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Abstract

Abstract. The authors report a case of unexplained fetal in-utero


intraventricular haemorrhage (IVH) with adrenal hemorrhage. The
suspected diagnosis was made at 23 weeks of gestation where
prenatal ultrasonography was suggestive of hydrocephalus with
intraventricular clots and adrenal hemorrhage.

Key words: Fetal ultrasonography - Neonatal ultrasonography -


Intraventricular haemorrhage, adrenal hemorrhage

Case report

23 years old woman Gravida 3, Para 3, Living 1, Intra Uterine Death 1,


Dead 1, Abortion 2 presented at 22 weeks and 6 days of gestation with
complaints of leaking per vaginum for the prior one week. Her
pregnancy was uneventful for the first 2 months, but she complained
of spotting in the 3rd month. The patient gave no history of viral
infections, trauma, or idiopathic thrombocytopenic purpura. There was
no family history of hydrocephalus.

TIFFA scan done at 18 weeks of gestation was normal. On physical


examination, her vitals were as follows: 1) Pulse – 82 bpm, 2) BP –
110/70 mm of hg. Per Abdominal examination revealed uterus to be at
24 weeks and relaxed. A sonogram revealed a single, live intrauterine
pregnancy with cephalic presentation. Fetal movements and cardiac
activity was noted. Placenta was found to be posterior up to the
fundus.
Grade I. Amniotic fluid was found to be less than normal. Ventricular
system was dilated with layered heterogeneous hyper echogenicity
seen in all the ventricles suggestive of an intra-ventricular bleed. No
midline shift was present.
Adrenal bleed to be described
Fetal measurements were as follows:

1) BPD - 65mm corresponding to 26 weeks and 4 days


2) FL - 40mm corresponding to 22 weeks and 6 days
3) HL - 37mm corresponding to 23 weeks and 0 days
4) AC - 232mm corresponding to 27 weeks and 4 days
5) HC - 244mm corresponding to 26 weeks and 4 days

Femur length and humeral length were found to be small in gestational


age when compared to the abdominal circumference and the head
circumference suggestive of short limb dwarfism.
Laboratory investigations done were normal.

Discussion:

Intra-ventricular hemorrhage is the most common type of intracranial


hemorrhage seen in pre-term infants less than 32 weeks of gestation.
These hemorrhages emanate from the small vessels within the sub-
ependymal germinal matrix in infants less than 33 weeks gestational
age [1, 3]. The incidence is known to decrease with increasing
gestational age and is influenced by certain peri-natal risk factors.

Reports of fetal sonography revealing intracranial haemorrhage are


exceptional, because both IVH and a prominent choroid plexus may
show the same echogenicity within the ventricles, and differentiation is
quite difficult [17]. The diagnosis of prenatal IVH is based on the US
appearance of the blood clot; cystic degeneration of the haemorrhagic
area suggests that the haemorrhage occurred days or weeks
previously.

Other cases of hydrocephalus related to prenatal haemorrhage have


been reported recently [5, 6, 11, 17] and prenatal IVH may not be an
uncommon cause of connatal hydrocephalus, even though this has not
been mentioned in extensive surveys [8, 10].

Pathogenesis is related to intra-vascular, vascular or extra vascular


factors. The pathogenesis of hemorrhage is probably related to a
number of factors including the fragility of the premature capillary bed
of the germinal matrix, the disproportionate amount of total cerebral
blood flow to the periventricular area, and the lack of auto regulation
of cerebral blood flow in the premature infant.

GRADING OF IVH (per J. Volpe):

Grade I: Bleeding confined to periventricular area (germinal matrix)

Grade II: Intraventricular bleeding (10-50% of ventricular area on sagittal view)

Grade III: Intraventricular bleeding (>50% of ventricular area or distends ventricle)


144

Intra-parenchymal echodensity (IPE) represents periventricular hemorrhagic


infarction and is often referred to as Grade IV IVH.
Details of Adrenal hemorrhage findings:

Adrenal hemorrhage text from site:

Adrenal hemorrhage is not uncommonly seen in newborn babies. It can occur


prenatally as a result of difficult labour or delivery in infants of diabetic
mother or infants who are large for their gestational age. Other causes which
can be considered are asphyxia, hypoxia, septicemia or hemorrhagic
disorders. Sometimes it can occur in association with renal vein thrombosis.
(2) Occasionally the bleeding may be sufficient enough to form a palpable
mass which can be mistaken for a tumor. Rarely it may rupture into
peritoneum & cause intestinal obstruction or scrotal hematoma. Clinically the
child may present with features of acute blood loss, anemia or jaundice. If
both glands are involved the newborn may be acutely ill with features of
hypoadrenalism. In our patient the cause for fetal adrenal hemorrhage was
not identified as there was no thrombocytopenia or coagulopathy. (3) No
definite causes for fetal adrenal hemorrhage have been reported in literature.
In this case the hemorrhage may have been spontaneous without trauma
and intrauterine complications, because of susceptibility to hemorrhage due
to the size and vascularity of the fetal adrenal gland. (4) ?stress induced
adrenal hemorrhage

Congenital neuroblastoma is an important differential diagnosis. There are no


pathognomonic sonographic features of neonatal hemorrhage but serial
ultrasound examinations can help to differentiate between these two
conditions. A hematoma decreases in size within one to two weeks whereas
neuroblastoma tends to retain its echogenicity and does not change in size.
Urinary catecholamines are elevated in the majority of cases of
neuroblastoma. Colour coded Doppler sonography is useful to differentiate
between congenital neuroblastoma and adrenal haemorrhage. In
neuroblastoma it shows a network of microscopic vessels that invade the
tumour and provide blood supply essential for its growth. This network gives
rise to characteristic high velocity doppler shifts. In contrast to
neuroblastoma, adrenal haemorrhage is characterized by diminished or
absent blood flow. (3) Investigations include imaging in form of ultrasound,
CT scan and MRI. MRI can determine the age of the hematoma. (2)

There are no reports in literature whether adrenal insufficiency is transient or


chronic following neonatal adrenal hemorrhage. In our patient it was
transient and recovery occurred within 4 months. A short ACTH stimulation
test has to be done at regular intervals for monitoring adrenal recovery.

References:
1. Johnson ML, Aurnack CM, Mannes EJ, Appareti KE. Detection of
neonatal intracranial hemorrhage utilizing real-time and static
ultrasound JCU 1981;9:427-433.
3. Volpe JJ. Current concepts in neonatal medicine. N EngI J Med
1981;304:886-890
5. Hill A, Rozdilsky B (1984) Congenital hydrocephalus secondary to
intrauterine germinal matrix]intraventricular haemorrhage. Dev Med
Child NeuroI 26 :524-527
6. Jackson JC, Blumhagen JD (1983) Congenital hydrocephalus due to
prenatal intracranical hemorrhage. Pediatrics 72: 344-346
7. Kim M, Elyaderan MK (1982) Sonographic diagnosis of cerebro-
ventricular hemorrhage in utero. Radiology 142:479-480
8. Larson SL, Banner EA (1966) Hydrocephalus: a 30 year survey.
Obstet Gynecol 28 :571-577
10. Lorber J, Bassi U (1965) The aetiology of neonatal hydrocephalus
(excluding cases with spina bifida) Dev Med Child Neurol 7: 289- 294
11. McGahan JI, Haesslein HC, Meyers M, Ford KB (1984) Sono- graphic
recognition of in utero intraventricular hemorrhage. AJR 142:171-173
17. Zalmeratis EL, Young RSK, Krishnamoorthy KS (1979)Intracra- nial
hemorrhage in utero as a complication of isoimmune throm-
bocytopenia. J Pediatr 95:611-614

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