Safety and efficacy of minimally invasive splenectomy with endotherapy for non-cirrhotic portal fibrosis: A retrospective cohort study

Abstract

Non-cirrhotic portal fibrosis (NCPF), a leading cause of non-cirrhotic portal hypertension (NCPH), commonly presents with splenomegaly, esophageal varices, and preserved liver function. While minimally invasive splenectomy (MIS) offers advantages over open splenectomy, concerns persist due to the risks associated with portal hypertension. This study evaluates the feasibility, safety, and long-term outcomes of MIS in non-bleeder NCPF patients, highlighting perioperative challenges and techniques. Thirteen consecutive non-bleeder NCPF patients undergoing MIS between November 2017 and December 2023 were analyzed. Procedures included eight laparoscopic and four robotic splenectomies, with one conversion to open surgery. Additionally, two patients underwent laparoscopic and one robotic gastric devascularization. Perioperative parameters such as operative time, blood loss, hospital stay, and complications were recorded. The median operative time was 240 minutes, and median blood loss was 150 mL. One patient required transfusion, and one developed splanchnic venous thrombosis, managed conservatively. Median hospital stay was three days, with no 90-day mortality. Follow-up assessments included clinical evaluation, blood tests, Doppler ultrasound of the splenoportal axis, and upper gastrointestinal endoscopy. At a median follow-up of 16 months (interquartile range 12–43), significant improvements were observed in hemoglobin, leukocyte, and platelet counts (p < 0.01). Esophageal variceal grades decreased from 2 to 1, while portal vein peak systolic velocity improved from 18 to 27.7 cm/sec (p < 0.01), indicating reduced portal hypertension. No postoperative infections or variceal bleeding recurrences were noted. MIS is a safe and effective treatment option for non-bleeder NCPF with favorable long-term outcomes when performed by skilled surgeons.

INTRODUCTION

Non-cirrhotic portal hypertension (NCPH) is a broad term denoting a group of liver disorders characterized by portal hypertension (PHTN) alongside normal liver function tests (LFTs) and typical liver morphology. The two principal causes of NCPH are non-cirrhotic portal fibrosis (NCPF) and extrahepatic portal venous obstruction [1]. NCPF, also known as idiopathic portal fibrosis, is identified by periportal fibrosis and presinusoidal PHTN and is a major contributor to NCPH, particularly in the Indian subcontinent [2]. This condition is characterized by moderate-to-massive splenomegaly, esophageal varices, a patent spleno-portal axis, normal LFTs, and the absence of cirrhosis. Various studies have reported a high incidence of NCPF in India, with rates ranging from 20% to 45% [3]. Patients with NCPF typically present with variceal upper gastrointestinal (UGI) bleeding and symptoms of splenomegaly or hypersplenism, including pain, transfusion-dependent anemia, and splenic infarction [4]. Pal et al. [5] reported a morbidity rate of 47% following shunt surgery in a retrospective study of 45 patients with NCPF, raising concerns about the appropriateness of shunt surgery as the primary treatment for those without a history of major UGI bleeding. Saluja et al. [6] demonstrated that splenectomy could be an alternative to shunt surgery for non-bleeder NCPF patients and might be effectively combined with endoscopic therapy, particularly in cases with high-risk varices. Notably, endotherapy requires regular, long-term follow-up and fails to address the underlying issues of symptomatic hypersplenism or splenomegaly, which negatively impact quality of life. Therefore, combining splenectomy (with or without gastric devascularization) with endotherapy offers a viable management option for NCPF patients without a history of UGI bleeding or those with endoscopically controlled esophageal varices.

The indications for minimally invasive splenectomy (MIS) are expanding, encompassing both benign and malignant hematologic disorders and cases of extensive splenomegaly (i.e., diameter > 20 cm) [7]. Compared to traditional open surgery, MIS offers numerous benefits, including diminished intraoperative blood loss, accelerated recovery, and shortened hospital stays. PHTN is considered a relative contraindication for MIS according to clinical practice guidelines due to the heightened risk of blood loss from collateral vessels [8]. Recent studies have explored the possibility of utilizing MIS in patients with cirrhosis-related PHTN [8,9], and case reports on the use of MIS for NCPF are emerging [10]. Technological advancements in laparoscopy and robotics have facilitated the implementation of minimally invasive techniques for both splenectomy and esophagogastric devascularization. We have thus examined the outcomes of MIS in patients with NCPF, addressing the associated challenges, tips, and surgical techniques.

MATERIALS AND METHODS

Study design

This study was a single-center, retrospective cohort study utilizing data from a prospectively maintained database. It included all patients with clinical, radiologic, and final pathologic confirmation of NCPF who were non-bleeders and underwent MIS from 1st November 2017 to 31st December 2023. The study adhered to the Declaration of Helsinki and received approval from the Institutional Ethics Committee (AIIMS/IEC/5494). Additionally, it was conducted in line with the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) guidelines [11].

Case definition and patient work-up

The diagnostic criteria for NCPF encompassed symptoms of upper or lower gastrointestinal bleeding (hematemesis, melena, or rectal bleeding) and/or signs of splenomegaly (left upper quadrant pain, lump awareness), with or without hypersplenism. Hypersplenism might manifest as haemolytic jaundice, bleeding gums, epistaxis, or menorrhagia. Non-bleeder status was determined by the absence of clinically significant major UGI bleeding, defined by UGI bleed coupled with postural hypotension, the necessity for blood transfusion, or hospitalization.

Routine blood tests performed for all patients included complete blood count, LFTs, renal function tests, and coagulation profile, along with screening for seropositivity for hepatotropic viruses (HBV, HCV). Diagnostic confirmation of NCPF was achieved using upper gastrointestinal endoscopy (UGIE), transient elastography, splenoportal-axis (SPA) Doppler ultrasound, and contrast-enhanced computed tomography (CECT). SPA Doppler ultrasound verified portal vein (PV) patency, documenting the peak systolic velocity of the PV. CECT was used to identify NCPF characteristics such as a dilated main PV, pruning of peripheral PV branches, and the absence of radiological signs of liver cirrhosis. Additional diagnostic features included extensive splenomegaly and numerous portosystemic collaterals in perisplenic, perigastric, and anterior abdominal wall regions as shown in Fig. 1A.

Fig. 1.

(A) A 41-year-old lady presented with symptomatic hypersplenism and symptomatic splenomegaly. The contrast-enhanced computed tomography (CECT) image shows features of non-cirrhotic portal fibrosis. An asterisk marks the dilated main portal vein (PV), pruning of peripheral PV branches (arrows), and absence of radiological signs of liver cirrhosis. Also noted are extensive splenomegaly and multiple portosystemic collaterals in the perisplenic and perigastric regions (arrowhead). (B) CECT image showing massive splenomegaly with a splenunculus (arrow). A thorough review of preoperative CECT imaging is pivotal for identifying any splenunculus (accessory spleen).

Liver stiffness measurements (LSMs) using transient elastography were conducted to exclude cirrhosis or advanced hepatic fibrosis. All patients underwent a preoperative UGIE; those with high-grade esophageal varices or red-color sign positive varices received prophylactic endoscopic band ligation. Esophageal varices were graded according to the Japanese Research Society for Portal Hypertension classification [12], while gastric varices were graded as per the Sarin classification [13]. Surgical intervention was indicated for histories of UGI bleeding, symptomatic hypersplenism, or symptomatic splenomegaly, with pneumococcal, Haemophilus influenzae type B, and meningococcal vaccinations administered preoperatively or two weeks post-splenectomy.

Preoperatively, packed red blood cell (PRBC) transfusions were only administered to patients with hemoglobin levels below 7 g/dL. No preoperative platelet transfusions were performed. Intraoperatively, a complete blood count was conducted one hour after ligation of the splenic artery, targeting a hemoglobin level of 7 g/dL for patients without cardio-respiratory comorbidities and 8 g/dL for those with pre-existing conditions.

Surgical management

Patients underwent either robotic or laparoscopic splenectomy, with or without gastric devascularization and intraoperative liver biopsy, based on the surgeon’s discretion. In cases involving endoscopically managed esophagogastric varices, splenectomy alone was conducted. Patients with significant gastric varices underwent combined splenectomy and gastric devascularization to address the variceal burden.

Surgical technique of MIS

Laparoscopic approach (Supplementary Video 1)

The patient was placed in a split-leg, supine position with a 30-degree reverse Trendelenburg tilt. A 10-mm camera port was inserted at the umbilicus; for cases with massive splenomegaly extending to the umbilicus, the camera port was positioned to the right to prevent puncturing the splenic parenchyma. Two 5-mm working ports were located along the mid-clavicular line: one at the subcostal margin and the other 2 cm above the umbilicus. (Fig. 2A) In patients with abdominal wall varices, transillumination through the camera port coupled with preoperative CECT assists in identifying abdominal wall collaterals, ensuring safe port placement (Fig. 3). The MIS technique adheres to standard principles of splenectomy, with a focus on meticulous dissection and haemostatic control. The procedure commences with the division of the gastrocolic ligament, followed by the sealing and division of the short-gastric vessels using a bipolar vessel sealing device. The lesser sac is then opened, and the tortuous splenic artery is dissected along the superior border of the pancreas and doubly clipped. Clipping the artery in continuity reduces the spleen size and allows for autotransfusion. Subsequently, the splenocolic ligament is divided, and the splenic flexure of the colon is mobilized along an avascular plane. A thin layer of tissue remains over the splenic capsule to facilitate counter-traction during dissection. The approach to the splenic hilum includes dividing the anterior peritoneal reflection and the associated fibrofatty tissue. Careful dissection of the pancreatic tail from the hilum is necessary to prevent pancreatic injury and potential fistula formation. The enlarged splenic vein is looped and divided using a white vascular-load endoscopic stapler, ensuring that no haemostatic clips are included within the staple line. Additional hilar collaterals are divided using either the stapler or bipolar vessel-sealing device as necessary. For patients with extensive splenomegaly, a “splenic hanging maneuver” is utilized to enhance dissection. The assistant provides upward counter-traction by placing an instrument beneath the spleen against the lateral abdominal wall, moving cranially as dissection progresses. The spleno-phrenic ligament is the final structure divided, which completes the spleen's mobilization. The assistant retracts the upper pole laterally to facilitate this final step.

Fig. 2.

Intraoperative images: (A) displays laparoscopic splenectomy port positions. Trocar locations are as follows: C1: 10-mm camera port, W1 and W2: working ports, A1: assistant working port; (B) illustrates robotic splenectomy port positions with four robotic ports (R1 to R4) and one assistant port A1.

Fig. 3.

(A) Contrast-enhanced computed tomography coronal maximum-intensity-projection image of a 25-year-old lady with non-cirrhotic portal fibrosis, displaying dilated anterior abdominal wall collaterals. The asterisk (*) indicates the umbilicus. (B) Intraoperative image of the same patient showing anterior abdominal wall transillumination through the camera port to mark the wall collaterals.

Gastric devascularization is performed when prominent gastric varices are identified preoperatively. Beginning along the lesser curvature of the proximal stomach, the procedure utilizes the same ports. Small branches of the left gastric vessels are divided close to the gastric wall to prevent a truncal vagotomy. Vascular control of the left gastric vein is achieved using vascular clips or a bipolar device. Posterior gastric vessels and collateral veins are managed with clips, a bipolar device, or a linear stapler, depending on their size. Sutures reinforce the seromuscular layer over large varices to mitigate the risk of delayed perforation.

Preoperative imaging is meticulously reviewed to identify and remove any splenunculus, as depicted in Fig. 1B. A core-needle biopsy of the liver is routinely performed to confirm the diagnosis. The spleen specimen is extracted through a Pfannenstiel incision.

Robotic approach (Supplementary Video 2)

The robotic splenectomy utilizes the da Vinci Xi platform (Intuitive) equipped with three robotic arms and a 10-mm assistant port. The patient is placed supine with a 45-degree reverse Trendelenburg tilt, and the robot is docked from the patient’s right side. Port positions are demonstrated in Fig. 2B. The camera is positioned in the infraumbilical 8-mm R3 port. After precisely assessing the abdominal wall collaterals, the remaining ports are placed. R2 is positioned 8cm to the right of R3 and R1 is set 8 cm further to the right and 2 cm above R2. R4 is positioned 10cm to the left of R3. A 12 mm assistant port is positioned 8 cm inferior to R3. Instruments include cadiere forceps in R1, fenestrated bipolar forceps in R2, and monopolar scissors with a vessel sealer in R4. This robotic splenectomy follows the steps outlined for laparoscopic splenectomy. The spleen is retracted upwardly using the R1 instrument against the lateral abdominal wall during the robotic hanging maneuver.

Outcome parameters and follow-up

Intraoperative data such as blood loss, operation duration, and incidences of conversion to open surgery were diligently recorded. Postoperative outcomes including complications and hospital stay durations were documented. Postoperative monitoring was conducted to evaluate recovery and symptom resolution. Complications were clinically assessed and, when necessary, confirmed radiologically and classified using the Clavien-Dindo system. The length of the hospital stay was noted.

Aspirin (75 mg) was administered daily to all patients undergoing MIS for NCPF from postoperative day (POD)-1 for a minimum of six months. Patients with platelet counts exceeding 400,000/microliter during follow-up were provided with extended-duration aspirin therapy. Routine follow-up included assessments of esophagogastric varices through UGIE every three months in the first year, biannually in the second year, and annually thereafter. SPA Doppler ultrasound, performed at 12 months post-MIS, measured the peak systolic PV velocity. Long-term outcomes such as PHTN resolution, last follow-up date, and clinical status were also documented.

Data analysis

All data were entered using Microsoft Excel 2010 (Microsoft). Statistical analyses were performed using SPSS version 21.0 (IBM Corp.). Continuous variables are presented as mean ± standard deviation (SD) or median with interquartile range (IQR), as appropriate. The data for pre- and post-splenectomy at the 12-month follow-up were compared using either the Student’s t-test or the Wilcoxon signed-rank test, depending on the distribution of the data. A p-value of less than 0.05 was considered statistically significant.

RESULTS

During the study period, thirteen patients underwent MIS for NCPF. The primary indication for surgery was symptomatic splenomegaly in every case, while symptomatic hypersplenism was observed in 85% (11 out of 13) of the patients. Of these thirteen patients, eight underwent laparoscopic splenectomy, four had robotic splenectomy, and one required conversion from laparoscopic to open splenectomy due to dense intra-abdominal adhesions resulting from previously infected ascites. Two patients underwent laparoscopic gastric devascularization along with splenectomy, and one patient underwent the robotic equivalent. The median (IQR) LSM value on preoperative transient elastography was 7.2 kPa (5.5–8.2 kPa), and the median spleen size based on preoperative imaging was 21.5 cm (IQR: 20.1–23.9 cm). The baseline characteristics are detailed in Table 1.

Table 1.

Showing baseline patient data along with postoperative 90-day morbidity and outcomes at 12-months following surgery

Patient	Age/sex	Preoperative						Procedure	90-day morbidity	Clavien-Dindo grade	12-month follow-up
		Hemoglobin (g/dL)	Platelet count (×103/mm3)	TLC (per mm3)	Preoperative imaging spleen size (cm)	UGI variceal grade	SPA Doppler PV PSV (cm/sec)				Hemoglobin (g/dL)	Platelet count (×103/mm3)	TLC (per mm3)	UGI variceal grade	SPA Doppler PV PSV (cm/sec)
1	34/F	6.8	24	1,200	22.7	2	19	Robotic splenectomy	None	0	10.6	479	11,700	1	24
2	30/M	9.1	69	4,500	20.0	2, gastric varices	16	Lap splenectomy + devascularization	Discharged with drain (high output)	I	11.2	287	9,000	0	26
3	45/F	8.6	50	2,900	20.5	1	16	Lap splenectomy	None	0	11.2	212	8,500	0	32
4	25/F	8.8	55	3,500	25.1	2	20	Lap splenectomy	Acute splanchnic venous thrombosis on POD-18	II	12.0	416	11,200	1	28
5	41/F	8.6	43	4,000	21.0	1	22	Robotic splenectomy	None	0	9.3	234	9,600	0	26
6	46/F	9.3	74	5,000	20.0	2, gastric varices	12	Lap splenectomy + devascularization	Discharged with drain (high output)	I	10.6	263	10,800	0	24
7	51/F	10	90	5,500	23.6	2	23	Robotic splenectomy	None	0	13.0	463	12,900	1	28
8	21/F	8	51	2,900	24.0	2	14	Robotic splenectomy	None	0	12.6	641	12,500	1	32
9	41/F	9.6	70	2,000	20.6	2	14	Lap splenectomy	None	0	8.5	256	10,200	1	26
10	27/F	7.4	39	1,600	20.0	1	25	Lap splenectomy	None	0	8.6	287	11,000	0	28
11	30/F	7.7	45	2,500	24.0	2	14	Lap splenectomy	None	0	13.5	435	13,000	1	24
12	44/F	7.2	28	1,400	22.0	1	21	Lap converted to open splenectomy	Discharged with drain (high output)	I	8.8	114	9,000	0	30
13	21/F	8.7	60	3,000	22.5	2, gastric varices	18	Robotic splenectomy + devascularization	None	0	11.0	300	10,000	0	32

F, female; TLC, total leucocyte count; UGI, upper gastrointestinal; SPA, splenoportal axis; PV, portal vein; PSV, peak systolic velocity; POD, postoperative day.

Perioperative outcomes

The median total operative time was 240 minutes (IQR: 180–300 minutes), and the median estimated blood loss during the operation was 150 mL (IQR: 100–250 mL). One patient required an intraoperative single-unit PRBC transfusion. One of the thirteen patients (7.7%) underwent conversion to open surgery, necessitated by the non-progression of laparoscopic splenectomy due to dense intra-abdominal adhesions following a prior episode of UGI bleed that was complicated by infected ascites.

The median length of hospital stay post-surgery was 3 days (IQR: 3–5 days). The median time for drain removal was 3 days (IQR: 3–22 days), and three patients were discharged with a drain due to high ascitic fluid output; these were removed during a follow-up visit.

One patient was readmitted on POD-18 with acute splanchnic venous thrombosis (SVT). She presented with abdominal pain, and a CECT scan revealed an acute thrombus in the main PV, extending to the superior mesenteric and splenic veins, as shown in Fig. 4A. Initially, she was treated with therapeutic doses of low-molecular-weight heparin (enoxaparin) followed by oral direct thrombin antagonist: dabigatran (150-mg twice daily). Complete radiological resolution of the thrombosis was achieved after 6 months of oral anticoagulation (Fig. 4B), with no subsequent clinical complications. The oral anticoagulation was continued for an additional 6 months after documenting the complete resolution of the SVT on CECT. Overall, three patients developed CD grade I complications, and one had a CD grade II complication. There was no 90-day mortality.

Fig. 4.

A 25-year-old lady diagnosed with non-cirrhotic portal fibrosis with symptomatic splenomegaly and hypersplenism underwent laparoscopic splenectomy. She was discharged home on postoperative day (POD)-3 on oral aspirin therapy (75-mg once daily). She was readmitted on POD-18 with abdominal pain. (A) A Contrast-enhanced computed tomography (CECT) scan in the coronal plane revealing acute thrombosis (asterisk) in the main portal vein, extending into the superior mesenteric vein and the splenic vein. Initially, she was treated with therapeutic doses of low-molecular-weight heparin (enoxaparin), followed by an oral direct thrombin antagonist: dabigatran (150 mg twice daily). (B) A repeat CECT scan at the 6-month follow-up showed normal opacification of the main portal vein and the superior mesenteric vein. She achieved complete radiological resolution of the thrombosis after 6 months of oral anticoagulation without any subsequent clinical complications. Oral anticoagulation was continued for an additional 6 months after documenting the complete resolution of the splanchnic venous thrombosis on CECT.

The final histopathological diagnosis concurred with congestive splenomegaly. Notable changes in the PV and surrounding portal tracts on core-needle liver biopsies included PV thickening in 9 out of 13 biopsies, thrombosis in 2, narrowing of the lumen in 4, PV dilation in 7, and peri-portal aberrant vascular channels in 7. Architectural disarray was noted in 5 out of 13 biopsies, yet none exhibited histology activity index stage greater than 2 fibrosis or cirrhotic changes.

Long-term outcomes

The median follow-up duration was 16 months (IQR: 12–43 months). Significant improvements were observed in all blood parameters by the 12-month follow-up. The median (IQR) values for hemoglobin, leukocyte count, and platelet count significantly increased from 8.6 g/dL (7–9.3 g/dL), 2.9 (1.7–4.4 K), and 53.2 K (24–90 K) to 10.9 g/dL (8.9–12.5 g/dL), 10.9 K (9.1–12.3 K), and 287 K (239.5–456 K) at 12 months post-MIS (p < 0.01, Wilcoxon signed-rank test).

The median grade of esophageal varices decreased from grade 2 (1–2) preoperatively to grade 1 (0–1) at 12 months post-MIS (p < 0.01, Wilcoxon signed-rank test). The three patients who underwent gastric devascularization (two laparoscopic and one robotic) exhibited complete obliteration of gastric varices on follow-up UGIE. None of the patients experienced recurrent variceal bleeding. SPA Doppler ultrasonography showed that the mean (SD) peak systolic velocity of the PV increased from 18 ± 4.2 cm/sec preoperatively to 27.7 ± 3.0 cm/sec at follow-up (p < 0.01, Student’s t-test), indicating a reduction in PHTN. Furthermore, no cases of post-splenectomy infection were reported.

DISCUSSION

Based on our analysis of the study, we observe that MIS, with or without gastric devascularization, is feasible, safe, and effective in reducing PHTN and alleviating hypersplenism in non-bleeder patients with NCPF. Despite substantial splenomegaly and elevated PHTN, more than ninety percent of cases were successfully performed using a minimally invasive approach. MIS in this patient cohort was associated with low perioperative morbidity and favorable short-term and long-term outcomes.

With the advancements in minimally invasive approaches (laparoscopic and robotic), MIS for moderate-to-massive splenomegaly has become increasingly common [14]. First described by Delaitre et al. [15] in 1992, MIS has shown several advantages over the traditional open approach, including shorter postoperative hospital stays, reduced blood loss, and improved cosmetic outcomes [16]. However, the use of MIS in cases with PHTN remains limited. NCPF presents a distinct challenge, featuring an enlarged spleen combined with PHTN, which increases technical difficulties and requires advanced surgical skills and excellent teamwork due to the restricted operative field and increased risk of operative hemorrhage. Additionally, extensive fibrous adhesions on the surfaces of massive splenomegaly, and previous endoscopic glue injections, induce inflammatory changes around the gastric fundus. Standardizing surgical steps for this complex procedure in this specific patient subset will promote wider acceptance within the surgical community. In this study, all cases involved PHTN accompanied by massive splenomegaly, with a median spleen size of 21.5 cm noted on preoperative imaging. We found the anterior approach adequate for MIS in all cases, as the hilum was displaced forward, downward, and inward by the enlarged spleen. Gadiyaram et al. [17] also reported that the anterior lienorenal approach is safe and effective in patients with massive splenomegaly. Moreover, early ligation of the splenic artery facilitated autotransfusion and reduced the spleen size, easing subsequent splenic mobilization [18]. We documented a median operative time of 240 minutes and an estimated blood loss of 150 mL, with one patient requiring a blood transfusion. This demonstrates the feasibility of MIS for NCPF patients with massive splenomegaly and PHTN. We have detailed the critical surgical steps for MIS in NCPF patients. The placement of the camera port and subsequent ports must be tailored based on spleen size and the location of abdominal wall collaterals. Preoperative CECT imaging and intraoperative abdominal wall transillumination assist in marking wall collaterals and guiding port placement. The adoption of advanced energy devices, such as harmonic scalpels or bipolar vessel sealing devices, has enhanced the feasibility of MIS and contributed to reduced blood loss. The use of vascular staplers near the splenic hilum to divide the splenic vein and/or collateral vessels further facilitates the procedure. The splenic hanging maneuver described in our study (Supplementary Video 1, 2) provides effective counter-traction during the mobilization of large spleens, which is crucial for facilitating dissection and mobilization, particularly in cases of massive splenomegaly. It is essential to remain close to the splenic capsule to avoid large splenorenal collaterals and prevent unnecessary intraoperative bleeding. However, it is advisable to leave some fibrofatty tissue attached to the splenic capsule to aid traction during MIS. Retrieving large spleens through a Pfannenstiel incision is not only cosmetically superior but also associated with reduced postoperative pain, as noted in the scientific literature [19]. We avoid morcellating the spleen due to the inherent risk of spleen rupture and subsequent splenosis [20]. It is often challenging to bag massive spleens during MIS; therefore, they are delivered using controlled traction and counter-traction (Supplementary Video 1). Avoiding large upper abdominal incisions in MIS results in less postoperative pain, a quicker return to daily activities in the short term, and in the long term, better preservation of abdominal wall function, improved cosmesis, and a lower risk of future incisional hernias, especially in these young patients with a normal life expectancy [21]. We did not encounter any pulmonary complications postoperatively, which may be attributed to avoiding long upper abdominal incisions, a primary cause of postoperative pain and related pulmonary morbidity.

Our study demonstrated favorable postoperative outcomes, with a median hospital stay of 3 days and no major complications in most patients. Notably, one patient developed acute SVT, a recognized complication following splenectomy for massive splenomegaly [22,23]. This patient was successfully managed with anticoagulation therapy, underscoring the importance of close postoperative monitoring for vascular complications. Given the high risk of developing SVT in cases with massive splenomegaly and PHTN, we have revised our protocol and now prescribe the oral direct thrombin inhibitor dabigatran at 75-mg twice daily to all such patients post-MIS for a period of 6 months. This practice is substantiated by evidence from several studies [24-26]. The long-term outcomes of our cohort are promising. Significant improvements in hematological parameters, including hemoglobin, leukocyte, and platelet counts, were observed at the 12-month follow-up. These results indicate the resolution of hypersplenism. The noted reduction in the grade of esophageal varices and obliteration of gastric varices in patients undergoing gastric devascularization underscores the efficacy of MIS in alleviating PHTN. Doppler studies further confirmed improvements in PV hemodynamics, providing objective evidence of ameliorated PHTN. Importantly, there were no instances of recurrent variceal bleeding, reinforcing the durability of the surgical outcomes and the safety of the procedure. The effects of splenectomy alone on reducing PHTN in non-bleeding NCPF patients have been demonstrated in several other studies [6,27]. Histopathological analysis provided crucial insights into the vascular changes associated with NCPF. Findings such as PV thickening, thrombosis, and aberrant vascular channels aligned with the underlying pathophysiology of NCPF [28]. Importantly, no cases showed cirrhotic changes, further validating the non-cirrhotic etiology of the condition.

Despite our encouraging results, surgeons attempting MIS should be aware that cases of PHTN may exhibit significant bleeding from collateral vessels. A low threshold for converting to an open approach should be maintained, ensuring patient safety as a priority over persistence with MIS during major intraoperative bleeding. We acknowledge that performing MIS in cases of massive splenomegaly with PHTN demands specialized skills and involves a learning curve [29]. Our study involved surgeons with over 10 years of experience in minimal-access surgery. We observed that one case required conversion to open surgery due to intra-abdominal adhesions resulting from prior infected ascites. This study is retrospective and small in scale, thus limiting the generalizability of our findings. The study’s single-center design could also introduce a risk of selection bias. Future multicentre prospective studies with larger samples are necessary to validate our findings and refine the indications for MIS in NCPF.

In conclusion, this study demonstrated the feasibility and safety of MIS in a challenging cohort of NCPF patients with massive splenomegaly, performed carefully by experienced surgeons. To the best of our knowledge, this is the first study examining the feasibility and long-term outcomes of MIS in non-bleeder NCPF patients. Detailed descriptions of surgical techniques such as early splenic artery ligation and the splenic hanging maneuver provide valuable insights for MIS in similar cases. The favorable outcomes, evidenced by improved hematological parameters, grades of esophageal varices, obliterated gastric varices, and enhanced PV hemodynamics, underscore the efficacy of the MIS approach. By offering practical solutions for overcoming specific technical challenges in NCPF, we aim to advance the role of MIS in the management of these patients.

SUPPLEMENTARY DATA

Supplementary data related to this article can be found at https://doi.org/10.14701/ahbps.25-033.

Supplementary Video 1. Illustrates salient surgical steps of laparoscopic splenectomy in a patient with non-cirrhotic portal fibrosis.

Supplementary Video 2. Illustrates salient surgical steps of robotic splenectomy in a patient with non-cirrhotic portal fibrosis.

Funding Statement

FUNDING None.