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 .
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 .
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 .
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 .
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 .
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 .
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 1 year after birth .
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 1 years .
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 / m , 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 .
detail of the long splenic hilar vessels 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 .
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 .
a 30 , 10 mm laparoscopic camera was used in the larger channel of the port .
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 ] .
ligature of splenic vessels using external tied polypropylene sutures individually applied 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 .
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 .
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 .
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 .
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 ] .
ligature of splenic vessels using external tied polypropylene sutures individually applied 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 .
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 .
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 .
all affected individuals have prenatal grown deficiency followed by post - natal growth retardation and muscle under - development .
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 . 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 , 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 .
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 .
 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
CASE REPORT
Surgical technique
DISCUSSION
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