploidies.15 The true incidence of CH remains unknown. It has been On the contrary, some studies have not confirmed reported to be 1:6,000 at birth and 1:750 among sponta- this affirmation.16 neous abortions.6 More recent data from the first- and Cystic hygroma in the first trimester has clearly visible second-trimester evaluation of risk (FASTER) trial showed septations running transversely between the fetal skin a prevalence of 1:285 in first-trimester pregnancies.8 and underlying subcutaneous tissue. It appears that the It is, therefore, likely that in busy clinical practice diagnosis of this finding early in pregnancy represents a performing routine first-trimester US, septated CH will completely different entity than the diagnosis of CH in 7,10,11 be encountered frequently (Table 1). later pregnancy.7 Cystic hygroma is normally caused by aberrant The latter condition is typically an isolated structural development of lymphatic vessels, as a consequence abnormality of lymphatic drainage and appears to have of an abnormal or absent connection with the venous little or no association with chromosomal abnormality or system, leading to lymphatic stasis and enlargement of other malformation.6,7 the jugular sacs.6,12 The anomaly is visible from the first trimester, and Abnormal lymphatic development in the fetal neck it represents the end of the spectrum of the increased could lead to distension of the
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