Unilateral Genital Tract Obstruction with Ipsilateral Renal Anomaly Syndrome: Classification, Clinical Manifestations, and Precise Diagnose

Figures & data

Table 1 The Anatomical Combination of the Utero-Cervical-Vaginal Anatomy and the Site of Communication in Different Types of UGTOIRA Syndrome

Type	Vagina	Cervix	Uterine	Communication Site
Type I (vaginal obstruction)	Low oblique vaginal septum	Double cervix	Complete bicorporal uterus	Without communication (Figure 1A)
				With a foramen in the oblique vaginal septum (Figure 1B), or with a fistula connecting the oblique vaginal septum and the contralateral cervix (Figure 1C)
		Septate cervix	Complete bicorporal uterus	Without communication (Figure 1D)
				With a foramen in the oblique vaginal septum (Figure 1E), or with a fistula in the cervix septum (Figure 1F)
			Bicorporal septate uterus	Without communication (Figure 1G)
				With a foramen in the oblique vaginal septum (Figure 1H), or with a fistula in the cervix septum (Figure 1I), or with communication between both internal orifices of the cervix (Figure 1J)
			Complete septate uterus	Without communication (Figure 1K)
				With a foramen in the oblique vaginal septum (Figure 1L), or with a fistula in the cervix septum (Figure 1M), or with communication between both internal orifices of the cervix (Figure 1N)
Type II (cervicovaginal obstruction)	High or middle oblique vaginal septum	Unilateral small cervix	Partial bicorporal uterus	With the communication between the left and right lower portions of the uterine cavity (Figure 2A)
			Bicorporal septate uterus	Without communication (Figure 2B)
				With a fistula in the cervix septum (Figure 2C), or with communication between both internal orifices of the cervix (Figure 2D)
			Partial septate uterus	With the communication between the left and right lower portions of the uterine cavity (Figure 2E)
			Complete septate uterus	Without communication (Figure 2F)
				With a fistula in the cervix septum (Figure 2G), or with communication between both internal orifices of the cervix (Figure 2H)
		Unilateral obliterated cervical orifice	Complete bicorporal uterus	Without communication (Figure 2I)
			Bicorporal septate uterus	Without communication (Figure 2J)
				With a fistula in the cervical septum (Figure 2K), or with communication between both internal orifices of the cervix (Figure 2L)
Type III (cervical obstruction)	A normal vagina	Unilateral obliterated cervical orifice	Bicorporal septate uterus	Without communication (Figure 3A)
				With a fistula in the cervical septum (Figure 3B), or with communication between both internal orifices of the cervix (Figure 3C)
			Complete septate uterus	Without communication (Figure 3D)
				With a fistula in the cervical septum (Figure 3E), or with communication between both internal orifices of the cervix (Figure 3F)
Type IV (unilateral partial cervical aplasia)	A normal vagina	Partial non-development of unilateral cervix with a blind end	Complete bicorporal uterus	Without communication (Figure 4A)
				With a fistula in the site of partial cervical fusion (Figure 4B)
Type V (Unilateral isthmus atresia)	A normal vagina	Completely undeveloped ipsilateral cervix which looks like a normal cervix	Unilateral isthmus atresia	Without communication (Figure 5)

Figure 1 Anatomical illustrations of the utero-cervical-vaginal anatomy in type I UGTOIRA syndrome (vaginal obstruction).Complete bicorporeal uterus, double cervix without cervical obstruction, and low oblique vaginal septum, without communication (A), with a foramen in the oblique vaginal septum (B), with a fistula connecting the oblique vaginal septum and the contralateral cervix (C); complete bicorporeal uterus, septate cervix without cervical obstruction, and low oblique vaginal septum, without communication (D), with a foramen in the oblique vaginal septum (E), with a fistula in the cervix septum (F); partial bicorporeal septate uterus, septate cervix without cervical obstruction, and low oblique vaginal septum, without communication (G), with a foramen in the oblique vaginal septum (H), with a fistula in the cervix septum (I), with communication between both internal orifices of the cervix (J); complete septate uterus, septate cervix without cervical obstruction, and low oblique vaginal septum, without communication (K), with a foramen in the oblique vaginal septum (L), with a fistula in the cervix septum (M), with communication between both internal orifices of the cervix (N).

Figure 2 Anatomical illustrations of the utero-cervical-vaginal anatomy in Type II UGTOIRA syndrome (cervicovaginal obstruction).Partial bicorporeal uterus, septate cervix with unilateral cervical dysplasia, and high oblique vaginal septum, with the communication between the left and right lower portions of the uterine cavity (A); partial bicorporeal septate uterus, septate cervix with unilateral cervical dysplasia, and high oblique vaginal septum, without communication (B), with a fistula in the cervix septum (C), with communication between both internal orifices of the cervix (D); partial septate uterus, septate cervix with unilateral cervical dysplasia, and high oblique vaginal septum, with the communication between the left and right lower portions of the uterine cavity (E); complete septate uterus, septate cervix with unilateral cervical dysplasia, and high oblique vaginal septum, without communication (F), with a fistula in the cervix septum (G), with communication of both internal orifices of cervix (H);complete bicorporeal uterus, double cervix with unilateral obliterated cervical os, and high oblique vaginal septum, without communication (I); partial bicorporeal septate uterus, septate cervix with unilateral obliterated cervical os, and high OVS, without communication (J), with a fistula in the cervical septum (K), with communication of both internal orifices of cervix (L).

Figure 3 Anatomical illustrations of the utero-cervical-vaginal anatomy in Type III UGTOIRA syndrome (cervical obstruction).Partial bicorporeal septate uterus, septate cervix with unilateral obliterated cervical os, with one normal vagina, without communication (A), with a fistula in the cervical septum (B), with communication between both internal orifices of the cervix (C); complete septate uterus, septate cervix with ipsilateral obliterated cervical os, with one normal vagina, without communication (D), with a fistula in the cervical septum (E), with communication between both internal orifices of the cervix (F).

Figure 4 Anatomical illustrations of the utero-cervical-vaginal anatomy in Type IV UGTOIRA syndrome (unilateral partial cervical aplasia). Complete bicorporeal uterus, double cervix with unilateral partial cervical aplasia, with one normal vagina, without communication (A), with partial cervical fusion and a fistula in the cervical fusion (B).

Figure 5 Anatomical illustration of the utero-cervical-vaginal anatomy in Type V UGTOIRA syndrome (Unilateral isthmus atresia). Complete bicorporeal uterus with unilateral isthmus atresia, double cervix with completely undeveloped ipsilateral cervix which looks like a normal cervix, with one normal vagina, without communication.

Table 2 Baseline Characteristics (n=59)

Baseline Characteristics	Total (n=59)		Communication (n=34)		Non-communication (n=25)		P-value
	Range (Min-max)	Median (25%, 75%)	Range (Min-max)	Median (25%, 75%)	Range (Min-max)	Median (25%, 75%)	Distribution	Median
Menarche age (years)	10–14	12 (11,13)	11–14	12 (12,13)	10–14	12 (11,13)	0.449	0.785
Age of onset of symptoms (years)	5–52	14 (12,23)	11–52	22 (14,25)	5–20	12 (11,14)	0.000	0.000
Age at diagnosis (years)	5–52	15 (12,23)	12–52	22 (16,26)	5–20	12 (11,14)	0.000	0.000
Operation age (years)	5–52	15 (12,23)	12–52	22 (16,26)	5–20	12 (11,14)	0.000	0.000
Interval between menarche and onset of symptoms (months)	0–480	24 (3135)	1–480	108 (24,171)	0–84	3 (2,11)	0.000	0.000
Interval between onset of symptoms and diagnosis (months)	0–72	0 (0,3)	0–72	0 (0,7)	0–60	0 (0,3)	0.904	0.800
Interval between menarche and diagnosis (months)	1–480	36 (5135)	2–480	120 (45,171)	1–84	4 (3,18)	0.000	0.000
Interval between diagnosis and operation (months)	0–12	0 (0,0)	0–12	0 (0,0)	0–12	0 (0,0)	0.203	0.364
Interval between onset of symptoms and operation (months)	0–72	0 (0,6)	0–72	0 (0,12)	0–60	0 (0,0)	0.089	0.150
Interval between menarche and operation (months)	1–480	36 (5135)	2–480	125 (45,176)	1–84	4 (3,18)	0.000	0.000

Figure 6 The number and incidence of different complaints in different types and subtypes of UGTOIRA syndrome (n=59).

Figure 7 The number and incidence of different complaints in communication and non-communication groups (n=59).

Table 3 The Numbers and Incidences of Different Types and Subtypes of UGTOIRA Syndrome (n=59)

Type	No. (% of All)	Communication No. (% of Type)	Subtype	No. (% of Type)	Communication No. (% of Subtype)	Uterine Abnormalities (No.)
Type I (V2, C1/C2)	45 (76.3)	29 (64.4)	I-a (low-OVS)	18 (40.0)	9 (50.0)	U3b (9), U3c (4), U2b (5)
			I-b (middle-OVS)	21 (46.7)	16 (76.2)	U3b (16), U3c (2), U2b (3)
			I-c (high-OVS)	6 (13.3)	4 (66.7)	U3b (5), U2a (1)
Type II (V2, C3a/C3b)	7 (11.9)	2 (28.6)	II-a (C3aV2)	5 (71.4)	2 (40.0)	U3b (5)
			II-b (C3bV2)	2 (28.6)	0 (0)	U3b (2)
Type III (V0C3b)	3 (5.1)	2 (66.7)	No subtype			U3b (2), U3c (1)
Type IV (V0C3c)	3 (5.1)	1 (33.3)	No subtype			U3b (3)
Type V (V0C0U4a)	1 (1.7)	0 (0.0)	No subtype			U4a (1)
Total no. (% of all)	59 (100)	34 (57.6)	U3b (42, 72.4), U3c (7,12.1), U2b (8,13.6), U2a (1, 1.7), U4a (1,1.7)

Figure 8 The number and incidence of different communication sites in different types and subtypes of UGTOIRA syndrome (n=59).

Figure 9 The number and incidence of different hematocele sites in different types and subtypes of UGTOIRA syndrome (n=59).

Figure 10 The number and incidence of different complications in different types and subtypes of UGTOIRA syndrome (n=59).