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Robotic surgery in a 12-year-old girl to achieve Cholecystectomy and total Splenectomy indicated for complications of hereditary spherocytosis: Case report

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AIM: The application of robotic-assisted laparoscopy in pediatric surgery. MATERIALS AND METHOD: We used robotic-assisted laparoscopy to perform cholecystectomy and total splenectomy in a 12-year-old girl suffering from complications of chronic anemia due to hereditary spherocytosis. This report has been approved by our hospital Ethical Committee and we got a consent statement from her mother. Patient confidentiality has been respected and the intention of this work is mainly for scientific consideration. RESULTS: We successfully removed the gallbladder and spleen with accessories using a robotic system. This intervention was safe and less risky for our patient. She improved rapidly and has been discharged after 11 days. The total length of hospital stay was 25 days. The first post-operative visit has been planned after 3 weeks. CONCLUSION: Robotic-assisted laparoscopy is giving another issue to perform major surgeries in children. Such surgeries have since been considered more invasive with conventional laparoscopy.
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Robotic surgery in a 12-year-old girl to achieve
Cholecystectomy and total Splenectomy indicated
for complications of hereditary spherocytosis: Case
report
TOBIE ERIC NTSOBE
First Aliated Hospital of Gannan Medical College: First Aliated Hospital of Gannan Medical
University https://orcid.org/0000-0002-6520-865X
PENG WEI
First Aliated Hospital of Gannan Medical College: First Aliated Hospital of Gannan Medical
University
LIU HAIJIN ( liuhaji@163.com )
First Aliated Hospital of Gannan Medical College: First Aliated Hospital of Gannan Medical
University
CHEN FENG
First Aliated Hospital of Gannan Medical College: First Aliated Hospital of Gannan Medical
University
HUANG HAIJIN
First Aliated Hospital of Gannan Medical College: First Aliated Hospital of Gannan Medical
University
Case Report
Keywords: Robotic surgery, hereditary spherocytosis, hemolytic anemia, cholecystectomy, splenectomy.
Posted Date: January 28th, 2022
DOI: https://doi.org/10.21203/rs.3.rs-1301025/v1
License: This work is licensed under a Creative Commons Attribution 4.0 International License. 
Read Full License
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Abstract
AIM: The application of robotic-assisted laparoscopy in pediatric surgery.
MATERIALS AND METHOD:
We used robotic-assisted laparoscopy to perform cholecystectomy and total splenectomy in a 12-year-old
girl suffering from complications of chronic anemia due to hereditary spherocytosis. This report has been
approved by our hospital Ethical Committee and we got a consent statement from her mother. Patient
condentiality has been respected and the intention of this work is mainly for scientic consideration.
RESULTS: We successfully removed the gallbladder and spleen with accessories using a robotic system.
This intervention was safe and less risky for our patient. She improved rapidly and has been discharged
after 11 days. The total length of hospital stay was 25 days. The rst post-operative visit has been
planned after 3 weeks.
CONCLUSION:Robotic-assisted laparoscopy is giving another issue to perform major surgeries in
children. Such surgeries have since been considered more invasive with conventional laparoscopy.
Introduction
Hereditary spherocytosis is recognized to be a rare genetic disease that causes hemolytic anemia due to
congenital defects of red blood cells membrane. The diagnosis is based on increasing blood spherocytes
cells [1]. Spherical red blood cells that had lost their deformability will be destroyed by the spleen when
passing through splenic capillaries. The hyper-destruction of red blood cells leads to hemolytic anemia [2].
At the same time, red blood cells lysis releases a large quantity of bilirubin that increases splenic activity
and stimulates compensatory hyperplasia and secondary liver enlargement [3]. Common complications of
hereditary spherocytosis reported in the literature are bilirubin stones, anemia, hepatomegaly,
splenomegaly, chronic jaundice, reticulocytosis, fatigue, and abdominal discomfort [4]. Bilirubin gallstones
are the most recurrent with an incidence of about 40-50% and occur usually at the age of 10-30 years [5].
The treatment of those complications includes cholecystectomy and total splenectomy. We chose to
achieve this surgery using DA Vinci robotic system.
Results And Discussion
A 12-year-old girl was admitted to our department one month ago due to repeated pain in the right upper
abdominal quadrant for 6 years that got worst 3 days ago. The patient has a family history of hereditary
spherocytosis and hemolytic anemia. Her mother had undergone massive splenectomy due to
spherocytosis. The genetic test report done in 2019 revealed a variant of the SPTB gene and the
diagnosis of spherocytosis type 2 has since been evoked. Physical examination revealed jaundice of the
skin and sclera, a mildly distended abdomen, obvious tenderness in the right upper abdomen and around
the umbilicus, positive Murphy’s sign, mild hepatomegaly, and splenomegaly. We assessed an Abdominal
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Enhanced CT scan that revealed, lower common bile duct stones with dilatation of intra hepatic and
extrahepatic bile ducts; multiple gallbladder stones; Splenomegaly with low splenic signal, suggesting
iron deposition (Figure 1A). Biological Report was: WBC: 16.06x10*9/L, HB: 71g/L, total bilirubin: 430.8
µmol/L, indirect bilirubin: 385.1µmol/L.
On admission, we considered diagnosis of Cholangio cholecystitis associated to chronic splenomegaly
due to high splenic activity. Indications of ERCP, cholecystectomy and splenectomy have been discussed
and adopted by our medical staff. Before intervention, the Patient received medical treatment constituted
by antibiotics, hepato protectors and correction of anemia. The ERCP has been performed 5 days after
admission and aimed to remove stones in the bile ducts and reduce pain. After this procedure, a control
CT scan has been assessed to conrm the patency of different bile ducts (Figure 1 B). The patient
underwent a robot-assisted laparoscopy 9 days following ECRP to achieve cholecystectomy and total
splenectomy. The patient was in Trendelenburg position and 5 ports have been created with disposable
trocars; an infra umbilical port for camera and 4 working ports as shown in Figure 2A. We proceed rst to
cholecystectomy according to the standard procedure of robotic surgery. The gallbladder was released
from the liver; after dissection of Calot triangle; cystic artery and cystic duct were exposed and ligated
with hemlock clamps before being cut with robotic knife and hemostasis was completed. Then robot’s
arms were adjusted to achieve splenectomy. The omentum was pulled away and splenic artery identied
at the upper edge of the pancreas and ligated. We continued dissection of splenogastric, splenocolic and
splenorenal ligaments that were cut progressively. The spleen was released from the diaphragm and
splenic artery cut under hemostasis control. We completed resection of spleen accessories and
surrounding omentum. Splenic and gallbladder beds have been checked after different resections and no
bleeding was active; blood loss evaluated in the suction reservoir was 30ml. Different resected organs
have been pulled out with endo bags via a para supra pubic incision and specimen sent for
histopathologic analysis (Figure 3). Results showed chronic gallbladder inammation, vasodilatation,
and congestion of splenic tissue and accessories (Figure 4). Post operative treatment consisted of
antibiotics, analgesics, hepato protectors and anti-platelet aggregation drugs. Biological assessment
control after operation was as follows: WBC: 7.46x10*9/L, HB: 110g/L, total bilirubin: 37.7µmol/L, and
indirect bilirubin: 19.1µmol/L. The patient recovered rapidly, the wound healing was good (Figure 2 B) and
she has been discharged 11 days after operation. The total length of hospital stay was 25 days and the
rst postoperative visit has been planned 3 weeks later.
Since the FDA approved the DA Vinci robotic surgery system in 2000, robotic surgery has gradually been
introduced in urology, general surgery, cardiothoracic surgery, gynecology, and n China. However, due to
the scarcity of this new technology, technical diculties and small body cavity in children, development
of robotic surgery in pediatric population has been slower than in other departments. But, with advances
in minimally invasive surgery, many hospitals have introduced the usage of robot in pediatric surgery. Out
of the number of DA Vinci robotic system that is increasing; implementation of robotic in Pediatric
surgery has been considerable in recent years [6]. Nowadays, many pediatric surgeons are already
qualied for robotic operations. Since 2004, some scholars in US projected that robotic surgery in
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pediatrics is the most promising [7]. This may be due to the current hardware equipment and the
characteristics of pediatric surgical diseases. Current authors recognize that robotic surgery is safe in
children with a body weight of more than 15 kilograms and aged more than 2 years [8]. Our patient was
12 years old and weighed 41 kilograms, which is still widely recognized as eligible for robotic surgery.
Robotic surgery is feasible to remove spleen and gallbladder at the same time. In case of an idiopathic
thrombocytopenic purpura, hereditary spherocytosis or thalassemia with indications of simultaneous
cholecystectomy and splenectomy, surgeons have been using laparoscopy to achieve this surgery [9–11].
Many cases of robotic surgery for multiple organ surgery have been reported in recent years. A 61-year-
old woman with multifocal intraductal papillary mucinous tumor underwent simultaneous removal of the
pancreas and spleen using DA Vinci robotic machine and 6 ports to achieve this surgery [12]. In addition, a
62-year-old male patient with pancreatic tail tumors and liver metastases, stomach involvement, splenic
inexion and colon compression underwent multiple organs removal with robotic system. The Surgeon
performed simultaneously pancreatectomy, splenectomy, gastric sleeve resection, and cholecystectomy
[13]. Recently in 2018, an Italian surgeon reported one case of a lymphoblastic auto-immune syndrome on
a 52-year-old woman where he used robot to remove spleen and gallbladder at the same time [14].
Certainly, this report of simultaneous removal of the gallbladder and spleens in a child using robotic
system will provide a new option for similar cases in the future. Patients with hereditary spherocytosis
Complicated with symptomatic gallbladder stones and aged more than 6 years have indications of
simultaneous removal of the gallbladder and spleen [15, 16]. Laparoscopic removal of the gallbladder and
spleen for treatment of hereditary spherocytosis and complications has since been reported [17, 18].
Splenectomy is safe and helpful for patients with hereditary spherocytosis [19]. A few numbers of patients
did not improve because of the presence of spleen accessories [20]. Our patient had an accessory spleen;
thanks to the high-denition camera of the robotic system, we carefully removed this accessory in order
to minimize the possibility of recurrence.
Conclusion
Hereditary spherocytosis is a rare disease in the general population and radical splenectomy has been
described as the best surgical treatment. Gallstones are recurrent complications and simultaneous
splenectomy and cholecystectomy have been constituted the aim of our treatment. Although the usage of
robotic-assisted laparoscopy in pediatric surgery is facing some diculties such as the lack of specic
equipment for children, narrow body cavities, we realized a successful surgery and our patient got
improved without complications. Robotic system has advantages to perform simultaneously multiple
interventions compared to conventional laparoscopy that requires minimal and noninvasive surgery.
Declarations
AUTHORS CONTRIBUTIONS AND DISCLOSURE
Dr. Peng Wei consulted the patient, accessed investigations, and gave an indication of surgical treatment.
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Dr. Liu Haijin was the surgeon and has been assisted by Dr. Huang Haijin and Dr. Chen Feng.
Dr. Eric Tobie Ntsobe was charged to write this report.
Doctors Peng Wei, Eric Tobie NTSOBE, Liu Haijin, Huang Haijin, and Chen Feng have no conict of
interest to disclose for this report.
ETHICS STATEMENT & CONSENT
This report has been approved by our hospital Ethical Committee and we got a consent statement from
her mother.
All authors have contributed signicantly and all are agreed with the content of the manuscript and we
declare no conict of interest for this work. 
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Figures
Figure 1
CTScan before and after ERCP operation
Figure 1A:Image before ERCP showed Stones in the lower part of the common bile duct with mild
dilation of intra- and extra hepatic bile ducts; multiple stones in the gallbladder; splenomegaly, low splenic
signal
.
Figure 1B: CT scan after ERCP showed postoperative changes of stones in the lower part of the common
bile duct; stones in the gallbladder; we considered retention of contrast agent in the gallbladder and bile
duct.
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Figure 2
Port positions and wound healing
Figure 2A: Per operative port positions.
Figure 2B: postoperative wound healing (the para supra pubic incision was done to take out specimen).
Figure 3
Pictures of surgery process
Figure 3A, 3B, 3C: Gallbladder resection
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Figure 3D, 3E, 3F: Splenectomy process
Figure 3G, 3H, 3I: Spleen accessory
Figure 4
Histopathologic results (10x)
Figure 4A:Chronic inammation of the gallbladder;
Figure 4B:Spleen tissue presenting vasodilatation and congestion;
Figure 4C: Spleen accessory showing also vasodilatation congestion
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Article
We report a rare case of hereditary spherocytosis (HS) and hereditary persistence of fetal hemoglobin (Hb) (HPFH) complicated with a β-thalassemia (β-thal) trait and a Krüppel-like factor 1 (KLF1) gene mutation misdiagnosed as β-thal intermedia (β-TI) due to a high percentage of Hb F. The proband presented with pale skin, jaundice and splenomegaly. Analysis of the thalassemia gene indicated βcodon 17/βA (HBB: c.52A>T), while Hb analysis showed significantly increased Hb F levels. The proband was diagnosed to carry β-TI, and a blood transfusion regimen together with iron chelation treatment was recommended. Due to the difference between the phenotype and genotype, next generation sequencing (NGS) was performed and the proband was found to carry a homozygous mutation on the SPTB gene combined with a heterozygous mutation in KLF1. An eosin-5-maleimide binding test (EMA-BT) showed that the mean fluorescence intensity decreased by 47.1%. The proband was finally diagnosed with HS and HPFH complicated with a β-thal trait and the high percentage of Hb F was believed to be ascribed to the KLF1 gene mutation, which is frequent in areas where thalassemia is prevalent. For patients with a β gene mutation accompanying significantly high percentage of Hb F, the diagnosis of β-TI could be warranted, and the influence of the KLF1 gene mutation should be carefully excluded to avoid misdiagnosis of other types of hereditary hemolytic diseases.
Article
Introduction: New generation osmotic gradient ektacytometry has become a powerful procedure for measuring red blood cell deformability and therefore for the diagnosis of red blood cell membrane disorders. In this study, we aim to provide further support to the usefulness of osmotic gradient ektacytometry for the differential diagnosis of hereditary spherocytosis by measuring the optimal cutoff values of the parameters provided by this technique. Methods: A total of 65 cases of hereditary spherocytosis, 7 hereditary elliptocytosis, 3 hereditary xerocytosis, and 171 normal controls were analyzed with osmotic gradient ektacytometry in addition to the routine red blood cell laboratory techniques. The most robust osmoscan parameters for hereditary spherocytosis diagnosis were determined using receiver operating characteristic curve analysis. Results: The best diagnostic criteria for hereditary spherocytosis were the combination of decreased minimal elongation index up to 3% and increased minimal osmolality point up to 5.2% when compared to the mean of controls. Using this established criterion, osmotic gradient ektacytometry reported a sensitivity of 93.85% and a specificity of 98.38% for the diagnosis of hereditary spherocytosis. Conclusion: Osmotic gradient ektacytometry is an effective diagnostic test for hereditary spherocytosis and enables its differential diagnosis with other red blood cell membrane diseases based on specific pathology profiles.
Article
Hereditary spherocytosis is an inherited hemolytic anemia caused by a deficiency in erythrocyte membrane proteins. Removal of the spleen may reduce the intra-splenic hemolytic process of the disease and, therefore, may correct the anemia. Furthermore, it seems to decrease the levels of serum bilirubin, thus reducing the formation of gallbladder stones. Indications and timing of splenectomy, however, are still debated. Twelve patients with severe hereditary spherocytosis operated on with laparoscopic splenectomy were retrospectively reviewed. Median age at diagnosis was 13.8 years (range 8-25 years). Male to female ratio was 5/7. Indications for laparoscopic removal of the spleen included anemia unresponsive to iron supplementation in eight patients (66.6 %) with increase need for red cells transfusions, and jaundice with symptoms related to cholelitiasis in four patients (33.3 %). Laparoscopic splenectomy was associated in four cases to laparoscopic cholecystectomy. Mean operative time was 50 min (range 40-75 min) with no conversion to open surgery. Mean hospital stay ranged from 3 to 7 days. In a 16-month follow-up, no complications were recorded and a persistent correction of anemia was observed. With the advent of laparoscopic surgery, splenectomy has been performed by this mini-invasive approach in referral centers. Laparoscopic splenectomy is an effective technique, when performed in patients with hereditary spherocytosis. Low complication rate and persistent correction of the hematologic disorders can be expected after the laparoscopic splenectomy, provided that a proper technique is performed and an experienced surgical team is available.
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Robotic surgery will give surgeons the ability to perform essentially tremorless microsurgery in tiny spaces with delicate precision and may enable procedures never before possible on children, neonates, and fetuses. Collaboration with radiologists, engineers, and other scientists will permit refinement of image-guided technologies and allow the realization of truly remarkable concepts in minimally invasive surgery. While robotic surgery is now in clinical use in several surgical specialties (heart bypass, prostate removal, and various gastrointestinal procedures), the greatest promise of robotics lies in pediatric surgery. We will briefly review the history and background of robotic technology in surgery, discuss its present benefits and uses and those being explored, and speculate on the future, with attention to the current and potential involvement of imaging modalities and the role of image guidance.
Analysis of gene mutation characteristics in 37 cases of hereditary spherocytosis
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  • Jin Linping
  • Zhang Li
Peng Guangxin, Yang Wenrui, Zhao Xin,Jin Linping, Zhang Li,et al. Analysis of gene mutation characteristics in 37 cases of hereditary spherocytosis[J]. Chinese Journal of Hematology 2018, Volume 39, Issue 11, Pages 898-903 MEDLINE ISTIC PKU CSCD CA, 2018. DOI 10.3760/cma.j.issn.0253-2727.2018.11.005
Diagnosis of hereditary spherocytosis and secondary hemochromatosis in a patient with jaundice
  • Y Gao
  • B Zhang
  • Y Song
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  • Y Bao
Gao Y, Zhang B, Song Y, Li G, Bao Y, et al. Diagnosis of hereditary spherocytosis and secondary hemochromatosis in a patient with jaundice[J]. Acta haematologica, 2018, 139(3): 168-170. DOI 10.1159/000486948
High incidence of early cholelithiasis detected by ultrasonography in children and young adults with hereditary spherocytosis
  • H Tamary
  • S Aviner
  • E Freud
  • H Miskin
  • T Krasnov
Tamary H, Aviner S, Freud E, Miskin H, Krasnov T, et al.High incidence of early cholelithiasis detected by ultrasonography in children and young adults with hereditary spherocytosis. J Pediatr Hematol Oncol 2003, 25(12):952-954.DOI 10.1097/00043426-200312000-00009
Robot-assisted surgery in children: current status and prospects
  • Tang S T
TANG S T. Robot-assisted surgery in children: current status and prospects [J]. Chinese Journal of Robotic Surgery,2021, 2(4):241-247.DOI 10.12180/j.issn.2096-7721.2021.04.00
Current Status of Pediatric Robot-Assisted Surgery in Italy: Epidemiologic National Survey and Future Directions
  • C Esposito
  • L Masieri
  • M Castagnetti
  • G Pelizzo
  • M Escolino
Esposito C, Masieri L, Castagnetti M, Pelizzo G, Escolino M. Current Status of Pediatric Robot-Assisted Surgery in Italy: Epidemiologic National Survey and Future Directions[J]. Journal of Laparoendoscopic & Advanced Surgical Techniques, 2020. DOI 10.1089/lap.2019.0516
Laparoscopic splenectomy and/or cholecystectomy for children with sickle cell disease
  • A Alwabari
  • L Parida
Alwabari A, Parida L, Al-Salem A H. Laparoscopic splenectomy and/or cholecystectomy for children with sickle cell disease[J]. Pediatric surgery international, 2009, 25(5): 417-421. DOI 10.1007/s00383-009-2352-8