Single port laparoscopic splenectomy for wandering spleen with splenomegaly in a patient with Wolf-Hirschhorn syndrome.

Wolf-Hirschhorn syndrome is a rare genetic condition characterized by typical facial appearance, growth delay, psychomotor retardation and seizures, with a mosaic of other abnormalities reported in the literature. The occurrence of symptomatic wandering spleen with massive splenomegaly and with an indication for splenectomy has not been yet described for this disease. This study reports the first case in the literature of single port splenectomy for this rare condition. In a 21-year-old female patient with Wolf-Hirschhorn syndrome, with abdominal pain and the diagnosis of wandering spleen with splenomegaly (25 cm diameter) led to an indication of elective splenectomy. In supine position under general anesthesia, single port umbilical splenectomy was performed without laparoscopic assistance, splenic vessels were ligated by sutures, and the specimen was transumbilically extracted. Operative time was 85 min, with minimal bleeding, and resumed oral intake on the same day. No intraoperative or post-operative complications occurred, and the patient was discharged in 48 h. Single port access splenectomy is feasible and is evolving as an attractive alternative therapy for hematological diseases requiring splenectomy.

INTRODUCTION

Laparoscopic splenectomy was first reported by Delaitre and Maignien in 1991 and soon became a procedure adopted as golden standard for most indications for splenectomy for haematologic diseases.[1] Advantages of laparoscopic splenectomy compared to the open procedure were demonstrated by many studies, including benefits as decreased blood loss, shorter hospital stay, rapid recovery and good cosmetic results.[234] Current efforts of the surgical community are to reduce even more the surgical trauma of minimally invasive procedures, and the single port approach has been proposed for several intra-abdominal surgical interventions.[5] Splenectomy represents a more complex procedure and shows specific problems for techniques as a single port, due to its volume and texture, and little clinical experience is available on the topic.

Wolf–Hirschhorn syndrome is a rare genetic condition characterized by typical facial appearance, growth delay, psychomotor retardation and seizures, and is confirmed by a deletion of the Wolf-Hirschhorn critical region (WHCR) in chromosome 4p16.3.[6] Splenomegaly and wandering spleen with a surgical indication for splenectomy in patients with the syndrome was not yet recorded in the literature. This study describes the first clinical application and technical aspects of single port access surgery for splenectomy for this disease.

CASE REPORT

A 21-year-old female patient with typical facial appearance, growth delay, psychomotor retardation and seizures was diagnosed as Wolf-Hirschhorn syndrome (delection of WHCR critical region, chromosome 4p16.3) within the 1st year after birth. The typical facial appearance of the patient included the ‘Greek warrior helmet appearance’ of the nose, high forehead, ocular hypertelorism, downturned mouth, micrognatia and small ears. The patient was submitted to three facial operations for leporine lip (buccal fissure) at 6 months, at 1 and 1½ years. There were no cardiac nor renal malformations, and the patient had regular menses. A large thyroid cyst (8 cm) was punctioned pre-operatively and showed no evidence of malignancy.

The patient presented diffuse abdominal pain for 6 months, and consecutive abdominal computed tomography scans showed change of splenic status and location during this period, with growing of two large splenic cysts (10 cm and 9 cm), and wandering splenomegaly, with a spleen size of 25 cm with mainly pelvic location [Figure 1]. Body mass index of the patient was 26.6 kg/m2, and pre-operative evaluation was classified as American Society of Anaesthesiologists I. White blood cell and platelet levels were at normal range, and no signs of anaemia were found in routine laboratorial examination. Elective splenectomy was indicated, and single port access surgery was offered, when potential benefits and disadvantages of the method were explained. Because of the nature of mental illness of the patient, the parents of the patient signed an informed consent for participating in the study. The patient received immunization for pneumococcus and Haemophilus influenzae 15 days before the procedure.

Figure 1

Pre-operative tomographic aspect of wandering spleen with splenomegaly in a pelvic location. Detail of the long splenic hilar vessels

Surgical technique

Under general anaesthesia, the patient was placed in supine position, with a 20° reverse-Trendelenburg inclination. A lateral position was not chosen, as the spleen had a full pelvic position with a long vascular supply. Antibiotic prophylaxis with 2 g cephalexin was given at the induction of anaesthesy. The operator was situated at the right side of the patient, and the first assistant was managing the camera at the left side of the patient, with the laparoscopic tower situated at the left shoulder of the patient. A 3 cm-long intraumbilical incision was performed, and the abdomen was entered with open technique, without previous insufflation. A single port access device (Triport, Olympus, Japan) with three working channels (10 mm, 5 mm, 5 mm), was introduced through the incision, and pneumoperitoneum with 14 mm of CO2 was gradually insufflated [Figure 2]. Right hand instruments used were straight laparoscopic monopolar scissors, straight Maryland graspers and ultrasonic shears. Left hand instrument was a straight atraumatic grasper. A 30°, 10 mm laparoscopic camera was used in the larger channel of the port.

Figure 2

Installation of single port device intra-abdominally using open technique. Position of instruments for splenectomy

The spleen was localized occupying the pelvis, and was fully mobilized to a left lateral position for vascular approach by careful manipulation with graspers. Monopolar dissection was performed to separate arterial and venous vessels, and double proximal, external tied polypropylene 0 sutures were applied individually [Figure 3]. Distal ligation by the same method was possible only after section of polar vessels using ultrasonic coagulation (Ultracision Ace, Ethicon Endosurgery, Cincinatti OH). Percutaneous aspiration of the cysts content (about 800 ml) allowed extraction of the whole spleen transumbilically without use of an extraction bag [Video 1]. After closure of the fascial defect, cosmetic aspect was satisfactory [Figure 4].

Figure 3

Ligature of splenic vessels using external tied polypropylene sutures individually applied

Figure 4

Post-operative aspect of transumbilical splenectomy

The single port system used allowed confortable manipulation of the straight instruments, with few collisions or limitations. In this case, because of the characteristics of wandering spleen, there was no need for dissection of lateral attachments. Difficult access for dissection was found with the spleen in the pelvic location, but it changed after mobilizing the organ to a left lateral position. Operative time was 85 min, and 8 min were necessary for the abdominal opening and insertion of the device, and 12 min necessary for organ extraction and closure. Operative bleeding was minimal (<50 ml). The patient was offered liquid diet, and resumed oral intake on the same day, 2 h after the procedure without occurrence of nausea or vomiting. The post-operative course was uneventful and the patient was discharged in 48 h without medications. Histopathological examination of the specimen showed 2 large cysts (9 cm and 8 cm diameter) with no malignancy. No complications were documented until follow-up consultation at 30 days post-operatively.

DISCUSSION

Wolf–Hirschhorn syndrome is characterized by typical facial features, post-natal growth retardation and mental retardation, seizures and feeding difficulties in more than 75% of the patients.[67] All affected individuals have prenatal grown deficiency followed by post-natal growth retardation and muscle under-development. Development delay or mental retardation of variable degree is present in all. Other findings are skeletal abnormalities, craniofacial asymmetry, abnormal teeth, antibody deficiency (50-75%), hearing defects, heart defects, eye or optic nerve defects, brain abnormalities and stereotypes (25-50%) and abnormalities for liver, gallbladder, gut, diaphragm, oesophagus, lungs and aorta (<25%). Surgical indications for splenectomy and wandering spleen in Wolf-Hirschorn syndrome patients were not reported yet in the literature.

Current laparoscopic techniques require several skin incisions (3 or 4 for splenectomy), meanwhile single port access surgery allows performing the same procedure by a single intraumbilical wound that produces one almost invisible scar. The major limitation of these techniques is the lack of triangulation to which laparoscopic surgeons are accustomed. The Triport device allowed the working of two instruments parallel to the camera, and was confortable for splenectomy in the reported case, with few collisions between instruments, and with a reliable anatomical visualization.

Single port access splenectomy was described by Targarona et al. performing two cases in 2008 for immune thrombocytopenic purpura and Hodgkin disease.[8] In one case, three trocars inserted transumbilically were used, in the other case the ports were placed in one subcostal single wound. In recent series by the same authors, eight cases were described without complications,[9] mostly using the same device, but conversion to traditional laparoscopy was needed in two cases due to adhesions and splenomegaly. Podolsky and Curcillo published a series of cases of single access techniques, including adrenalectomy, splenectomy and cholecystectomy.[5] They performed the operations using standard laparoscopic trocars inserted transumbilically with different fascial orifices.

In the case described in this study, the situation of a wandering spleen facilitated the surgical technique, as the splenic vessels were very long, and there were no lateral attachments to the abdominal wall. Use of the Triport system was very adequate for this case, and there was no need for articulated instruments, neither for additional trocars. In our view, ligature of the vessels using unabsorbable sutures is the most efficient method for a safe vessel sealing. Single port access surgery can be comfortably used for operative visualization, hilar transection and spleen removal, thus reducing parietal trauma and obtaining a scarless result. Further studies are suggested to evaluate the role of single access techniques and proper indications in comparison to standard techniques. The incidence of cystic splenomegaly and wandering spleen for Wolf-Hirschhorn syndrome has to be evaluated in further case series.

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Nil.

Conflicts of interest

There are no conflicts of interest.