Ultrasound Obstet Gynecol 2002; 19: 250 – 253
The ‘tulip sign’: a sonographic clue for in-utero diagnosis of
Blackwell Science Ltd
severe hypospadias
I. MEIZNER*, R. MASHIACH*, J. SHALEV*, Z. EFRAT* and D. FELDBERG*
*Ultrasound Unit, Women’s Health Center, Rabin Medical Center, Petah-Tikva and Sackler Faculty of Medicine, Tel-Aviv, Israel
KEYWORDS: Hypospadias, Prenatal diagnosis, Ultrasound
ABSTRACT
Objective To describe a unique sonographic sign for pre-
natal detection of severe penoscrotal hypospadias.
Methods Ultrasound findings of hypospadias diagnosed in
the second trimester of pregnancy in seven patients were
compared with postnatal clinical features. All patients were
recruited from routine sonographic examinations performed
for various obstetric indications.
Results In six of the seven cases with hypospadias, a severe
form of peno-scrotal hypospadias was found. In all six cases,
a unique ultrasound feature was observed represented by
extreme ventral angulation of the penis, with or without
chordee, in a form resembling a tulip flower. This ‘tulip’ is
formed by the ventrally bent penis located between the two
scrotal folds. In all six patients, the postnatal pictures of the
newborns’ genitalia corresponded perfectly to the prenatal
sonograms. Associated anomalies occurred in two cases,
with Nager syndrome in one case and mild renal hydroneph-
rosis in the other case.
Conclusions The ‘tulip sign’ is a specific ultrasonic finding of
severe hypospadias. The recognition of this may help to dis-
tinguish between severe hypospadias and other genital
abnormalities (e.g. ambiguous genitalia).
INTRODUCTION
Hypospadias is an anomaly of the male urogenital tract with
an incidence of between 0.2 and 4.1 per 1000 live births1,2.
The term is derived from the Greek language and refers to a
rent (spadon) on the ventrum of the penis. The anomaly
occurs as a result of failure of complete development of the
anterior urethra. The urethra may terminate just proximal to
the glans (glandular hypospadias), at some point along the
penile shaft (penile hypospadias), at the anterior margin of
the scrotum (penoscrotal hypospadias), or in the perineum
(perineal hypospadias). Associated with this defect is severe
Correspondence: Dr I. Meizner, Ultrasound Unit, Women’s Health Center, Rabin Medical Center, Petah-Tikva 49100, Israel
(e-mail: imeizner@clalit.org.il)
Accepted 10-10-01
250
ventral curvature of the penis or chordee which results
from a fibrous band occurring in the projected course of the
urethra. The embryologic defect is a failure of closure of the
urethral groove. In extreme cases, particularly if there is
associated bilateral testicular maldescent, the configuration
of the genitalia may be so ambiguous that an intersex state
results3.
Determination of fetal sex and detection of external geni-
talia anomalies has become an integral part of targeted ultra-
sound examinations. Prenatal diagnosis of various types
of hypospadias has been reported previously4–12; however,
differentiation between severe and mild hypospadias has not
been attempted. We present a specific sonographic indication
of severe hypospadias (penoscrotal), diagnosed in six of
seven patients with hypospadias, which we termed the ‘tulip
sign’.
PATIENTS AND METHODS
The study population comprised patients who were referred
for routine ultrasonic assessment for various medical indica-
tions and in whom ultrasound findings indicative of hypo-
spadias were detected.
These findings included: (i) absence of distal normal
morphology of the penile shaft in the form of a bulbous tip;
(ii) appearance of chordee in the form of ventral incurvation
of the penis; (iii) a small penile shaft with two echogenic lines
corresponding to small lateral folds belonging to the dermal
remains of the prepuce (dorsal hood); and (iv) an anomalous
urinary stream during fetal micturition.
Ultrasound scans were performed either using a trans-
abdominal (2–5-MHz) transducer or a transvaginal (5–
9-MHz) probe (ATL Ultramark 9, HDI, ATL 5000; Advanced
Technology Laboratories, Bothell, WA, USA).
The sonographic picture representing the ‘tulip’ flower
manifests as a ventrally bent penis located between the two
scrotal folds (Figures 1–5).
Ultrasound documentation of patients suspected of having
hypospadias was compared with postnatal clinical findings.
ORIGINAL PAPER
The ‘tulip sign’ Meizner et al.

Figure 1 Severe penoscrotal hypospadias detected at 30 weeks of Figure 2 Postnatal pictures of the newborn described in Figure 1. Notice
gestation. Notice the severely incurved penile shaft (arrow) in a sagittal the incurved penis between the scrotal folds (a) and the extremely low
view. The arrowhead aims at the scrotum (a). The ‘tulip sign’ is evident, location of the meatus opening (b).
marking the incurved penis between the two scrotal folds (b).

RESULTS
During the period between 1 May 1995 and 31 March 2001,
seven cases of the severe form of hypospadias were diagnosed
at the Ultrasound Unit of the Rabin Medical Center. A
description of all cases is presented in Table 1. In six of the
seven cases, the ‘tulip sign’ was easily recognized. In one
case, the diagnosis was made postnatally and with ‘tulip sign’
being observed when the video films were reviewed. In two
cases, associated anomalies were detected: one case of Nager
syndrome and one case with hydronephrosis. In two other
cases, bilateral undescended testis was also present.
Deliveries occurred at term in all seven cases, and all were
normal spontaneous deliveries. The ultrasound findings did
not play any role in the decision making regarding mode and
type of delivery. Figure 3 The ‘tulip sign’ diagnosed at 22 weeks of gestation. Notice the
severely downward deviation of the penile shaft (arrow) surrounded by
the scrotal rims.
DISCUSSION
The cause of hypospadias remains enigmatic. Most likely, the for the placental production of human chorionic gonado-
etiology is polygenic because of the higher familial incidence. tropin (hCG) in phase 3 organogenesis. It is also possible that
The condition is found more commonly in Whites than in a single placenta (as in monozygotic twins) is incapable of
Blacks, and is more common in Italian and Jews13. Interest- meeting the hCG requirements for masculinization of too
ingly, the incidence of hypospadias in monozygotic twins is male fetuses. Conflicting evidence is found in the literature
8.5-fold higher than in singletons14. The explanation for concerning an association between hypospadias and sea-
these findings may be the existence of two fetuses competing sonal variations14,15.

Ultrasound in Obstetrics and Gynecology 251

The ‘tulip sign’ Meizner et al.

Figure 4 Hypospadias diagnosed at 28 weeks of gestation. The incurved

penile shaft is evident with a ‘squashed’ appearance of the penile end due
to excessive preputium (a). The ‘Tulip Sign’ is evident, with the incurved
penis located between the two scrotal folds (b).

Hypospadias is characterized primarily by dystopia of the

meatus, curvature of the penis and radical changes in the skin
covering the penis. As the opening of the urethral defect
develops more proximally, it is associated with a series of
changes determining the morphology of each anomaly. Sev-
eral classifications of hypospadias exist. However, the most
practical is the one proposed by Barcat16 according to meatal
location. This classification is more practical for clinical use:
(i) anterior hypospadias (glandular, coronal, anterior penile);
(ii) middle hypospadias (middle penile); and (iii) posterior
hypospadias (posterior penile, penoscrotal, scrotal, perineal).
The diagnosis of all but the most minor forms of hypo-
spadias is self-evident on examination of newborn males. The
incomplete formation of prepuce with the excess of skin (dor-
sal hood) leads to an immediate recognition of the urethral
defect. Only in the last decade have we been able to make the
diagnosis of hypospadias with prenatal ultrasound. The
sonographic markers for prenatal diagnosis include the
following:
(i) Absence of the normal pointed morphology of the penile
shaft changing into a blunter bulbous tip, also described as a
squashed cone. This correlates with excess dorsal prepuce.
(ii) Various degrees of abnormal curvature of the penis,
labeled chordee. This term implies a strand of connective
Figure 5 Postnatal pictures of the newborn appearing in Figure 4.
Notice the incurved penis and the two scrotal halves when observed
from above (a) and below (b). The resemblance to the ‘Tulip’ flower is
striking. The meatus orifice is marked by the arrow (c).
tissue stretched like a cord between the meatus and glans, giving
rise to bowstringing.
(iii) A small penile shaft; this is not a diagnostic marker but
arouses suspicion of the presence of hypospadias.
(iv) It is possible, in the second trimester, to observe the two
parallel echogenic lines corresponding to lateral folds of the
dermal remains of the prepuce (dorsal hood). Depending on
the location of the meatus and the degree of penile curvature,
these oblique raphes end at the apex of a small skin cone or
peaks of a cowl-shaped monk’s hood. They are sometimes
described as cobra eyes.

252 Ultrasound in Obstetrics and Gynecology

The ‘tulip sign’ Meizner et al.

Table 1 Description of the prenatal and postnatal findings in the patient population

GA at diagnosis Mode and time Postnatal

(weeks) Ultrasound features Associated anomalies (weeks) of delivery findings

28 Extremely small incurved penis None Vaginal (39) Penoscrotal hypospadias

22 Small, caudally directed penis with Undescended testis Vaginal (41) Severe penoscrotal hypospadias
parallel lines
22 Ventral curvature of penis with Undescended testis Vaginal (40) Severe scrotal hypospadias
parallel lines
24 Small lateral preputial fold, Nager syndrome, mandibular facial Vaginal (40) Severe penoscrotal hypospadias,
incurved penis, ventral micturition dysostosis, absent first metacarpal Nager syndrome
30 Ventral curvature of penis None Vaginal (40) Penoscrotal hypospadias
22 Distal lateral folds, bifid scrotum Hydronephrosis Vaginal (42) Penoscrotal hypospadias
25 Abnormal configuration of penis None Vaginal (39) Penoscrotal hypospadias

All seven cases had a 46,XY karyotype. GA, gestational age.

(v) Ventral deflection of urinary stream may occur due to
dystopia of meatus. The stream is not linear but rather
fan-shaped. Color Doppler studies may help demonstrate
the abnormal micturition.
The significance of the ‘tulip sign’ rests on the fact that it
represents the severe curvature of the penis in association
with penoscrotal transposition of a bifid scrotum. Thus, this
sign indicates the most severe form of hypospadias. The
configuration is analogous to the normal female anatomy
with severe curvature of the clitoris that is caudally located
between the labia. Therefore, in certain cases, it is almost
impossible to distinguish sonographically between a normal
female gender anatomy and a male with severe penoscrotal
hypospadias. The ‘tulip sign’ may help to distinguish between
severe penoscrotal hypospadias and other forms of genital
abnormalities, especially some forms of ambiguous genitalia.
The ‘tulip sign’ may also aid in counseling the worried par-
ents. Establishment of a correct prenatal diagnosis will reas-
sure the parents and encourage them to believe in a better
prognosis, since surgical results (cosmetically and function-
ally) are excellent with the use of one-stage hypospadias
repair at an early stage.
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