Type-I complex regional pain syndrome of umbilical port site: An unforeseen complication of laparoscopic surgery

A 16-year-old male developed acute appendicitis for which laparoscopic appendectomy was done in another hospital. The telescope was 10 mm, which was passed via the 12-mm umbilical trocar inserted by open technique. The appendix was found to be inflamed with a fecolith and appendectomy was done in the usual way. Postoperative period was uneventful and patient was discharged on 5th postoperative day. The biopsy of the appendix was reported as chronic appendicitis. After 15 days, he developed pain in the umbilicus with no fever, vomiting, or pus discharge. Ultrasonogram abdomen showed no abnormality. Patient was treated with oral antibiotics and analgesics for 15 days. There was no response, and after 1 month umbilical exploration was planned assuming stitch abscess as the cause of pain. Exploration was done by a vertical incision around the umbilicus and no abscess was found, and the wound was closed with 1/0 Ethilon. After exploration, there was increase in the severity of umbilical pain and it was aggravated by touch. The pain was severe enough that he could not continue his school and often removes the clothing which causes pain due to rubbing over the umbilicus. The patient visited the operated surgeon 25 times in one and half year time. He was treated with oral antibiotics, NSAIDs, vitamins, and topical steroids and topical antibiotics. Once, the patient developed severe pain and fainted and became unconscious for 10 min. EEG and CT scan of brain were done, and both were found to be normal. The operated surgeon advised him to follow-up in our institute which is in close proximity to his residence and also to get psychiatrist opinion to rule out functional cause. The patient was submitted for psychiatrist, neurosurgeon, medical gastroenterologist, and cardiologist opinion before coming to our department. When we examined the patient, he was malnourished and the umbilicus was scarred with a vertical scar around it. The umbilicus was extremely tender, and the skin was damp and erythematous as shown in Figure 1 . There was no underlying mass or discharge. There were two other port site scars in the abdominal wall which were normal and painless. We treated empirically with oral antifungal and NSAIDs for 2 weeks and there was no response. We evaluated for systemic tuberculosis by IgM and IgG assay and found to be negative. Because there was no response to conservative treatment, we assumed any port site hernia, foreign body, deep seated infection, adhesion, etc. as the cause and we explained to the parents and got consent for umbilical exploration and proceeded with the intention to deal any secondary cause otherwise to excise the umbilicus. Under general anesthesia, vertical incision was made over the previous scar. The rectus sheath closed with Ethilon suture in the previous surgery was undone and the umbilical cicatrix was lifted from its bed. We found no secondary cause for the pain. The peritoneal cavity and bowels were normal. The umbilicus was excised with the erythematous skin margin. The rectus sheath was closed with 1/0 vicryl. Neo-umbilicus was created by fixing the edge of the skin to the rectus sheath. The postoperative period was uneventful. There was no pain or hyperesthesia from 2nd postoperative period till 5 months follow-up. The neo-umbilicus is cosmetically good with no redness or dampness. The umbilical skin biopsy showed thinned out epidermis and clusters of polymorph and lymphocytic infiltrates around the small vessels as shown in Figure 2 . There was no granuloma and the picture was consistent with leucocytoclastic vasculitis from our pathologist point of view. Umbilicus was tender with scaly and damp skin and a vertical scar around Histopathology of umbilical skin with H-E staining in low power [H and E, ×75], (a) showing thin epidermis with inflammatory infiltrates and in high power [H and E, ×300], (b) showing polymorph and lymphocyte infiltrates around the small vessels mimicking leucocytoclastic vasculitis

CRPS, although very rare, should be considered in the differential diagnosis when there is persisting unexplainable port site pain after laparoscopic surgery. This highlights a hitherto unforeseen complication of laparoscopic surgery.

Neurologist S. Weir Mitchell first described “causalgia” (Greek word “kausis” burning and “algos” pain) following war time as nerve injury characterized by persistent distal limb burning pain, swelling, abnormal skin color, temperature, and sweating.[ 1 ] Similar symptoms were later identified in posttraumatic patients without overt nerve injuries. Evans coined the term “reflex sympathetic dystrophy” (RSD) to label this entity. A consensus expert panel recommended the presently used term “complex regional pain syndrome”. CRPS is subdivided into CRPS type I and CRPS type II. CRPS-I is diagnosed when there is no obvious nerve injury, whereas CRPS-II refers to cases with nerve injury. The CRPS-I is equivalent of RSD and CRPS-II is also known as causalgia.[ 2 ] According to the international association for the study of pain, the diagnostic criteria for CRPS are as follows:[ 3 ] The presence of an initiating noxious event or cause of immobilization. Continuing pain, allodynia, or hyperalgesia disproportionate to the inciting event. Evidence of edema, changes in skin blood flow, or abnormal sudomotor activity in the area of pain. Exclusion of the existence of any condition that would otherwise account for the degree of pain and dysfunction. The presence of an initiating noxious event or cause of immobilization. Continuing pain, allodynia, or hyperalgesia disproportionate to the inciting event. Evidence of edema, changes in skin blood flow, or abnormal sudomotor activity in the area of pain. Exclusion of the existence of any condition that would otherwise account for the degree of pain and dysfunction. In our case there was persisting pain for one and half years after surgery which could not explained by any means, and there was hyperalgesia, skin color change, and dampness. The pain was not responding to any form of medical therapy. The dramatic pain relief after umbilicectomy and review of literature after biopsy finding made us to label this case as CRPS type I. The pathophysiology of CRPS is still controversially discussed for “inflammatory” and for a “sympathetic” pathogenesis. Basically, there is facilitated neurogenic inflammation, autonomic dysfunction, and neuroplastic changes within the nervous system.[ 1 ] The predisposing factors for CRPS are trauma, infection, fractures of bones, amputation, and joint surgeries.[ 4 ] CRPS occurs hypothetically due to partial peripheral nerve injuries with distal degeneration of small-diameter peripheral axons and inappropriate firing and neurosecretion by residual axons with denervation supersensitivity.[ 5 ] Small-fiber axon damage also causes increased release of nor-epinephrine, which causes vascular symptoms locally and rise in temperature of the affected part. Indeed, small-fiber-predominant polyneuropathies cause CRPS-like abnormalities and small distal nerve injuries in rodents reproduce many CRPS features further supports this hypothesis.[ 6 ] In CRPS-affected skin, immnuoflorescence and biopsy studies showed decrease in epidermal, sweat gland and vascular innervations, loss of vascular endothelial integrity, and extraordinary vascular hypertrophy.[ 2 ] The umbilical skin biopsy in our cases showed polymorph and lymphocytic infiltration of small vessels which was labeled as leucocytoclastic vasculitis (a localized form of vasculitis) by our pathologist. Leucocytoclastic vasculitis is a small vessel inflammatory disease due to deposition of immune complexes.[ 7 ] In our case there was no purpura or systemic symptom of vasculitis, and presence of hyperalgesia and increased sweating were not explainable by sepsis or vasculitis. Necrotizing arteritis and microvasculitis with inflammatory infiltrates in the peripheral nerve and skin biopsies of diabetic neuropathy patients support that inflammatory cell infiltration of vessels and nerves are character of ischemic axonopathy or patchy nerve fiber loss and need not be always an infective or vasculitis cause.[ 8 9 ] The symptoms, signs, biopsy findings, and pain relief after excision of umbilicus support neuralgic origin of pain in our case. Infection is the most common complication of any surgical procedure including laparoscopy. Persisting pain after port insertion through umbilicus may be due to infection, port site hernia, entrapment of bowel or omentum, endometriosis, metastasis, and tuberculosis.[ 10 ] This literature review before surgery made us to think that the above mentioned causes may be the etiology and thus umbilical exploration was contemplated assuming to deal any secondary cause. Because there was no secondary cause, we excised the umbilicus and neo-umbilicus was created. Because exaggerated soft tissue trauma and inflammatory response may underlie CRPS-I in some cases, laparoscopic surgery is not immune to it, as it involves injury at port site. There are also case reports of causalgia after simple venipuncture.[ 11 ] Hence, our hypothesis that excess trauma at the port site in laparoscopic surgery can lead to CRPS-I is supported by these studies. There is a surge in interest in single-incision laparoscopic surgery (SILS) in the recent years. There is a possibility that repeated exacerbated trauma in SILS may cause more pain in the immediate postoperative period or in long term like CRPS and is evidenced by a report on contusion of the infraumbilical region after single-incision cholecystectomy.[ 12 ] Physicians use a variety of drugs to treat CRPS and sometimes nerve blocks and surgical intervention may be needed in some cases with limited involvement. The dramatic relief of symptom in our case supports this.

The term “causalgia” was first described by S. Weir Mitchell to refer intense burning neuralgic pain of part or region of the body that is also experiencing high degree of sensitivity to the slightest vibration or touch. Presently, the term complex regional pain syndrome (CRPS) is used. At present, there is no consensus as to why causalgia occurs, but soft tissue trauma or entrapment of a nerve is considered to be the main underlying cause. Although laparoscopic surgery causes less tissue injury than its open counterpart, it is wrong to say that it is totally free from tissue damage and complications. Persisting pain in the umbilical port site after laparoscopic surgery is due to infection, hernia, endometriosis, metastasis, etc. There is no case report of umbilical pain of neuralgic origin after laparoscopy, and we have dealt a case which is described with relevant literature review.