832639

brief-report2019
CPJXXX10.1177/0009922819832639Clinical PediatricsMiller and Seske

Brief Report
Clinical Pediatrics

Severe Anemia in the Newborn Nursery 1­–4

© The Author(s) 2019
Article reuse guidelines:
sagepub.com/journals-permissions
DOI: 10.1177/0009922819832639
https://doi.org/10.1177/0009922819832639
journals.sagepub.com/home/cpj

Jennifer J. Miller, MD1 and Laura M. Seske, MD, MS1

Introduction Table 1. CBC at Presentation.

Splenic rupture in the newborn period is a rare and WBC 19.34 × 103/mm3 ↑
unusual event that can be life-threatening if unrecog- RBC 1.16 × 106/mm3 ↓
nized. The underlying cause for splenic injury has mul- Hemoglobin 4.1 g/dL ↓
tiple etiologies, and most neonates present many hours Hematocrit 11.8% ↓
Mean corpuscular volume 101.7 fL
after delivery. The subsequent hospital course is unpre-
Mean corpuscular Hgb 35.3 pg
dictable, and many infants will require surgical interven-
Mean corpuscular Hgb concentration 34.7 g/dL
tion and possible splenectomy. In this article, we describe
RBC distribution width 18.0%
a case that despite a critically low hemoglobin did not
Platelet count 153 × 103/mm3
require surgical intervention, and we provide a review of Nucleated RBC number 0.92 × 103/mm3
cases reported in the literature from the past 15 years to
highlight the variable clinical stability of these patients Abbreviations: CBC, complete blood count; WBC, white blood
and evolving surgical trends in management. cells; RBC, red blood cells; Hgb, hemoglobin.

Case At this time, she was transferred to the neonatal

A female infant was born at 39 5/7 weeks gestation by
spontaneous vaginal delivery to a 29-year-old G3P2002.
Maternal history is notable for hemoglobin S trait and
anemia, and medications during pregnancy included
prenatal vitamins and iron. Maternal serologies were
negative, her blood type is B positive, and antibody
screen was negative. The pregnancy was complicated
by fetal tachycardia noted at her 29-week gestation
visit. After an extended evaluation, she had normal
fetal monitoring and a reactive nonstress test. At term,
the mother presented to labor and delivery in spontane-
ous labor, and there was rupture of membranes for
meconium-stained fluids 1 hour prior to delivery. The
infant received delayed cord clamping for 60 seconds,
and APGAR scores were 8 and 9 at 1 and 5 minutes,
respectively. The infant was transferred to couplet care
without incident until 40 hours of life when she was
noted to have general pallor and a new heart murmur. A
complete blood count (CBC) and echocardiogram were
obtained, and the capillary CBC was notable for a
hemoglobin of 4.1 g/dL (see Table 1 for CBC) and
echocardiogram notable for an atrial septal defect with
left to right shunting and by septal position, the right
ventricular pressure was greater than or equal to half
systemic.
intensive care unit and the low hemoglobin was con-
firmed on a free-flowing sample. The infant had normal
vital signs for age, and physical examination was nota-
ble for a quiet, alert female infant in no acute distress
with general pallor, a soft systolic murmur II/VI at the
left sternal border, normal perfusion, and no abnormal
rashes. To evaluate for a bleeding source, a head ultra-
sound and abdominal ultrasound were obtained. Blood
and urine cultures were collected, and ampicillin and
cefotaxime started. A transfusion of 10 mL/kg of packed
red blood cells (RBCs) was given over 4 hours. The eti-
ology of her anemia was unclear, but initial laboratory
work was concerning for an acute bleed due to evidence
of hypovolemic shock with acute kidney injury (blood
urea nitrogen = 25 mg/dL, creatinine of 1.4 mg/dL) and
elevated liver enzymes (aspartate aminotransferase =
159 U/L and alanine transaminase = 288 U/L). A com-
prehensive family history was taken, and the father denied
any history of bleeding disorders, RBC abnormalities
1
Johns Hopkins Hospital, Baltimore, MD, USA
Corresponding Author:
Jennifer J. Miller, Johns Hopkins Hospital, 1800 Orleans Street,
Baltimore, MD 21287, USA.
Email: jvia2@jhmi.edu
2 Clinical Pediatrics 00(0)
(G6PD deficiency, hereditary spherocytosis, etc), child-
hood cancers, environmental exposures, or travel.
The head ultrasound was read as normal, but the
abdominal ultrasound confirmed the presence of an
acute bleed. Findings were notable for a heterogeneous
mass in the left upper quadrant measuring approxi-
mately 4.8 × 3.1 × 4.6 cm that was most likely to rep-
resent splenic hematoma in addition to a large volume of
ascites concerning for blood products. As this ultrasound
was being obtained, there were increasing clinical con-
cerns about the infant’s clinical status as she had increas-
ing abdominal distension and developed an oxygen
requirement for intermittent desaturations. A chest
radiograph revealed no abnormalities, and an arterial
blood gas was within normal limits. In consult with the
general pediatric surgery team, they recommended serial
abdominal ultrasounds and expectant management
given the patient’s hemodynamic stability.
In consult with pediatric hematology, the peripheral
blood smear had findings consistent with disseminated
intravascular coagulation that was also confirmed by a
low fibrinogen (111 mg/dL) and an elevated D-Dimer
(7.18 mg/L fibrinogen-equivalent units). Coagulation
studies were within normal limits for age. Her subsequent
medical management included a transfusion of cryopre-
cipitate, a 10 mL/kg platelet transfusion for thrombocyto-
penia (nadir of 88 × 103/mm3), and an additional 20 mL/
kg of packed RBCs. The patient required maximum respi-
ratory support of 2 L by nasal cannula and 34% FiO2
(fraction of inspired oxygen) but otherwise remained
hemodynamically stable. After improvement in her renal
function, an abdominal computed tomography (CT) scan
with intravenous contrast was done, which showed blood
outlining the splenic hilum, and confirmed the spleen
was the source of the bleeding.
In subsequent days of admission, she required photo-
therapy for hyperbilirubinemia and antibiotics were dis-
continued after 48 hours with negative blood and urine
cultures. She received 3 doses of vitamin K for her coagu-
lopathy, and an evaluation for a bleeding diathesis was
negative. One month after birth, she had a repeat abdomi-
nal ultrasound that showed a persistent, but smaller, splenic
hematoma. Pediatric hematology continued to evaluate her
as an outpatient for a coagulation disorder, but a compre-
hensive evaluation was unrevealing. The newborn screen
showed that the patient is a carrier for hemoglobin C trait.
Discussion
Neonatal splenic rupture is a rare clinical entity, and
the first patient to survive was not reported until the
1960s.1 Previously published case reports on this condi-
tion generally describe an infant who presents from 6 to
120 hours after a vaginal delivery with general pallor,
abdominal distension, poor feeding, and hypovolemic
shock.2 Cases of male infants have described scrotal
findings such as discoloration and swelling3,4 secondary
to the hemoperitoneum, which can distract clinicians
from the splenic etiology of the bleeding.4 Of note, a
recently published article describes 5 cases of infants
with splenic rupture who were critically ill at birth and
had signs of fetal distress prior to delivery, perhaps
describing an alternative mechanism of splenic injury
than those reviewed in this article.5
The underlying cause for the bleeding is highly vari-
able. One case describes maternal trauma prior to delivery
as the possible cause for splenic damage,6 but most cases
assume the delivery itself was the inciting event, espe-
cially in the case of a traumatic or difficult delivery,2,7-9 an
intrinsic bleeding disorder such as hemophilia A10-12 or
other clinical entities such as a wandering spleen,13
splenic hemangioendothelioma,14 and splenomegaly
caused by erythroblastosis fetalis.15 Many cases are idio-
pathic,2,4 with no cause identified, such as this case.
A theory first proposed in 1948 that continues to
propagate in the medical literature theorizes that
increases in intrathoracic pressure during a vaginal
delivery forces the liver and spleen from the diaphrag-
matic hollow causing excessive tension on the support-
ing ligaments16 predisposing hematoma formation. After
this inciting injury, the spleen develops a subcapsular
hematoma that can rupture17 causing hemoperitoneum
and eventual circulatory failure and death without
prompt intervention. The time delay between hematoma
formation and splenic rupture helps explain why most
infants have no symptoms immediately following birth
and cord blood hemoglobin levels are normal.
Historically, the management for splenic injury was
splenectomy, but in more recent years, the immunologi-
cal protection the spleen confers has shifted the prefer-
ence to conserving the spleen if able with surgical
methods aimed at achieving hemostasis,2,7,14 and if the
infant is hemodynamically stable, the preferred manage-
ment is nonoperative. The first report of nonoperative
management of neonatal splenic rupture was in 2000,7
and this case describes the sixth.3,10,18,19
In a description of cases from 1968 to 2002 published
by Hui and Tsui,2 all patients had surgery and 7 out of the
10 cases had splenectomies. Table 2 summarizes 13
cases of neonatal splenic rupture reported over the past
15 years. Seven of the cases required surgical manage-
ment: 3 required splenectomy, 3 were able to preserve
part of the spleen, and 1 case had an intraabdominal drain
placed. Of these 7, 6 infants were explicitly described as
hemodynamically unstable. The hemoglobin nadir or the
presence of a coagulopathy did not predict which infants
 Table 2. Cases of Neonatal Splenic Rupture With Hemoperitoneum Since 2003.

Gestational Age, Timing of Presentation, Hemoglobin

Study and Delivery Type, Birth Hour of Life (HOL), Day Nadir Coagulation Hemodynamic
Year Weight of Life (DOL) (in g/dL) Abnormalities? Instability? Surgical Management Proposed Etiology
Perdomo Full-term, spontaneous HOL 4: scrotal swelling 8.7 No No None Large infant
et al, 20033 vaginal delivery and discoloration
(SVD), 5.08 kg
Balliu et al, 36 weeks twin, HOL 22: pallor 3.4 Yes, ↑ aPTT 52 Yes Exploratory laparotomy Wandering spleen
200413 forceps-assisted SVD, seconds (s) (ex-lap) with syndrome
5.08 kg preservation of the
spleen
Driscoll Full-term, SVD, 3.6 kg DOL 2: poor feed, 5.8 Yes, ↑ PT 17.9s, ↓ No None Birth trauma
et al, abdominal distension, fibrinogen 120 mg/ (clavicle fracture
200418 emesis, pallor dL also present)
Ting et al, 27 weeks, cesarean HOL 20: apnea, 6.4 Yes, DIC described Yes Ex-lap for hemostasis Suspected birth
20069 section, 845 g hypotension, pallor, poor postoperatively and preservation of trauma, footling
perfusion the spleen breech extraction
Lewis et al, Full-term, forceps- HOL 30: poor feeding Hematocrit Yes, ↑ PT 17.3s, ↑ Yes Intraabdominal drain Suspected birth
20088 assisted SVD, 3.2 kg 18.9% aPTT 27.3s placed trauma
Tengsupakul Full-term, SVD, 3.4 kg DOL 3: poor feeding, 5 Yes, ↑ aPTT >260s Yes Ex-lap with evacuation Hemophilia A
et al, lethargy, emesis, pallor of blood clot and
201012 splenectomy
Lloyd and Full-term, SVD, 3.6 kg HOL 12: poor feeding and 7.5 No Yes Diagnostic paracentesis Unknown—
de Witt, lethargy and ex-lap with near- maternal assault
20116 total splenectomy during pregnancy
Adamu et al, Full-term, SVD, 3.45 kg DOL 3: poor feeding, Hematocrit Yes, ↑ aPTT >260s Unknown Ex-lap with splenectomy Hemophilia A
201211 emesis, pallor 14%
Tiboni et al, Full-term, SVD, 3.5 kg HOL 48: general pallor 7.1 Not reported Yes Scrotal exploration Unknown
20164 with scrotal swelling and followed by ex-lap
discoloration with splenectomy
Badawy Full-term, SVD, 2.95 kg DOL 11: poor feeding, 8.1 Yes, ↑ aPTT 76.5s, ↓ Unknown None Hemophilia A
et al, abdominal distension, VIII activity <1%
201710 and jaundice
Joshi et al, 24 weeks, cesarean HOL 16: anemia on 5.8 No No None Suspected
201719 section, 710 g routine bloodwork secondary to
breech extraction
Our case, Full-term, SVD, 3.02 kg HOL 40: pallor and a new 3.6 Yes, ↓ fibrinogen 111 No None Unknown
2018 heart murmur mg/dL, ↑ D-dimer
7.18 mg/L FEU

Abbreviations: aPTT, activated partial thromboplastin time; PT, prothrombin time; DIC, disseminated intravascular coagulation; FEU, fibrinogen-equivalent units.

3
4 Clinical Pediatrics 00(0)
were clinically unstable. While the preferred method of
3-dimensional abdominal imaging in neonates is ultra-
sound, in only 3 out of 8 cases that obtained an ultra-
sound prior to CT and/or surgery was the correct
diagnosis of splenic injury made.
To conclude, splenic rupture in the newborn period is
an uncommon, life-threatening event, and the potential
causes are diverse. The presentation can vary from criti-
cally ill at birth to the more commonly described insidi-
ous pattern of illness with a delayed onset of symptoms.
A difficult to predict hospital course often follows with
no reliable laboratory metrics to predict whether an
infant will become hemodynamically unstable and need
surgical intervention. Based on published literature, the
rates of splenectomy for this condition have decreased
in the past 15 years, although many infants continue to
require surgical intervention. And while an abdominal
ultrasound is often preferred over abdominal CT in neo-
nates, our literature review shows that splenic injury and
rupture may be visualized on ultrasound in less than
50% of cases. Splenic rupture continues to be an impor-
tant yet challenging diagnosis for pediatricians and neo-
natologists to recognize and manage given the variable
timing and presentation of the condition.
Author Contributions
JJM wrote and edited the manuscript and LMS reviewed and
edited the manuscript.
Declaration of Conflicting Interests
The author(s) declared no potential conflicts of interest with
respect to the research, authorship, and/or publication of this
article.
Funding
The author(s) received no financial support for the research,
authorship, and/or publication of this article.
Informed Consent
The family of our patient consented to this publication.
ORCID iD
Jennifer J. Miller https://orcid.org/0000-0002-5279-8208
References
1. Sokol DM, Tompkins D, Izant RJ Jr. Rupture of the spleen
and liver in the newborn: a report of the first survivor and
a review of the literature. J Pediatr Surg. 1974;9:227-229.
doi:10.1016/S0022-3468(74)80127-2
2. Hui CM, Tsui KY. Splenic rupture in a newborn. J Pediatr
Surg. 2002;37:E3. doi:10.1053/JPSU.2002.31641
3. Perdomo Y, Fiore N, Reyna T. Splenic injury present-
ing with isolated scrotal findings in a stable newborn.
J Pediatr Surg. 2003;38:1673-1675. doi:10.1016/S0022-
3468(03)00581-5
4. Tiboni S, Abdulmajid U, Pooboni S, Wighton C, Eradi
B, Dagash H. Spontaneous splenic hemorrhage in the
newborn. Eur J Pediatr Surg Rep. 2015;3:71-73. doi:
10.1055/s-0035-1564610
5. Descamps CS, Cneude F, Hays S, et al. Early hypovolemic
shock and abdominal distention due to neonatal splenic rup-
ture: urgency of diagnosis and management. Eur J Pediatr.
2017;176:1245-1250. doi:10.1007/s00431-017-2968-y
6. Lloyd L, de Witt W. Splenic rupture in a neonate—a rare
complication. S Afr J Child Health. 2011;5:94-96.
7. Bickler S, Ramachandran V, Gittes GK, Alonso M,
Snyder CL. Nonoperative management of newborn
splenic injury: a case report. J Pediatr Surg. 2000;35:500-
501. doi:10.1016/S0022-3468(00)90222-7
8. Lewis L, Sanoj KM, Poojari G, Kamath SP. Neonate
subcapsular splenic hematoma. Indian J Pediatr.
2008;75:950-952. doi:10.1007/s12098-008-0199-y
9. Ting JY, Lam BCC, Ngai CSW, Leung WC, Chan KL.
Splenic rupture in a premature neonate. Hong Kong Med
J. 2006;12:68-70.
10. Badawy SM, Rossoff J, Yallapragada S, Liem RI,
Sharathkumar AA. Successful medical management of
a neonate with spontaneous splenic rupture and severe
hemophilia A. Hematol Oncol Stem Cell Ther. 2017;10:29-
32. doi:10.1016/j.hemonc.2016.04.001
11. Adamu I, Asarian A, Xiao P. Splenic rupture and intracra-
nial haemorrhage in a haemophilic neonate: case report
and literature review. Afr J Paediatr Surg. 2012;9:163-
165. doi:10.4103/0189-6725.99408
12. Tengsupakul S, Sedrak A, Freed J, et al. Splenic rup-
ture in a newborn with severe hemophilia—case report
and review. J Pediatr Hematol Oncol. 2010;32:323-326.
doi:10.1097/MPH.0b013e3181d640ae
13. Balliu PR, Bregante J, Pérez-Velasco MC, et al. Splenic
haemorrhage in a newborn as the first manifestation of
wandering spleen syndrome. J Pediatr Surg. 2004;39:240-
242. doi:10.1016/j.jpedsurg.2003.10.025
14. Cerdá J, Mialdea RL, Soleto J, Martín-Crespo R, Aguilar
F. Segmentary splenectomy of the lower tip because of
spontaneous rupture of a splenic hemangioendothelioma
in a new-born child—a case report. Eur J Pediatr Surg.
1994;4:113-115.
15. Coulter JB, Raine PA. Rupture of the spleen in erythro-
blastosis fetalis. Arch Dis Child. 1975;50:398-400.
16. Gruenwald P. Rupture of liver and spleen in the newborn
infant. J Pediatr. 1948;33:195-201. doi:10.1016/S0022-
3476(48)80057-0
17. Chryss C, Aaron W. Successful treatment of rupture of
normal spleen in newborn. Am J Dis Child. 1980;134:418-
419. doi:10.1001/archpedi.1980.04490010072021
18. Driscoll K, Benjamin LC, Gilbert JC, Chahine AA.
Nonoperative management of neonatal splenic rupture.
Am Surg. 2004;70:1085-1087.
19. Joshi S, Mulinge I, Kamat M. An extremely premature
neonate with severe anemia. J Neonatal Surg. 2017;6:33.
doi:10.21699/jns.v6i2.547