The Wandering Spleen: Laparoscopic Splenectomy in a Pregnant Woman. Case Report and Review of the Literature

doi:10.21203/rs.3.rs-615425/v1

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The Wandering Spleen: Laparoscopic Splenectomy in a Pregnant Woman. Case Report and Review of the Literature

https://doi.org/10.21203/rs.3.rs-615425/v1

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Introduction: Wandering spleen (WS) is a rare condition, which is characterized by an augmented mobility of the spleen, due to congenital or acquired causes. It’s more frequent in multiparous women, but only a few cases are reported during pregnancy. WS is usually asymptomatic until the onset of ìts possible complications, and this can mislead clinicians in reaching the correct diagnosis.

Case Report: We report the case of WS with acute splenic infarction in a pregnant woman and its mininvasive surgical treatment, focusing on the clinical and radiological findings that could mislead or drive clinicians to the right diagnosis, or to a potential disaster.

Conclusion: Splenic preservation in WS where it’s safe and possible and is the main goal of the treatment. Clinical and radiological findings are the most relevant elements to drive surgical treatment.

WS is an insidious condition, and must be included in differential diagnosis in determined cases, to reach the best and opportune treatment for the patient.

wandering spleen

pregnancy

splenopexy

acute abdomen

case report

Wandering spleen (WS) is a rare and still relatively unknown condition. It is characterized by an augmented mobility of the spleen, due to congenital lack of splenic ligaments or an acquired increased laxity of these structures.

This relaxation of the tissues may lead to spontaneous torsions of the splenic hilum, with possible subsequent acute or chronic ischemic infarctions of the spleen, making WS a deceptive and misleading condition.

Until 2012 WS cases reported in the medical literature were about 500 worldwide, almost a 0,5% of splenectomy performed [1]. Since 2012 there have been reported in literature almost 150 new cases.

The real incidence of this pathology is surely underestimated because of the healthy carriers, making WS a silent condition until the appearance of its complications, which could also be life-threatening.

Most of the cases are reported in child bearing age women, in particular multiparous ones. probably in relation with the augmented laxity of the tissue due to hormonal values acquired during pregnancies.

This hypothesis is confirmed by the observation of an asymmetrical distribution of WS in the adult population between female and male, with a proportion approximately of 7:1 [2].

The second peak incidence is in the pediatric population, with equal distribution between male and female, and a medium age less than 10 years old [3]. In these cases the WS is probably related to congenital lack of splenic ligaments, giving the organ augmented mobility and subsequent possible acute and/or chronic torsion on its longitudinal axis.

In the medical literature there are only few cases of WS during pregnancy [4, 5], getting to complex and dangerous clinical pictures, complicated from the coexistence of the fetus.

This study is a review of publications regarding WS and a case report of the most advanced gestational age WS during pregnancy, as far as we know in the medical literature.

This rare condition was first described by the Van Horne in 1667, while the first scientific report about this pathology was redacted by a Polish doctor, Jozef Dietl, who described this anatomical variation, assuming for the first time the laxity of the ligaments as its possible etiogenesis [7].

WS rests usually unknown until the appearance of a complication, such as chronic or acute ischemic damage, resulting from the torsion of the vascular axis, leading to potentially life-threatening conditions.

For this reason, there are no clear clinical features related to WS, because it depends on the phases of the splenic wandering, and could remain undiagnosed all lifelong.

The principal diagnostic tool in these cases is a radiological exam, such as ultrasound, CT scan and MRI [8, 9, 10, 11], directing clinicians to correct treatment of this condition.

Radiological findings of WS are enlarged and hypertrophic splenic vessels, no spleen in left hypochondrium and a mobile mass in the belly.

Ultrasound and CT scan with contrast are the most helpful techniques in case of acute torsion or thrombosis of the vascular splenic peduncle. The pathognomonic radiological appearance of the splenic vessels, called the “Whirlpool sign” [12], is associated with an absence of flow in the splenic parenchyma, and an augmented enhancement of the splenic capsule [11].

Treatment of WS, once diagnosis is reached, is strongly correlated with the risk of almost 65% of occurrence of WS complications using conservative treatment, leading clinicians to invasive treatment of this condition once the diagnosis is confirmed [13, 14, 15].

Surgery is the main treatment of WS, and there are two alternative approaches, depending on the WS condition.

Treatment of WS is founded on preoperative and intraoperative findings, based on splenic vascular state, grade of acute and/or chronic torsion of its hilum.

In case of an asymptomatic patient, in paediatric/young population with no irreversible damage of the splenic parenchyma and function, prevails the need of preserving splenic function, and splenopexy is the most indicated treatment, with many different possible techniques [5, 14, 16, 17].

From medical publications review, splenopexy offers optimal short and long term results regarding maintenance of vascularization of the splenic parenchyma and correct splenic location, making this technique world-wide accepted in case of elective treatment of WS [14, 17].

In an acute case of WS, with ischemic compromission of the splenic parenchyma, urgent splenectomy is the most indicated treatment, acting in a potentially life-threatening clinical situation [5, 7, 13].

Both these techniques can be performed laparoscopic, after evaluation of surgical experience and disposability of surgical material for surgery [7, 13, 14, 16, 17, 18]. The technique allows for relevant diminution of postoperative pain and complications, a minor length of stay and an earlier return to normal activities [13].

This case report has been reported in line with the SCARE criteria [6].

LZ, a 31 years white woman, BMI 20, primigravida, with no pathological history, no pharmacologic chronic therapy nor allergies reported.

Mild thrombocytopenia was found in previous blood exams, minimal value of 95⁹ platelets/L in 2016, never examined with second level exams.

During the first obstetrical ultrasound check, a vascularized and capsulated mobile mass in the abdomen was detected, with a major axis of 12,5 centimetres, characterized from an hypertrophic and elongated hilar vascular axis. In parallel, there was no spleen in the left hypochondrium, confirming WS diagnosis.

The WS had some parenchymal calcifical strias, as per previous parcellar ischemic infarctions.

This condition was totally asymptomatic, the physical examination was negative for any pathological finding, no palpable masses, no abdominal evocable pain.

The result of the multidisciplinary consult executed was an indication of close monitoring of WS through frequent ultrasound examinations and blood exams, with conservative treatment because of the initial weeks of the pregnancy.

During week 17th of the pregnancy the patient had blood exams, demonstrating a reduction of PLTs count to 87⁹/L, with stability of the WS in ultrasound check.

MRI has been planned in order to have more precise information and set the best treatment for the patient. Some days before the MRI, on October 30, 2019, during the 22th week of pregnancy, the patient got to the ER because of the onset of intense and diffuse abdominal pain during the night, without fever or any other clinical manifestation.

Blood exams were in the range of normality, except for further thrombocytopenia, with PLTs count to 74⁹/L.

Urgent ultrasound check was performed, observing augmented volume of the WS (diameter 18x8 cm), parenchymal ischemic injury, no vascular signal detected at the splenic hilum with Color-Doppler technique, without free fluid in the belly (Fig. 1).

For this reason, the patient underwent an urgent surgical procedure.

A laparoscopic approach was decided, noticing an hypertrophic mobile spleen in subhepatic space, surrounded by a multitude of ectasic and congested vessels. The principal vascular axis of the WS was also hypertrophic, with a diameter around 4 cm (Fig. 2).

After initial mobilization of the WS and partial dissection of the perisplenic vessel and isolation of the principal vascular axis, a median mini-laparotomy was performed to dissect and tie the hypertrophic splenic vessels with non resorbable suture (Fig. 3).

Using laparoscopy, a complete mobilization of the WS was performed from its remaining ligaments.

A subsequent extraction of the spleen from the laparotomy was executed from the service laparotomy. Fetal parameters resulted physiological and unaltered after surgery. Post operative course was regular, the PLTs count normalized in a few weeks, with a media of 300⁹/L. Subsequent blood exams confirmed normalization of PLTs values (Fig. 4).Patient had natural childbirth at week 39th with no complications.

Patient subsequently received Streptococcus Pneumoniae, Haemophilus Influenzae and Neisseria Meningitidis vaccinations, as standard prevention for most frequent severe infections after splenectomy.

WS is a rare and insidious condition, with many different possible manifestations, from asymptomatic patients to urgent and emergency situations, requiring prompt and opportune diagnosis and treatment, making this condition very challenging for clinicians.

WS is a diagnosis that must be included in differential diagnosis in particular cases, such as multiparous women with abdominal palpable mass or recurrent and vague abdominal pain.

Increase in WS diagnosis is supported by a constant and rapid improvement in radiological technique, getting more easily and accurately diagnosed in elective and acute examination.

Surgical splenic preservation is mandatory for the patient’s safety, with many possible laparoscopic and laparotomic treatments, depending on the surgeon’s ability and hospital’s available resources.

Splenectomy instead must be preferred when splenic vascularization is compromised, and the risk of splenic rupture is very high and risky for the patient.

WS knowledge is principally based on an increasing number of single case reports, such as this one, driving clinicians to manage this rare and complex diagnosis, both in emergency and in elective surgery.

A better understanding of WS etiopathogenesis is important to reach a complete comprehension of its pathological mechanisms, to get a better diagnostic and therapeutic ability, preventing a possible life-threatening condition.

Further studies are necessary to obtain a full understanding of WS’s genesis, to get a unique and standard diagnostic and therapeutic guideline, making WS best known and less dangerous condition.

i. Funding

No funding were received for this study

ii. Conflict of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

iii. Ethics approval

Not applicable

iv. Consent to participate

Fully informed written consent were obtained for surgery

v. Consent for publication

Fully informed written consent were obtained for publication

vi. Availability of data and material

All data and material are available from main author

vii. Code availability

Not applicable

viii. Authors' contributions

Lorenzo Crepaz and Alberto Di Leo wrote the manuscript.

Lorenzo Crepaz, Giuseppe Borzellino and Paola Pomini took part to surgical procedure.

Lorenzo Crepaz, Michele Genna managed preoperative and postoperative indications.

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The Wandering Spleen: Laparoscopic Splenectomy in a Pregnant Woman. Case Report and Review of the Literature

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