172 Case report

Osteoid osteoma of the pisiform. Can it exist in a child? Ioannis V. Papachristos and Dimitrios Pasparakis Carpal osteoid osteomas are extremely rare, and only six cases in the pisiform have ever been described, but all concerned exclusively adults. We have treated a unique case of osteoid osteoma in the pisiform of a 13-year-old girl. We excised en bloc the nidus, which resulted in total excision of the pisiform. At follow-up after 15 years there was no recurrence and the patient remains asymptomatic with equal functionality of both hands. Henceforth, we must include osteoid osteoma in the differential diagnosis of pisiform lesions in children. En bloc resection prevents its recurrence and even the complete pisiform excision renders excellent results.

c 2014 Wolters Kluwer J Pediatr Orthop B 23:172–175 Health | Lippincott Williams & Wilkins.

Introduction

pisiform and triquetrum (Fig. 1). The patient also underwent electromyography due to the slight decrease in flexion force, but this only showed a reduction in maximum voluntary contracture in the flexor carpi ulnaris (FCU) and abductor digiti minimi (ADM) muscles, without denervation. A computed tomography (CT) scan was immediately performed, which revealed the osteoid osteoma by showing a typical lesion with a central granular opacity surrounded by a well-defined sclerotic margin under the cortex of the pisiform (Fig. 2). The lesion measured B2 cm in diameter, occupying nearly the whole pisiform. Neither technetium-99m bone scan nor magnetic resonance tomography was carried out since the diagnosis was highly certain. We decided to treat this lesion by open surgical approach. Radiofrequency ablation was not available at that time but even if it had been, its use would not have been justified due to the proximity of the lesion to the ulnar nerve and artery. Under endotracheal anaesthesia, with the use of Esmarch’s tourniquet, we approached volarly the ulnar side of the pisiform, which was found to be irregular, roughened, and enlarged. Minimal articular cartilage could be found on either surface of the pisotriquetral joint with marked synovitis. It was crucial to excise the whole pisiform en bloc, since its enucleation and curettage would leave remnants of bone unable to be reconstructed with the use of bone autograft. The insertions in the FCU and ADM were stably sutured at the roof of Guyon’s canal and the incisions were closed. A volar splint was put in place for 15 days and then full range of motion exercises were encouraged. The patient had an uneventful postoperative course and was dramatically relieved from pain. Cultures of the lesion were negative and histologic examination under hematoxylin–eosin stain showed the presence of immature trabeculae surrounded by proliferative osteoblasts in a richly vascular stroma, which is typical of an osteoid osteoma nidus (Fig. 3). Review of the

Jaffe et al. [1] first described in 1935 the pathological entity termed ‘osteoid osteoma’. Since then, there have been numerous studies on this benign osteoblastic tumor in both adults and children. Carroll [2] was the first to publish cases of osteoid osteomas affecting the hand and specifically metacarpals and proximal phalanges. It is estimated that this lesion in the hand and wrist accounts for 6–13% of all cases of osteoid osteomas, but the real percentage in children has never been estimated since reported studies are few and scarce [3]. Carpal bones are remarkably rare sites of osteoid osteoma, 2% of all cases, with scaphoid and capitate being the most prevalent [4–6]. Only six cases of osteoid osteoma in the pisiform have ever been described in the medical literature to date, with all patients being adults. We treated a unique case of a child suffering from this entity in the pisiform, which is the first case that has been ever reported. In this paper, we highlight the characteristics of this rare entity and emphasize this new knowledge in pediatric osteoid osteomas.

Case report In September 1997, a 13-year old-girl presented to our department complaining of pain in the ulnar side of her wrist. The pain was intermittent for the last 2 years, gradually deteriorated, and was initially attributed to injury. In the last 6 months a swelling without redness could easily be seen on careful inspection but the patient and her family did not seek orthopaedic consultation as they thought that it would pass. Pain was greatest at night and the patient experienced relief from taking aspirin. Thorough examination revealed slight reduction in flexion force in the affected hand. Full blood count, Creactive protein, and erythrocyte sedimentation rate were unremarkable. The typical radiological workup revealed a radiopacity surrounded by a vague radiolucency in the c 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins 1060-152X

Journal of Pediatric Orthopaedics B 2014, 23:172–175 Keywords: child, hand, osteoid osteoma, pediatric, pisiform Second Orthopaedic Department of Children’s Hospital of Athens ‘Aglaia Kyriakou’, Athens, Greece Correspondence to Ioannis V. Papachristos, MD, MSc, Second Orthopaedic Department of Children’s Hospital of Athens ‘Aglaia Kyriakou’, 1B Danais Street, Ekali, PO Box 14578, Athens, Greece Tel/fax: + 30 697 585 2104; e-mail: [email protected]

DOI: 10.1097/BPB.0000000000000012

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Osteoid osteoma in child pisiform Papachristos and Pasparakis 173

Fig. 1

Fig. 2

Radiograph of the left hand of a 13-year-old girl showing enlarged radiolucent lesion in the pisiform.

patient’s file was carried out after obtaining institutional board approval. One year postoperatively the patient was asymptomatic and at follow-up 15 years from the operation the patient had equal flexion force and functionality in both hands with no recurrence and a clear radiograph (Fig. 4).

Discussion Osteoid osteoma is very rarely observed in the hand (its incidence ranges from 2.8 to 10.23% of all cases of osteoid osteomas) and only one-third of hand cases affect the carpal bones [7,8]. Scaphoid and capitate are the predominant sites where this lesion (henceforth osteoid osteoma will be referred to here as OO) is observed [9]. The first to report OO in the pisiform was Kernohan et al.

Computed tomography scan, the nidus is depicted. (a) Sagittal plane where the nidus is depicted. (b) Transverse plane.

in 1985 [10]. In their paper, two cases were included and complete surgical removal of the nidus provided cure [10]. Five years later in 1990 Miller reported the case of an 18-year-old man who suffered from OO in the pisiform and the complete excision of the bone yielded excellent results [11]. Murray et al. [6], from 26 800 primary bone tumor cases collected in 2 years, found 11 cases with OO in the carpal bones and only one in the pisiform (in a 22-year-old man). Wiesner et al. in 2007 [12] reported the ‘light bulb sign’ in an MR angiography of a pisiform OO in an adult male. This image was produced by the vascularity of the nidus in contrast to the hypointen-

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174 Journal of Pediatric Orthopaedics B 2014, Vol 23 No 2

Fig. 3

Hematoxylin-eostin stain ( 200 magnification) showing proliferative osteoblasts in rich vascular stroma.

Fig. 4

Radiograph 15 years postoperatively.

sity of surrounding tissues in T1 images and to the hyperintensity of these tissues in T2 and T1 postcontrast images. This sign helps to differentiate OO from vascular lesions during MR angiography. The most recent report of OO in a pisiform came from Thomsen and Dumontier in

2011 [13]. They had treated a 21-year-old man suffering from this rare entity, with true enucleation of the nidus and curettage of the remaining bone. No autogenous bone graft was used and the result was excellent. Thus, to date only six cases of OO in the pisiform have been reported, with all of them in the adult population. Our report is unique since it is the first of an OO in the pisiform in a child, and its value becomes greater if we consider that in 1997 when the treatment took place only three cases of adult OO in the pisiform had been reported, and thus it was unlikely for us to find and be consulted about a similar case. This benign tumor shows a selectivity in age between the second and third decade and a male preponderance (2 : 1) [14–16]. Pain is the usual symptom, which is usually greater at night due to prostaglandin secretion, but painless OO has also been reported [9,17]. Carpal OO often produces misleading symptoms and a history of injury to the wrist during sports at school is common and may lead to deviations in the diagnosis and subsequent loss of time [18]. The reported delay until diagnosis can reach 18 months and in our case was 2 years [19,20]. A patient suffering from OO in carpal bones can present with local swelling, inflammatory joint disease, and tenosynovitis, like the patient in our case [18,19,21]. Typically, the lesion is radiographically depicted as a radiolucency with a peripheral ring of dense bone and a well-demarcated central nidus of calcification [14]. In the case of carpal bones this appearance may differ, presenting as a granular opacity with a perimeter of radiolucency, referred to by other authors as ‘ring sequestrum’, which applies to our case [2,14,22–24]. Moreover, the small size of these bones causes superimposition of the nidus and bone sclerosis, and especially in children they can be mistaken for ossification centers. All these findings can be absent for several months after the onset of symptoms. Technetium99m bone scan is positive even from the initial stages of the disease due to the high vascularity of the nidus, showing a non-specific typical ‘hot spot’. In our case we did not choose to perform a bone scan and proceeded immediately with thin-slice CT scan. This imaging modality is the most specific in the diagnosis of OO, depicting perfectly the nidus surrounded by sclerosis as was also confirmed in our 13-year-old patient [20,25]. In recent years, CT has been used in combination with MRI [26]. The typical pattern of OO on MRI is a circumscribed area of low or isointense signal surrounded by edema and a low-signal rim of sclerosis [20]. On T2weighted fat-saturated images intraosseous edema and soft tissue changes generate high-intensity signal [5]. Intravenous gadolinium injection enhances the nidus, which is vascular, whereas the sclerotic rim and the central calcified part of the nidus generate low-intensity signal on all the sequences, giving the ‘light bulb sign’ as previously mentioned in this paper [12]. MRI helps to rule out abscesses and avascular necrosis in carpal bones, but it lacks specificity compared with CT when the surrounding swelling is marked.

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Osteoid osteoma in child pisiform Papachristos and Pasparakis 175

Since the first report of hand OO in 1953, in which Carroll stated that the treatment applied to some hand OO was amputation, evolution was marked. CT-guided percutaneous thermocoagulation, introduced in 1992, has gained in popularity as a less invasive treatment of OO, with excellent results, as with open procedures [27]. Unfortunately, the thermal necrosis caused round the electrode in a spherical area of 1 cm in diameter prevents its use in the hand as there are many vital structures (nerves, arteries) in close proximity. The treatment of choice for carpal OO remains the excision of the nidus with subsequent curettage of the remaining sclerosis and the use of autologous cancellous bone in cases of residual large bone defects. The tumor is usually a round lump surrounded by marked reactive synovitis. The appearance is that of a red and white bull’s eye with moderate bleeding [18]. In our case, due to the relatively large size of the lesion (almost the whole pisiform) we had to resect the pisiform and reattach FCU and ADM muscles in the local retinaculum. Postoperative immobilization is usually unnecessary, but in our case the reattachment of the muscles necessitated the use of a volar splint extending until the little finger for 15 days. It was strange that the lesion occurred immediately after the ossification procedure of the pisiform was finished, and therefore it is suspected that perhaps these two factors, ossification and OO, are connected (perhaps the OO was caused by a defect in the ossification process). The age of the patient and the location of the lesion were so exceptional that although we were certain of the diagnosis, we waited with great impatience for the pathology report. Long-term follow-up (15 years) showed that we had operated using the best method, and despite being performed on a child, complete excision of the pisiform provided excellent results.

References 1 2 3 4 5 6 7

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Conclusion

The purpose of this report was to analyze a unique case of a pediatric carpal OO and to highlight its exceptional characteristics. Henceforth, orthopaedic surgeons should include OO in the differential diagnosis of pisiform lesions.

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Acknowledgements Conflicts of interest

There are no conflicts of interest.

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Osteoid osteoma of the pisiform. Can it exist in a child?

Carpal osteoid osteomas are extremely rare, and only six cases in the pisiform have ever been described, but all concerned exclusively adults. We have...
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