Keywords
spleen - hemorrhage - spontaneous - neonate
Introduction
Splenic ruptures are an uncommon entity that can be catastrophic, with the diagnosis
frequently made at autopsy.[1] An early diagnosis of these injuries is imperative as delays are associated with
an increased mortality.[2] As such, awareness of the condition and a high index of suspicion are required for
prompt diagnosis. Splenic hemorrhage, however, presents variably and can mimic adrenal
hemorrhage with similar clinical features and predisposing factors. Imaging, such
as computed tomography (CT) or ultrasound (USS), therefore, aid in diagnosis with
the presence of a hemoperitoneum being suggestive. We present an unusual case of spontaneous
neonatal splenic rupture.
Case Report
A full term 3.5 kg male infant was born in good condition via normal spontaneous vaginal
delivery. There had been no antenatal concerns. He was discharged home following a
normal baby check and represented on day 2 of life with a swollen, discolored, and
tender left hemiscrotum. He appeared pale, but general and abdominal examination was
normal with no evidence of discoloration or distension. His pulse was 180/min and
capillary refill was < 2 seconds.
He was taken to the theater for a scrotal exploration urgently to rule out testicular
torsion. There was a concern because the baby was pale and his hemoglobin level returned
at 7.1 g/dL while on the table. Groin exploration revealed blood and clot around the
cord structures. Although there was a scrotal hematoma, there was no blood within
the tunica vaginalis and the testis was normal. A diagnosis of adrenal hemorrhage
was presumed based on the presence of blood in the groin outside the processus vaginalis.
The neonate received a blood transfusion and returned to intensive care for continuous
monitoring. USS showed a 6 × 6 cm hematoma in the left suprarenal area which was consistent
with our suspicion of an adrenal bleed. Nonoperative management was attempted, but
the baby deteriorated overnight and required a further 100 mL/kg of blood products.
There was increasing abdominal distension and scrotal swelling. A CT scan revealed
that the adrenal glands were normal and there was no evidence of retroperitoneal bleed.
The blood was intraperitoneal and the spleen appeared displaced ([Fig. 1]). A splenic anomaly resulting in bleeding was suspected. As the baby was not stabilizing
despite maximal resuscitation (40 mL/kg of blood, cold peripherally and with a capillary
refill time of 3 seconds), we proceeded immediately to laparotomy (24 hours after
admission). The abdomen was full of clot. The spleen was devoid of capsule on its
lateral aspect and this was oozing blood. Splenic preservation was attempted unsuccessfully
(using Surgicel [Ethicon, a Johnson & Johnson Company, Somerville, New Jersey, United
States] and Floseal [Baxter Healthcare Corporation, Hayward, California, United States]),
and splenectomy was required.
Fig. 1 Coronal CT of abdomen showing massive pneumoperitoneum.
The patient had an uneventful recovery and was discharged on postoperative day 4.
A thorough set of investigations were performed (coagulation profile, skeletal survey,
direct Coombs test, and infection profile) and these were normal.
Discussion
Intra-abdominal bleeding in the newborn is uncommon. The presentation can be variable
and can occur as a consequence of several etiologies. Some of the differentials entertained
in this case included hemorrhagic disease of the newborn, sepsis with disseminated
intravascular coagulation, perforated necrotizing enterocolitis, as well as a solid
organ injury. This included adrenal injury, liver injury as well as a splenic injury.
Splenic injury is the least common of these conditions, with less than 50 case reports
in the world's literature.[3] Various etiologies have been reported as a cause of splenic bleeds, with trauma
being the most common. This includes maternal trauma with precipitate delivery[4]
[5] as well as birth trauma secondary to difficult delivery.[3]
[6] There are, however, a growing number of reports of spontaneous and idiopathic splenic
bleeding after a normal delivery,[7]
[8] the likely mechanism in this case. The pathogenesis of this condition is not fully
understood, although the mechanism may be related to an increased intrathoracic pressure
that forced the liver and spleen out of the diaphragmatic hollow.[9] This subsequently causes severe tension on the supporting ligaments, with splenic
rupture occurring in two stages following this precipitating event. The first stage
is usually the formation of a subcapsular hematoma; this is followed by a sudden deterioration
as the capsule eventually ruptures. This staged progression explains the delay in
presentation, with most cases presenting beyond 10 hours of life, with our case presenting
at 48 hours of life.
True spontaneous rupture of the spleen was first described by Peskin and Orloff in
1958.[10] There are four criteria:
-
No history of trauma
-
No evidence of disease
-
No evidence of perisplenic adhesions or scarring of the spleen
-
The spleen should be normal on gross and histological examination apart from the findings
of hemorrhage and rupture
A fifth criterion was added in 1991 by Crate and Payne saying that there should be
no evidence of recent viral infection (infectious mononucleosis, cytomegalovirus,
typhoid, and HIV).[11]
Our case is interesting as it presented initially with isolated scrotal findings as
previously reported by Perdomo et al.[12] The scrotum can act as a window for intra-abdominal pathology, however, in this
case the hematoma appeared to have tracked via the retroperitoneum. We do not have
an explanation for this except to conjecture that the blood tracked along the gonadal
vessels via the splenorenal ligament. In other cases reporting scrotal hematoceles
the blood tracked down through open internal rings into the inguinal canal.[13]
[14]
[15] Both internal rings in our patient were observed to be closed at laparotomy.
This case also illustrates how vital it is to obtain a prompt diagnosis—first as it
is a life-threatening event and also to try and salvage the spleen, in turn not rendering
the child to a lifetime infection risk. In this case, the diagnosis was delayed because
the presentation was consistent with adrenal hemorrhage and the surgeon was not aware
of neonatal splenic rupture as an entity and differential diagnosis. If cross-sectional
imaging had been instituted earlier, there may have been less of a diagnostic dilemma.
A nonoperative approach with expectant monitoring may still have been the preferred
initial management.
