Urosepsis is the most common complication of Purn-Belly Syndrome

Raisa Desti. M. Rizky D.M

General Practitioner of Emergency Room at Sayang Public Hospital of Cianjur
Pediatrician at Sayang Public Hospital of Cianjur

Introduction

Purn-Belly Syndrome or Eagle Abret Syndrome is a congenital anomaly

syndrome which is characterized by 3 things, including abdominal muscle
deficits, bilateral cryptococcal and urinary tract anomalies. This phenomenon is
classified as rare and is usually more common in baby boy, and it is reported to
be 1 in 30.000-50.000 cases of newborns. The cause of Purn Belly-Syndrome is
not clearly identified however it possible that there is a defect during Mesoderm
development. Pulmonary hypolpasia and renal failure plays an vital role in the
mortality rate of baby with Purn Belly Syndrome as much as 60%. On the other
hand, other complications such as urinary tract infections and sepsis can not be
ignored. The treatment for this case depends on the severity of the symptoms
experienced by patients, some cases are medicated conservatively and in some
cases surgery is performed to patch up the structure of the abdominal muscles
and urinary tract.

Case Presentation
A 16 day old baby boy who was referred to Sayang Public Hospital of
Cianjur, moan of wrinkled stomach since birth, and he had high fever for 2 days
and had short pf breath. There was seepage of urine on the right side of the
abdomen, but it was not clearly visible and had a hole and the baby boy was
fussy and also looked painful. The baby boy was born to a 22 year old mother,
spontaneous vaginally with a birth weight of 3100 grams. Shortly after giving an
birth, the baby cried and had immediately an IMD and breast fed. The baby’s

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abdomen was wrinkled and loose, a midwife said that the baby mother did not
routinely check the womb, she had never had an ultrasound scan during
pregnancy and she was not immunized. In her family, there was no baby who has
similar complaints. She claimed that during pregnancy, she was healthy and
nothing was happened. When she arrived in Emergency Room (ER), a physical
examination was completed. The condition of baby boy was alert, vital signs,
weight 3899 grams, HR 190x/m, R 64x/m, S 39, Spo2 100%. The examination of
baby boy head, neck and thorax obtained normal results, for abdominal
examination. The impression of the abdomen was found to appear orange peel,
gentle Palpation with very soft and warm contours, no Palpable organs,
percussion of all the abdominal fields, dull sound, auscultation, bowel bissing 2-
8 times per minuted and examination of the extremities appeared normal, genital
examination showed no testes in the scrotum.
The baby boy had breastfeeding and routine care for 16 days at home,
meanwhile a local midwife suggested a baby mother to go to hospital in order to
obtain intensive care. Her family wanted to discuss it first and when the fever
and shortness of breath attack the baby, her family took him to Emergency Room
(ER) of Sayang Public Hospital of Cianjur. The ER staffs directly direct some
actions, they are injection of intravenous fluids, a parental therapy (supply him
with antibiotics and antiparetics) and routine blood tests, GDS, electrolytes and
urine analysis. Fluids therapy was given D10% drops per micro minute,
antibiotics were given Ampicillin 2x190 mg IV and Gentamicin 1x15.2 mg IV,
and Samol infusion 4x38 mg IV. Lab examination results showed and increase in
Leukocytes, namely 22.000 and platelets 550.000. This condition revealed
Neonatal Sepsis due to uninary tract infection, on the X-ray examination results
are obtain…… A treatment of sepsis and infection were conducted and the
baby’s hemodynamics of baby boy was stable. The next step was he would be
referred to West Java Province type A hospital for intensive treatment.

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Discussion
The clinical condition of the baby boy when he arrived at the ER, had a
sepsis and urinary tract infection. He was suspected an abdominal fistula and
breathless. The urinary tract infection is caused by imperfect formation of the
urinary bladder and ureters, hence bladder emptying contraction are disrupted
resulting in urinary retention. This formation of fistulas can cause fluid’s
extravasation from urinary tract to abdominal cavity. On genital examination, no
testes were found in the strum. This phenomenon is possible caused by urethral
obstruction and prologue urinary bladder distension, which causing testicular
hypoplasia to prostate and kidneys. At the final stage, this process will affect the
growth of abdominal muscle hence there is a great risk for abdominal fistula.
Meanwhile, shortness of breath can be caused by difficulty coughing and in
respiratory tract mucus accumulates and can cause inflammation. The cough
reflex is reduced due to imperfect muscles in the abdominal area.
Even though the etiopathophysiology is not identified for sure, the only
theory for us to learn is the theory of mesoderm developmental arrest that causes
defect in the genitourinary tract, testes and abdominal wall. Examination reveals
the clinical symptoms are classified as severe to this patient. The severity of Purn
Belly Syndrome can be categorized into three groups. Number one, a group with
the highest mortality rate with the cause of death. It is caused by pulmonary
hypoplasia oligohydramnios and the arena of severe renal failure and 33% of
baby were born dead. Number two, the babies survive in neonates but they are
likely to experience recurrent urosepsis. This second group of babies with
extensive uterine dilatation and renal dysplasia. Number three, the babies who
have mild urinary tract dilatation without kidney disorders and kidney damage.
The last group of babies is classified as proper nursing.
This case can be imagined by chest X-ray, abdominal ultrasound and urinary
tract, PIV (Intravenous pyelography), VCU (Voiding cystourethrography) and
MSU (Miksio cystourerography). On the other hand, antenatal screening can be
conduced in this case with fetal ultrasound. The picture will obtained as if the
patients have Purn Belly Syndrome. Vesicourinary or urethral dilatation,
abdominal distention and amniotic oligohydramnions are usually diagnosed at 30

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weeks’ gestation. Those treatments depend on the degree of Purn Belly
Syndrome it self. Number one category, palliative treatment is carried out.
Number two and three category, surgical intervention may be needed. The most
common complications are urinary tract infection and urosepsis. As a doctor who
works on type B health care facilities, the infection treatment is the main choice
as the solution. The prognosis for these cases generally depend on the severity. It
is reported that about 20% f babies with Purn Belly Syndrome die in neonates
and 50% die in the first 2 years of life and 25-30% of babies are alive but they
have kidney problems and the las 70% babies were born died. Also PBS can be
classified into three prognostic groups. The first group comprises 20% of cases;
these infants are typically stillborn or die shortly after birth because of significant
pulmonary hypoplasia and renal dysplasia. Infants in group 2, representing 40%
of cases, have adequate renal function at birth but require intervention; without
intervention, one-third of these patients will die from renal failure or sepsis.
Patients in group 3, making up the remaining 40% of cases, have mild urinary
tract abnormalities and most will survive.

Conclusion
The enforcement of clinical diagnosis is not generally difficult, as long as
during gestation this abnormality can be enforced. The patient prognosis is poor,
the life’s expectation is small considering he is in number three category of Purn
Belly Syndrome. After having some treatments for urinary tract infection and
sepsis, the patient is referred to a type A hospital to be treated by pediatrician and
pediatric surgeon.

Reference