4
29
9
renal parenchyma. Serial E, U, Cr showed deteriorating
renal function in both twins and intravenous urogram
normal intrauterine life. Ramasamy et al while studying
11 cases of familial PBS had suggested a sex linked
autosomal pattern of inheritance. It may be tempting to
speculate familial PBS in our patients, but we do not
have sufficient information about the aborted fetuses to
support this speculation.
(
IVU) was therefore considered unsafe. A radionuclide
renogram was conducted and this showed poorly func-
tioning kidneys with bilateral vesico-ureteric reflux in
both twins.
Fig 1: A set of male twins with Prune Belly Syndrome
Although our patients did not die in the neonatal period,
their clinical presentation at birth and the progressive
renal functional deter1i0oration put them in the most se-
vere category of PBS.
There have been few reports of PBS in Nigeria. Okeniyi
3
et al postulated reasons for the rarity and these include:
low awareness, missed diagnosis at maternity centers
and high rate of deliveries outside the health institutions2.
Twinning is said to be associated with PBS. Balaji et al
first reported concordant PBS in a set of monozygotic
twins. To the best of our knowledge, this is the first re-
port of PBS in concordant twins from Nigeria. On the
other hand, familial PBS is rarer with less than 20 cases
reported worldwide. The case reported from UBTH in
Nigeria occurred in a child with a5n affected older sibling
suggesting a familial occurrence.
The discoloration on one of the twins resulted from reflection
of light from the overhead radiant warmer
The limitation of obstetric ultrasonography in the detec-
tion of congenital malformations which may be an indi-
cation of the experience of the ultrasonographer was
amply demonstrated in our patients. Although it is a
significant improvement over previous reports where
babies were only discovered at delivery to be abnormal,
the non-specificity and inadequacy of the ultrasound
reports precluded any form of emergency obstetric inter-
vention (such as bilateral nephrostomy) and appropriate
preparation for neonatal care.
They received intravenous fluids, antibiotics and Nevi-
rapine (Anti Retroviral Viral prophylaxis). Bilateral
loop ureterostomy for urinary diversion was done on the
fifth day of life following which there was some im-
provement in renal function.
Additional treatment included top-up blood transfusions
done on two occasions. The first twin also developed
scrotal swelling due to hypoproteinemia during the sec-
ond week of life that resolved gradually with the intro-
duction of enteral feeds after an initial infusion of albu-
min. Their subsequent hospital stay was essentially un-
eventful. The parents were counseled and the mother
was encouraged to participate in the feeding and general
care of the babies.
The presence of the classical triad of lax abdominal
wall, cryptorchidism and enlarged kidneys at birth
enabled immediate clinical diagnosis. The results of
serial electrolytes, urea and creatinine showed progres-
sive deterioration in renal function with some improve-
ment when ureterostomy was done. Although it was
contraindicated to carry out intravenous urography, we
had the option of radionuclide renal scan which demon-
strated bilateral poor renal function in both babies. It
was therefore possible to prognosticate and appropri-
ately counsel the parents.
Both babies were discharged home at the age of 24 days
to be followed up in the clinic. However, the first twin
died from suspected aspiration of feeds at home three
days after discharge while the second twin died from a
respiratory infection at the age of three months.
Prognosis for patients with PBS usually depends on the
state of the urinary tract at birth as well as the degree of
pulmonary hypoplasia. In those with the severe form of
disease1,0death occurs in utero or in the early neonatal
period. Our patients presented with evidence to sug-
gest abnormal urinary system at birth. While the first
twin died in the late neonatal period, the second twin
died in the immediate post neonatal period.
Discussion
The exact aetiology of PBS is unclear, however postula-
tions on the pathogenesis of this congenital disorder
include prostatic hypoplasia resulting in functional ure-
thral obstru8 ction or a basic defect of the embryonic
1
mesoderm. Although sex-influenced mode of inheri-
tance has also been postulated because of the male pre-
ponderance of the disease, this may be linked with the
theory of prostatic deficiency. Our patients were both
males confirming the predominant occurrence of PBS in
males. The previous history of recurrent spontaneous
first trimester abortions may have resulted from severe
congenital malformations that were not compatible with
The inaccurate prenatal report constituted an ethical
issue and also compounded parental counseling. Addi-
tionally, the long-term benefit of surgical urinary diver-
sion despite apparent poor prognosis was difficult to
justify.