Single port robotic splenectomy for pyruvate kinase deficiency in a five–year-old patient, a case report of a surgical first

Abstract

Introduction
Adoption of robotic surgery in pediatrics has been slow. Robotic surgery within spatially-constrained workspaces in children makes traditional platforms less translatable. Da Vinci's newest single port (SP) robotic platform provides narrow, and deep access, making pediatric robotic surgery more feasible.

Case presentation
A five-year old female presented with hepatosplenomegaly due to hemolytic anemia from pyruvate kinase deficiency (PKD). When she progressed to requiring monthly transfusions, a splenectomy was performed to avoid the complications associated with frequent blood transfusions. The robotic approach was used to remove the intact spleen because traditional minimally invasive surgery can result in post-operative splenosis.

Discussion
The patient successfully underwent single-port, robotic splenectomy - the first known splenectomy in a child using this approach. Furthermore, during the operation an accessory spleen was encountered in the omentum and was also successfully removed robotically. The patient tolerated the procedure well.

Conclusion
This case demonstrates that the SP robot can be used for splenectomy to eliminate the risk of splenosis and achieve a superior cosmetic result.

Full text

International Journal of Surgery Case Reports 84 (2021) 106122
Available online 19 June 2021
2210-2612/© 2021 The Author(s). Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
Case report
Single port robotic splenectomy for pyruvate kinase deciency in a
ve–year-old patient, a case report of a surgical rst
Greg Klazura
*
, Thomas Sims, Marko Rojnica, Nathaniel Koo, Thom Lobe
University of Illinois Hospital and Health Sciences System, Division of Pediatric Surgery, Department of Surgery, 840 S. Wood St. Ste 406, Chicago, IL 606112, United
States of America
ARTICLE INFO
Keywords:
Case report
Splenectomy
Robotic
Single port
Pediatric
da Vinci
ABSTRACT
Introduction: Adoption of robotic surgery in pediatrics has been slow. Robotic surgery within spatially-
constrained workspaces in children makes traditional platforms less translatable. Da Vinci's newest single port
(SP) robotic platform provides narrow, and deep access, making pediatric robotic surgery more feasible.
Case presentation: A ve-year old female presented with hepatosplenomegaly due to hemolytic anemia from
pyruvate kinase deciency (PKD). When she progressed to requiring monthly transfusions, a splenectomy was
performed to avoid the complications associated with frequent blood transfusions. The robotic approach was
used to remove the intact spleen because traditional minimally invasive surgery can result in post-operative
splenosis.
Discussion: The patient successfully underwent single-port, robotic splenectomy - the rst known splenectomy in
a child using this approach. Furthermore, during the operation an accessory spleen was encountered in the
omentum and was also successfully removed robotically. The patient tolerated the procedure well.
Conclusion: This case demonstrates that the SP robot can be used for splenectomy to eliminate the risk of sple-
nosis and achieve a superior cosmetic result.
1. Introduction [1–6]
Widespread adoption of robotic surgery in pediatrics has been slow
because minimal access surgery within spatially-constrained work-
spaces in children makes traditional platforms less translatable. Da
Vinci's single port (SP) robotic platform appears to make pediatric ro-
botic surgery more feasible.
SP surgery can be performed with a single incision. We used a
Pfannenstiel incision, well concealed in the lower abdominal crease, for
removal of the intact spleen, eliminating the concern for splenosis. This
approach also minimized the scarring and incisional pain associated
with multiple points of entry used during laparoscopic surgery.
The SP consists of a exible 3-D camera and three exible, inter-
changeable instruments, all of which pass through a 2.5 cm cannula. The
instruments have mid instrument exion and can be manipulated
immediately beyond the end of the cannula. Once deployed the tele-
scope and instruments are functional within a 7 cm diameter space. This
technology provides narrow, deep access [1] making it highly applicable
to pediatric surgery.
A full array of interchangeable instruments are available for tissue
manipulation, suturing, and cauterization. Notably, the SP allows the
telescope to move in a full 180◦arc without moving instruments relative
to the tissue eld, improving visualization in areas that previously were
difcult to access. Finally, the robot can rotate in a complete 360◦circle
without changing ports, allowing the surgeon to access the complete
abdomen.
Our patient suffered from Pyruvate kinase deciency (PKD), a rare
hemolytic anemia. Hepatosplenomegaly is due to extramedullary he-
matopoiesis and trapped cells leading to engorgement. Patients present
with anemia, jaundice, hepatosplenomegaly and possibly cholelithiasis.
The primary treatment is blood transfusion [2]. Splenectomy for
hypersplenism is reserved for patients who require chronic transfusions.
When splenectomy is required, the standard of care is laparoscopic
splenectomy [3]. However, splenosis is a risk with this approach. The
advent of the SP system made a robotic approach more feasible, safer
and provided an opportunity for the standard of care to be advanced.
* Corresponding author.
E-mail addresses: greg.klazura@gmail.com (G. Klazura), tlsims@uic.edu (T. Sims), mrojnica@uic.edu (M. Rojnica), nkoo2@uic.edu (N. Koo), tlobe@uic.edu
(T. Lobe).
Contents lists available at ScienceDirect
International Journal of Surgery Case Reports
journal homepage: www.elsevier.com/locate/ijscr
https://doi.org/10.1016/j.ijscr.2021.106122
Received 26 May 2021; Received in revised form 14 June 2021; Accepted 16 June 2021

International Journal of Surgery Case Reports 84 (2021) 106122
2
2. Patient information and clinical ndings
This patient was a ve years-old Romani female who suffered from
hemolytic anemia due to PKD. Splenectomy was delayed because this
patient's mutation was associated with decreasing transfusion re-
quirements with age. Transfusion requirements, however, progressively
increased. The patient suffered from hepatosplenomegaly, transfusion
iron overload, erythrocyte alloimmunization, multiple admissions for
fever of unknown origin and chronic cholestatic jaundice. After
extended discussions the family agreed to splenectomy by Dr. Thom
Lobe.
On preoperative examination the patient was active with normal
affect. She had mild scleral icterus, the liver was noted 2–3 cm below the
costal margin and the spleen 11 cm below the costal margin. The skin
was jaundiced in the face, trunk and abdomen. Her blood-work 4 days
prior to operation revealed hemoglobin =8.1, total bilirubin =3.5 and
ferritin =3046 NG/ML (ref range 5–116 NG/ML).
Preoperative marking for Pfannenstiel incision and palpable caudal extent of spleen. Skin ap raised in preparation for single port.
Clipping of splenic vessels. Ex-vivo spleen removed completely intact, measuring 16.9 cm.
G. Klazura et al.

International Journal of Surgery Case Reports 84 (2021) 106122
3
3. Timeline
•Born 1/2016 at 38 WGA, shortly after diagnosed with PKD.
•4/2016 Right upper extremity midline venous catheter placed for
frequent transfusions.
•10/2017 MRI heart/liver demonstrated splenomegaly to 12 cm and
transfusion-associated iron overload.
•3/2018 Patient prescribed iron chelator.
•2/2019 MRI abdomen demonstrated splenomegaly (14.6 cm).
•7/2019–8/2020 Admitted multiple times for fever and empiric
antibiotics.
•1/2021 - Received pre-splenectomy pneumococcal & meningococcal
vaccines (Hib administered in 2016).
•2/28/2021 SP robotic splenectomy, patient discharged four days
later taking acetaminophen for pain and prophylactic penicillin.
•3/28/2021 Patient was tolerating diet, having regular bowel move-
ments, and not requiring pain medications.
4. Diagnostic assessment
•Physical exam at birth revealed hepatosplenomegaly, laboratory
values showed hyperbilirubinemia, anemia, and thrombocytopenia.
•Genetic testing revealed homozygous PKLR mutational deletion of
exon 11, an autosomal recessive inheritance seen in Romani families
causing PKD.
•Postoperative pathology reported a 16.9 ×8.3 ×6.7 cm spleen
weighing 361 g consisting of benign splenic tissue with congestion
and extramedullary hematopoiesis and conrmed the presence of an
accessory spleen.
5. Intervention
The patient was admitted the night prior to the operation for RBC
transfusion and IV uid administration. Overnight IV uids were
administered to prevent sludging secondary to hypersplenism in the
setting of NPO.
Under general anesthesia, Foley catheter was placed and the patient
was prepped and draped and prophylactic cefazolin was administered.
A curvilinear incision was made in the lower abdominal crease and
the ap was raised cephalad to the level of the umbilicus. Midway be-
tween the incision and the umbilicus, underneath the ap, the single-
port was inserted in the midline, and the abdomen was insufated.
The port was used to dock the robot, and the short gastric vessels
were divided with cautery. A laparoscopic Kittner was placed as a
retractor through an accessory 5 mm cannula, inserted in the midline
just cephalad to the port. Attention was then turned posteriorly where
the spleen was retracted anterolaterally, exposing the hilum. Hilar tissue
was divided with cautery until the splenic vessels were encountered.
The splenic vein and artery were divided between clips, and the rest
of the hilum was divided to completely free the spleen. Prior to
undocking the robot, a small accessory spleen was seen in the omentum
and removed. The incision was opened in the midline from the umbilicus
to pubis to insert a hand and extract the intact spleen.
Once the spleen was removed, the abdomen was closed. Estimated
blood loss was 20 ml. A caudal regional block was performed for post-
operative analgesia. The length of the surgery was 2 h and 44 min. Work
has been reported in line with the SCARE 2020 criteria [6] and has been
registered at ClinicalTrials.gov (NCT04899557).
6. Follow-up and outcomes
One month follow up photograph of hidden incision in abdominal
crease.
The patient tolerated the procedure well and was seen in clinic one
month after surgery. She did not require additional analgesics but took
benadryl for a rash that developed around the incision. She had not
required blood transfusion since before surgery.
7. Discussion
Partial splenectomy, open splenectomy, and laparoscopic splenec-
tomy are all alternatives to robotic splenectomy. Although robotic sur-
gery can be more costly than traditional approaches, the increased
functionality gives the experienced robotic surgeon far more tools to
safely perform an operation. The robotic approach has the same relative
and absolute contraindications as any laparoscopic surgery: inability to
tolerate pneumoperitoneum, uncorrectable coagulopathy, abdominal
compartment syndrome, abdominal wall infection, and previous
extensive abdominal surgery [4].
When the splenic capsule is violated there is opportunity for tiny
fragments of splenic tissue to seed the abdomen and the risk of splenosis
after laparoscopic splenectomy is well documented. Splenosis can cause
abdominal pain, adhesions and the need for additional surgery [5].
Remaining splenic tissue can also defeat the benets of splenectomy for
patients suffering from hemolytic disease. Given this concern the family
followed our recommendation for a robotic approach.
8. Conclusion
In this case, the SP robotic splenectomy was safe and effective. The
curvilinear incision in the abdominal crease provided cosmetic
improvement over multiple abdominal trocar sites, and the patient re-
ported less pain than usual with a prompt return to full activity. The
cost, length of procedure and long-term complications must all be
considered, but this operation sets an exciting precedent for SP robotic
surgery and pushes the needle forward for what is possible in children's
robotic surgery.
G. Klazura et al.

International Journal of Surgery Case Reports 84 (2021) 106122
4
Provenance and peer review
Not commissioned, externally peer-reviewed.
Sources of funding
University of Illinois at Chicago Department of Surgery.
Ethical approval
Research studies involving patients require ethical approval. Please
state whether approval or exemption has been given, name the relevant
ethics committee and the state the reference number for their judge-
ment. Please give a statement regarding ethnical approval that will be
included in the publication of your article, if the study is exempt from
ethnical approval in your institution please state this.
Approval given by the University of Illinois at Chicago Institutional
Review Board. The research protocol number is 2014-0396.
Informed consent(s)
Written informed consent was obtained from the patient for publi-
cation of this case report and accompanying images. A copy of the
written consent is available for review by the Editor-in-Chief of this
journal on request.
a) Waiver of Informed Consent granted under [45 CFR 46.116(d)]
Assent(s):
a) Waiver of Child Assent granted [45 CFR 46.116(d)] for the
retrospective phase of
research.
Parental Permission(s):
b) Waiver of Parental Permission granted [45 CFR 46.116 (d)] for the
retrospective chart
review phase.
HIPAA Authorization(s):
a) Waiver of Authorization granted under [45 CFR 164.512(i)]
Author contribution
Greg Klazura, original draft, project administration.
Thomas Sims, review & editing.
Marko Rojnica, review & editing.
Nathaniel Koo, review & editing.
Thom Lobe, review and editing.
Registration of research studies
Clinicaltrials.gov.
NCT04899557.
https://clinicaltrials.gov/ct2/show/NCT04899557.
https://clinicaltrials.gov/ct2/show/NCT04899557?id=04899
557&draw=2&rank=1.
Guarantor
Greg Klazura, MD, Thom Lobe, MD.
Declaration of competing interest
No conicts of interest.
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