CASE REPORT

Niger J Paed 2014; 41 (2): 141 –143

Mohamed CR

Sagbo GG

Hounnou GM

Can children with sickle cell disease

undergo open splenectomy without

preoperative transfusion despite

severe anemia? A report of three

cases

DOI:http://dx.doi.org/10.4314/njp.v41i2,14

Accepted: 23rd November 2013

Abstract Preoperative red cells

transfusion to correct anaemia and

to reduce the proportion of sickle

red cells is part of standard prepa-

ration of children with sickle cell

anaemia (HbSS) for major proce-

dures including open abdominal

surgeries. We report three chil-

dren with sickle cell anaemia pre-

senting with chronic massive

splenomegaly and hypersplenism.

The children were initially denied

surgery because of extremely low

haemoglobin levels and the ineffi-

cacy of transfusion. Subsequently,

they underwent successful open

abdominal splenectomy without

any red cells transfusion. These

observations are important to pae-

diatricians and surgeons in settings

where HbSS is common. They

highlight the fact that surgery

should not be withheld from chil-

dren with sickle cell anaemia and

massive splenomegaly purely on

the basis of difficulty in correcting

anaemia before the procedure.

(

)

Mohamed CR

National Institute for Infants and

Pregnant Women with Sickle Cell

Disease Care,

P.O. 01 Box 2640 RP Cotonou, the

Republic of Benin, West Africa.

E-mail: mrahimy@bj.refer.org

mrahimy2@yahoo.fr

Tel: +229 21 30 72 42,

Fax : +229 21 30 18 88

Sagbo GG

Department of Pediatrics

Key words: Sickle cell disease,

surgery, splenectomy, transfusion.

Hounnou GM

Department of Pediatric Surgery,

Faculty of Health Sciences, University of

Abomey-Calavi, Republic of Benin

Introduction

tres below the left costal margin, had a weight of 11,220

grams and a height of 76 cm.

Red cells transfusion to reduce the proportion of sickle

erythrocytes and to correct anaemia is part of standard

preoperative management of children with sickle cell

anaemia (HbSS), undergoing moderate or hig_-₃h-risk pro-

During the first 24 months of follow-up, several SCD-

related acute events were registered. These included

three mild vaso-occlusive crises successfully managed

on outpatient basis with aspirin and acetaminophen;

three episodes of fever without focus, two of which re-

quired hospitalization and intravenous antibiotics; and

notably the initiation of hypersplenism with progressive

enlargement of spleen, reaching the left iliac fossa. This

was associated with a progressive decrease in the values

of haemoglobin, declining from 65 g/L to 39 g/L. Two

attempts at red cell transfusion to correct the anaemia

resulted in acute abdominal pain associated with an in-

crease of spleen volume with no substantial change of

value of haemoglobin level. Splenectomy was proposed,

but both paediatric surgeon and anaesthetist dissented

because of his very low haemoglobin level, the ineffi-

cacy of red cell transfusion and the fear of peri-operative

complications leading to death.

1

cedures such as open abdominal surgeries. However

in an Africa setting, applying this preoperative care may

be difficult due to the unavailability of safe blood prod-

ucts or certain clinical conditions that may render trans-

fusion impossible. This may result in an anaesthetist or

surgeon denying the patient surgical procedures. Such a

situation poses a serious concern to the paediatrician

because of the predictably poor short-term outcome of

splenomegaly in HbSS disease. Here we report three

children with HbSS who successfully underwent open

splenectomy without any preoperative red cell transfu-

sion, despite the very low values of their haemoglobin,

demonstrating that it is possible for the paediatrician to

overcome this dilemma.

However, several discussions resulted in an agreement

on the following protocol, which was approved by the

Faculty of Health Sciences Ethics Committee. (1) In

addition to administration of a booster injection of 23

valence polysaccharide pneumococcal and meningococ-

cal vaccines three weeks before the procedure, the pre-

operative preparation would comprise hydration with

intravenous fluids (6-7 ml/kg/hour), starting 24 hours

before the procedure, administration of oxygen, prophy-

lactic intravenous administration of bactericidal antibiot-

Case presentation

th

Patient A, born on April 29 2006 was diagnosed HbSS

at the age of nine months and enrolled at the age of 12

months into our established comprehensive clinical care

programme (CCCP), which includes an intensive socio-

medical intervention programme to ameliorate the dis-

ease course in severely ill children. On admission he

presented with jaundice, enlarged spleen two centime-

4

1

42

ics, as well as careful monitoring of serum electrolytes,

heart rate, pulse oximetry and urinary outflow. (2) The

intra-operative management would comprise monitoring

of respiratory and heart rates, pulse oximetry, blood

pressure, electrocardiographic features and haemoglobin

level; in addition, appropriate bleeding control to mini-

mize intra-operative blood loss would be combined with

red cells transfusion after the ligature of the main splenic

artery. (3) The post-operative management would com-

prise continuation of the intravenous hydration and anti-

biotics, quinine administration until the third post-

operative day, at least, intravenous acetaminophen and

codeine to control postoperative pain, and recording of

all peri-operative clinical events.

showed a physical growth recovery pattern: during the

first 12 months post-operation, the weight and height

increases were 4,500g, 5,500g, 4,840g and 13cm, 7cm,

9cm for patients one, two and three, respectively, com-

pared to 2,000g, 1200g, 1400g, respectively, during the

follow-up time before surgery. At the time of this report,

no further long term adverse event was recorded in any

of the patients. Abdominal ultrasound scan performed

50, 48 and 34 months post-operatively in patients one,

two and three, respectively, showed no re-growth of the

residual spleen.

Table 1: Patient Demographics and Pre-operative Clinical

Characteristics

Patient Patient

Patient

C

After obtaining informed written parental consent, the

child underwent open abdominal surgery_t_h(laparoscopic

equipment was not available) on April 8 2009, at the

age of 36 months under general anaesthesia. The

Characteristic

A

B

Age (months)

Sex

Weight (grams)

Height (cm)

36

172

70

Male

14,900

109

15,000 20,500

procedure lasted 75 minutes and the surgeon performed

partial splenectomy, removing 850g portion of spleen;

the retained portion represented approximately 20% of

the spleen. Because no significant intra-operative

changes were observed in heart rate, pulse oximetry,

blood pressure, electrocardiographic features and values

of haemoglobin level, it was decided to refrain from the

planned intra-operative red cells transfusion. The recov-

ery room stay lasted three days and no adverse clinical

events were recorded post-operatively. Progressive in-

crease in haemoglobin levels were observed, reaching a

steady state level of 7.5 ± 5 g/L up to six months post-

operation, without any red cell transfusion. A transient

thrombocytosis was observed for which patient received

daily aspirin at 10 mg/kg for 45 days.

94

126

Length of follow (months)

24

93

54

SCD history

Painful crises

Acute chest syndrome

Fever without focus

Malaria attack

3

0

3

0

2

0

2

0

5

1

Focal infection

Transfusion

Hospitalization

Other medical history

0

3

2

0**

0

2

4

3

2

None

Weight increase* (grams)

2,000

1,200

1,400

Height increase* (cm)

Hemoglobin level (grams/L)

Platelets count (x 10 /L)

10

39

59

3

37

88

10

31

148

9

Given these encouraging results, two other children,

with a similar clinical presentation, previously denied

surgery, were then accepted for open abdominal

*

Observed increase during the follow-up before surgery

* This patient had never received a blood transfusion

th

splenectomy. Patient B was born on Janu_ta_hry 29 1995

Table 2: Patients Post-operative Data on Growth, Platelets

Counts and Hemoglobin Levels

and Patient C was born on November 4 2004_t_h. They

were enrolled _ti_hnto the CCCP on September 20 2001

and March 15 2006 respectively and underwent the

Patient

A

Patient Patient

th

Characteristic

procedure on June 10 2009 (at 172 months) and on

B

C

th

September 16 2010 respectively (at 70 months). About

Clinical events

Weight (grams)

Month + 1

Month + 3

Month + 6

Month + 12

Height (cm)

Month + 1

Month + 3

Month + 6

None

3

9

0% of the spleen (1,200g) was removed in patient two

while about 85% (1,100g) was resected in patient three.

Peri-operative fever without any identified focus of

infection was recorded in patient two on the first post-

operative day. A third generation cephalosporin, Ceftri-

axone was added to treatment. In patient three, cellulitis

of the left leg was observed on day 34 post-operatively,

requiring a two day hospitalization and intravenous

antibiotics.

14,000

16,000

17,200

18,500

17,500 14,200

20,000 16,420

22,000 17,880

23,000 18,840

95

98

103

108

126

127

131

133

110

113

118

119

Month + 12

Additionally, two episodes of vaso-occlusive crises were

recorded post-operatively on days 118 and 140, respec-

tively. None of these two patients required red cells

blood transfusion. Patients’ demographic and pre-

operative clinical characteristics are detailed in Table 1

and the post-operative growth, platelet count and hae-

moglobin levels are given in Table 2, Progressive in-

crease of steady-state haemoglobin levels was observed

reaching 78 ± 3 g/L and 81 ± 2 g/L in patients 2 and 3,

respectively up to 6 months post-operation. All patients

Hemoglobin level (grams/L)

Month + 1

Month + 3

Month + 6

Month + 12

Platelets count

Month + 1

Month + 3

Month + 6

Month + 12

55

65

75

78

51

67

76

80

60

77

82

80

857

650

450

330

981

631

535

440

704

585

486

420

1

43

Discussion

in their physiological status may not be suitable since

the patients have spent several months with their very

low levels of haemoglobin. Indeed, the physical growth

of the subjects after surgery far exceeded pre-operative

observations but also expectations of their otherwise

well, non-HbSS peers, suggesting catch-up growth. The

progressive increase in the haemoglobin levels and the

catch-up pattern of physical growth observed in these

cases provide new insights into the dynamic of erythro-

poiesis and organ function in children with HbSS. These

observations highlight the positive changes that our

CCCP can produce on HbSS children with severe pres-

entation of the disease. We hope that the cases presented

herein may help paediatricians convince anaesthetists

and surgeons not to abandon some of the affected chil-

dren presenting with massive chronic splenomegaly with

hypersplenism and extreme low haemoglobin level.

In children with HbSS, major surgery without preopera-

tive transfusion, has been reported to be associated with

serious peri-operative complications. Therefore, these

1

children usually receive pre-operative red cell transfu-

sion. However, the cases presented herein demonstrate

that in desperate situations, close cooperation among the

paediatrician, anaesthetist, and surgical team can still

permit the procedure without preoperative transfusion

and with minimal risk of peri-operative-related mortality

and morbidity. Despite the very low pre-operative hae-

moglobin levels and the open abdominal surgery, no

serious peri-operative or post-operative complications

were recorded in the three cases. Some recent studies

recommended that preoperative red cell_-t₇ransfusion was

5

not necessary for laparoscopic surgery. However; the

subjects of our report differ in two very important re-

spects. Firstly, the preoperative haemoglobin concentra-

tions of our subjects were all below 4.0g/L in contrast to

Conflict of interest: None

Funding: None

≥

6.0g/L in the reference studies. Secondly, our patients

underwent open abdominal surgery which is potentially

more associated with blood loss than laparoscopic sur-

gery.

Acknowledgments

Given the status of the respiratory and heart rates, pulse

oximetry and blood pressure; we have refrained from

post-operative red cell transfusion, despite low haemo-

globin levels. Instead, we reasoned that abrupt changes

We would like to thank the team of anaesthetists of the

National Teaching Hospital at Cotonou for their collabo-

ration during the procedures.

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