Splenectomy for Klippel-Trenaunay syndrome: Systematic review and case series
Year: 2024
Type: review
Abstract: Dear Editor, Klippel-Trenaunay syndrome (KTS) is a rare form of vascular malformation disorder characterized by the appearance of slow-flow vascular malformations with limb overgrowth.[1] So far, there are very few reports on splenic lesions presenting concomitant with KTS in the literature. We report our institution's experience about KTS with splenic abnormality managed with open splenectomy (OS), and further provide a systematic review of relevant literature. Four cases were retrieved from China Medical University Hospital (CMUH) from 1983 to February 2023. All patients possessed signs of classical KTS, which included unilateral lower limb overgrowth, lymphatic malformations, venous malformations, and port-wine stains (Fig. 1). All had splenic abnormalities noted on imaging (see Fig. 2). In terms of surgical indications, all cases presented with nonspecific gastrointestinal symptoms (see Table 1). They received prophylactic vaccination for Overwhelming Post-Splenectomy Infection (OPSI) and postoperative antibiotic as prophylaxis. All cases had good clinical outcome on follow-up.Figure 1: Representative case of lymphedema from our KTS patient series. (A) and (B) both are images of lower extremity lymphoedema from our case 4 KTS patient.Figure 2: CT scans from cases 1–4. (a) CT from case 1 patient with KTS showing multiple hypodense nodules in spleen. (b) CT from case 2 showing similar findings of hypodense nodules in spleen. (c) CT from case 3 showing similar findings of hypodense nodules in spleen. (d) CT from case number 4 patient showing similar enlargement and heterogeneity of splenic parenchyma. Table 1 - Characteristics of KTS Patients With Splenic Lesions Treated With Open Splenectomy in our Department Case 1 Case 2 Case 3 Case 4 Sex Female Female Female Male Age (y) 24 11 12 4 Indication for splenectomy Persistent abdominal symptoms Persistent abdominal symptoms Persistent abdominal symptoms Persistent abdominal symptoms Method of splenectomy OS OS OS OS Preoperative prophylaxis antibiotics received − − − − Preoperative vaccinations to prevent OPSI PCV 13 PCV13, PSV23 PCV13 PCV 13, Preoperative platelets (103/μL) 261 336 263 307 Pathology of removed spleen Size 14 × 11 × 5 cm 17 × 12.5 × 8 cm 26 × 15 × 9 cm 8 × 6 × 4 cm Finding Hemangiolymphangioma characterized by multicystic lesion composed with variable-sized lymphatic duct Lymphangioma characterized by multicystic lesion composed with variable-sized lymphatic duct Mixture of dilated lymphatic and venous channels extensively replacing the splenic parenchyma; focal infarct with organized change Diffuse dilated vascular spaces lined by flattened cells with luminal red blood cells; focal capsule defect Operative blood loss 250 30 20 50 Postoperative antibiotics + + + + Postoperative vaccinations PPSV23 PPSV23 PCV13, Menatra PPSV23 Menatra, Pediacel Early postoperative complications within 3 mo after splenectomy − − − + Total hospital length of stay (d) 5 85 240 180 Follow-up months 48 60 72 48 Long term follow-up Pneumonia − − − − Meningitis − − − − Other infection* − − − − Readmission − − − − Postoperative mortality − − − − Improvement of symptoms + + + + + means yes; − means no.*Other infection = post–splenectomy-induced infection, excluding skin infection resulted from ulcers with discharge.OS, open splenectomy; PCV 13, pneumococcal conjugate vaccine; PSV23, 23-valent polysaccharide vaccine. A systematic review was conducted based on the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement.[2] A modified version of the tool proposed by Murad et al. was used to assess for the risk of bias.[3] Interventional studies, case-control studies, case series, and case reports in English were included in our systematic review. Non-English articles, review articles, meta-analysis, systematic review, letters to the editor, and conference abstracts were excluded. A comprehensive literature search was performed via database including Scopus, Google Scholar, PubMed, and EMBASE from the earliest record in the literature to April 9, 2023. Search terminology consisted of “Klippel-Trenaunay syndrome,” “splenectomy,” “splenomegaly,” “splenic hemangioma,” or “splenic lymphangioma.” Finally, 10 articles published between 2006 and 2021 were identified for final inclusion (see Fig. 3). OS was performed in 5 cases (50%), whereas no specific surgical technique was specified in the other 5 cases (see Table 2). Four cases (40%) in the literature were reported to have some form of complications post-splenectomy. Venous thrombus was observed in 2 out of 4 cases, which were resolved by anticoagulants with or without inferior vena cava filter placement. Despite this, 90% of these patients recovered from their postoperative complications at subsequent follow-up and experienced resolution of presenting symptoms after splenectomy.Figure 3: PRISMA flowchart for the systematic review. Table 2 - Characteristics of the Patients From Included Articles Study Age/Sex Indication of Splenectomy Technique of Splenectomy Surgical Complications Symptoms Relieved After Surgery Histology Vaccinations for Surgery Total Hospital Length of stay (d) Follow-Up Months Jindal et al. {2006} 22, female Intermittent vomiting and left hypochondrium discomfort for 8 months Open − + Hemangiolymphangioma N/A 7 N/A Karakayali et al. {2010} 40, female Acute abdominal pain and hemodynamic instability Open Rectus sheath hematoma + Hemangioma rupture N/A 12 12 Lin et al. {2011} 40, female Abdominal fullness, poor appetite, intermittent ileus, and left hypochondrium discomfort for 5 years N/A − + Hemangiolymphangioma N/A 7 N/A Herman et al. {2012} 16, male Thrombocytopenia N/A Septic shock secondary to peritonitis, DVT + Hemangiolymphangioma N/A 6 12 Spasic et al. {2012} 30, female Occasional left hypochondrial pain for several months Open − + Hemangiolymphangioma N/A 21 N/A Dekeyzer et al. {2013} 45, female Chronic left hypochondrial pain N/A − + Hemangioma N/A 7 N/A Karunamurthy et al. {2013} 19, female N/A N/A Mortality N/A Hemangioma N/A 2 N/A Misawa et al. {2014} 40, female Severe dull abdominal pain Open Insignificant thrombi formed in the portal vein and the pulmonary vein + Hemangioma N/A 40 N/A Zhang et al. {2014} 29, female Noncirrhotic portal hypertension related splenomegaly, thrombocytopenia, and left-sided abdominal pain Open (splenic artery embolization prior to splenectomy) − + Hemangiolymphangioma Pneumococcal and hemophilus vaccines 5 6 Ma et al. {2021} 33, female Hematemesis for 1 years N/A − + Lymphangioma N/A N/A 3 DVT, deep venous thrombosis; N/A, nonavailable; + means yes; − means no. The decision to choose which method of splenectomy depends on various factors. KTS patients tend to present with large and multiple splenic lesions and associated splenic vascular and lymphatic malformations, which might explain why OS tends to be the primary choice among literature studies rather than laparoscopic splenectomy. Another point is the length of hospital stay. A related prospective study published in JAMA by Park et al. reported 63 non-KTS open splenectomy patients. Average length of stay post-OS was 9.2 days.[4] It should be noted that our increased length of stay compared to literature reflects the comorbidities with KTS rather than due to the complications of the splenectomy itself. In conclusion, our study adds to the evidence that OS is a viable treatment for KTS complicated with splenic lesions.
Source: Formosan Journal of Surgery
Language: English
Subfield: Surgery
Field: Medicine
Domain: Health Sciences
Open Access status: diamond
OpenAlex ID: W4402895518