Neoadjuvant chemotherapy before surgery of hepatoblastoma.

Though surgical resection is the main stay of treatment for childhood hepatoblastoma (HB), many are unsuitable for radical surgery at diagnosis due to extensive intrahepatic and/or extra hepatic disease. We report experience in five patients of HB from a single institution (2001-2005) with preoperative Neoadjuvant chemotherapy (NACT) followed by surgery. Three patients received cisplatin, doxorubicin; and two cisplatin / vincristine /5-fluorouracil. All showed more than 50% reduction in tumor size confirmed by CT scan. Hepatic resection R0 was performed in all. There was no chemotherapy related toxicity nor post surgical morbidity or mortality. All are disease free at median follow up of 4 years. NACT produces adequate down staging of the HB with acceptable toxicity. Though cisplatin with doxorubicin produced good results, new protocol with cisplatin, vincristine and 5FU is promising without cardiotoxicity.

C3-4 level cervical spondylotic myelopathy.

Cervical spondylotic myelopathy (CSM) is uncommon at the C3-4 level. Fourteen patients with C3-4 CSM were treated over a period of 3 years. The radiological factors contributing to CSM at the C3-4 level were studied. These factors included the assessment of static and dynamic canal diameters, retrolisthesis, posterior osteophytes and degenerative spinal segmental fusion on plain X-rays; and, the antero-posterior cord compression ratio (APCR) on magnetic resonance imaging (MRI). The clinical status of the patients was assessed by the modified Japanese orthopedic association scale (mJOAS). The mean difference between the static and dynamic canal diameters was statistically significant at C3-4 (p < 0.01). The APCR obtained at different levels showed a significant compression at the C3-4 level in comparison to the lower level. There was a correlation between the APCR and the preop mJOAS, r=0.6 (p< 0.05). The mean mJOAS improved from 9.35 to 14.35 at follo-up. The recovery rate calculated using the modified Hirabayashi rate was 66.9%. Degenerative changes at lower cervical segments predispose to increased mobility and spondylotic changes at the C3-4 level. The patients in this study were young as compared to those reported in the international literature.

Choroid plexus papillomas of the cerebellopontine angle.

The cerebellopontine angle (CPA) is a rare site for the growth of choroid plexus papilloma (CPP). The clinicoradiological diagnosis of this tumor in the CPA is difficult because of the nonspecific clinical presentation and radiological features. Five cases of choriod plexus papilloma (3 males, 2 females) operated upon at this center are reviewed. All the cases were operated upon by retromastoid suboccipital craniectomy. As they all presented with a typical CPA syndrome without any distinctive radiological feature, a clinicoradiological diagnosis of CPP could not be reached in any of these. The diagnosis of CPP could only be suspected at the operation table and established on histopathological examination. Two patients developed recurrences at the primary site following surgery. One patient developed recurrence twice despite gross total excision of tumor in each sitting. Subsequently, this patient remained symptom free for a follow-up period of 1 year. Another patient developed recurrence 2 years following surgery, but he died due to septicemia and aspiration pneumonitis. Therefore definitive surgery could not be performed. Radiotherapy was offered to one of the patients having residual mass post operatively, to render her symptom free for a 4 year follow-up. The remaining two patients have also showed progressive improvement in their symptoms following surgery for 4 years on follow-up. Hydrocephalus was a common feature in all the cases preoperatively, but only one required shunt CSF diversion, because of rapid deterioration in visual equity. In all other cases, hydrocephalus was managed conservatively. Surgery remains the main modality of treatment for CPP, both for primary and recurrent tumors, but radiotherapy may have a role in cases of recurrence, which are quite frequent.

Hepatic resection for benign liver pathology--report of two cases.

Hepatic resection for benign pathology is rare. We report our experience in two patients with hepaticolithiasis. One patient underwent left hemihepatectomy with removal of segments II, III and IV for localized Caroli's disease with multiple intrahepatic calculi. The second patient underwent resection of segment II and III with Roux-en-Y hepaticojejunostomy for right and left intrahepatic calculi with an abscess in the left lobe of the liver and a choledochal cyst.

Anterior retropharyngeal approach to the cervical spine.

The anterior retropharyngeal approach (ARPA) accesses anteriorly situated lesions from the clivus to C3, in patients with a short neck, Klippel Feil anomaly or those in whom the C2-3 and C3-4 disc spaces are situated higher in relation to the hyoid bone and the angle of mandible where it is difficult to approach this region using the conventional anterior approach, due to the superomedial obliquity of the trajectory. The ARPA avoids the potentially contaminated oropharyngeal cavity providing for a simultaneous arthrodesis and instrumentation during the primary surgical procedure. Experience of five patients with high cervical extradural compression, who underwent surgery using this approach between 1994 and 1999, is presented. The surgical procedures included excision of ossified posterior longitudinal ligament (n=2); excision of prolapsed disc and osteophytes (n=2); and excision of a vertebral body neoplasm (n=1). Following the procedure, vertebral arthrodesis was achieved using an iliac graft in all the patients. Only one patient with vertebral body neoplasm required an additional anterior cervical plating procedure for stabilisation the construct. The complications included transient respiratory insufficiency and neurological deterioration in two patients; and, pharyngeal fistula and donor site infection in one patient.

Medulloblastomas of the cerebellopontine angle.

It is unusual for a medulloblastoma to occur in the cerebellopontine angle (CPA). Four of the total 63 cases of medulloblastoma managed in our department between 1988 to 1998, presented with a mass in the cerebellopontine angle. There were two children amongst these four patients, aged eight and nine years. The other two patients were young adults. Three patients had primary CPA medulloblastoma, while the fourth case had a recurrence in this region, following the excision of the primary vermian tumour. All the four cases were managed by gross total microsurgical excision and craniospinal irradiation. One of the two children, developed subfrontal recurrence after 1 year and 8 months of initial treatment, while the other child developed a large recurrence at primary site, within three months of post-operative irradiation. However, the adult patient developed CPA recurrence and subsequent cervical intramedullary metastasis within two and a half years of diagnosis. These three cases succumbed to their disease, between three months to thirty months of their initial management. However the fourth patient, a 20 years female with desmoplastic variant, was well at two and a half years of follow up.

Extended frontobasal approach to the skull base.

The extended frontobasal approach provides an adequate midline exposure from the anterior cranial fossa to the sphenoclival region. Between November 1991 and August 1999, 13 patients with extensive anterior and anterolateral skull base tumours extending to supra and parasellar regions, cavernous sinus and sphenoclival regions were operated upon using this approach alone (7 patients) or in combination with subtemporal -infratemporal (4 patients) or transfacial (2 patients) approaches. Gross total excision was performed in 8 patients while in 4 patients with malignant tumours and in a patient with extensive skull base fungal granuloma, only partial excision was possible. Basal repair was performed using pedicled pericranium, temporalis muscle or fascia lata. The complications included increase in the cranial nerve paresis, endophthalmitis, facial oedema, CSF leak, frontal haematoma and internal carotid artery injury. This study reviews the operative technique, the indications and the complications of extended frontobasal approach.

Split cord malformation (scm) in paediatric patients: outcome of 19 cases.

There had been considerable debate regarding the surgical outcome of neuro-orthopaedic syndromes (NOS) and neurological syndromes in cases of split cord malformation (SCM). On retrospective analysis of 19 cases of SCM, thirteen were grouped under (Pang) type I and 6 in type II. Their age ranged from 1 month to 9 years (mean 3.5 years). 14 of these were male children. The NOS without neurological signs was detected in 6 cases where as pure neurological signs without NOS were seen in 8 patients. However, the rest 5 had mixed picture of NOS and neurological dysfunction. Nine of 19 cases presented with cutaneous stigmata, mainly in the form of hairy patch. 18 cases had other associated craniospinal anomalies i.e. hydrocephalus, meningomyelocoele, syrinx, dermoid, teratoma etc. Detethering of cord was done in all cases by removal of fibrous/bony septum. Associated anomalies were also treated accordingly. Follow up of these cases ranged from 6 months to 6 years. Six cases of NOS group neither showed deterioration nor improvement, and remained static on follow up. However, four of 8 children with neurological signs showed improvement in their motor weakness, and 1 in saddle hypoaesthesia as well as bladder/bowel function. In 5 cases of mixed group, two had improvement in their weakness and one in hypoaesthesia, but no change was noticed in NOS of this group as well. Hence surgery seemed to be effective, particularly in patients with neurological dysfunction.

Infected Ewing's sarcoma of spine.

An unusual case of extradural, intraspinal Ewing's sarcoma with significant extraspinal extension is reported. In view of associated constitutional symptoms, suggestive signs, leucocytosis, elevated ESR and operative findings, the initial diagnosis of Pott's spine was entertained. The lesion however turned out to be infected Ewing's sarcoma on histopathology and bacteriological examination.

C1-C2 rotary subluxation following posterior stabilization for congenital atlantoaxial dislocation.

The authors report a rare complication of C1-C2 rotary subluxation in two children following posterior stabilization for congenital atlantoaxial dislocation (AAD). A patient, with mobile AAD, underwent Brook's C1-C2 fusion while the other, with fixed AAD, underwent transoral decompression followed by Jain's occipitocervical fusion. A pre-existing ligamentous laxity associated with an asymmetrical wire tightening or slippage of the wires due to rotation of the neck in the former, and the drilling of the C1-C2 lateral joints during the transoral procedure in the latter, could have contributed to the rotary subluxation. Both patients presented with persistent torticollis due to fusion in an asymmetrical position with dislocated facet joints. Rotary C1-C2 subluxation, when coexisting with anterior dislocation, has the potential to cause severe and occasionally fatal cord compression. Well defined criteria to diagnose this entity by conventional radiology exist, however, due to the overlap of anatomy, the condition is often overlooked. In the present study, three dimensional reconstruction images using helical computerized tomography were very useful in delineating the subluxation and in planning its surgical reduction and arthrodesis.

Transoral decompression for craniovertebral osseous anomalies: perioperative management dilemmas.

The surgical outcome of 74 patients, who underwent transoral decompression (TOD) for ventral irreducible craniovertebral junction anomalies between January 1989 to September 1997, was studied to evaluate the perioperative complications and problems encountered. The indications for TOD included irreducible atlantoaxial dislocation (n=24), basilar invagination (n=16), and a combination of both (n=35). Following TOD, occipitocervical stabilization using Jain's technique was carried out in 50 (67.5%) and atlantoaxial fusion using Brooks' construct in 18 (24.3%) patients. The pre- and postoperative radiology was compared to assess the adequacy of decompression and stability. The major morbidity included pharyngeal wound sepsis leading to dehiscence (20.3%) and haemorrhage (4%), valopharyngeal insufficiency (8.1%), CSF leak (6.7%) and inadequate decompression (6.7%). Neurological deterioration occurred transiently in 17 (22.9%) and was sustained in 7 (9.4%) patients. The mortality in six cases was due to operative trauma, exanguination from pharyngeal wound (one each), postoperative instability and inability to be weaned off from the ventilator (two each). Of the 47 (63.5%) patients available at follow up ranging from 3 months to 2 years, 26 (55.3%) showed improvement from their preoperative status while 14 (29.8%) demonstrated stabilization of their neurological deficits. Seven (14.9%) of them deteriorated. Though TOD is logical and effective in relieving ventral compression due to craniovertebral junction anomalies, it carries the formidable risks of instability, incomplete decompression, neurological deterioration, CSF leak, infection and palatopharyngeal dysfunction.

Association of lower cranial nerve schwannoma with spinal ependymoma in ? NF2.

A 15 year old male, who had earlier been operated for intraspinal intramedullary ependymoma, subsequently developed a right cerebello pontine (CP) angle mass. A diagnosis of right CP angle ependymoma was considered, in view of established histology of previously operated spinal lesion. Histopathological examination of the well defined extra-axial mass, which was attached with ninth cranial nerve, however revealed a schwannoma. A diagnosis of Neurofibromatosis-2 (NF2) is strongly suspected, because of well established fact, that the spinal ependymomas may have association with lower cranial nerve schwannomas in NF2. Cranial and spinal MRI screening for early diagnosis of associated, asymptomatic lesions, in suspected cases of NF2, particularly in children, is recommended.

Extreme lateral transcondylar approach to the skull base.

In this study, the authors present their experience of using extreme later transcondylar approach (ELTC) for treating 7 patients with lesions in the anterolateral foramen magnum, upper cervical spine and cerebellopontine angle reaching upto jugular foramen. The tumours included meningiomas, neurofibromas (2 cases each), chondrosarcoma, epidermoid and aneurysmal bone cyst (one case each). The approach was used alone, in combination with retrolabyrinthine presigmoid approach in a patient with lower cranial nerve neurofibroma extending extracranially through the jugular foramen, or in combination with partial C1-C3 laminectomy in two patients with meningiomas situated anterolateral to the cord from the foramen magnum to C3. In two patients with extradural vertebral artery (VA) entrapment by a chondrosarcoma and aneurysmal bone cyst respectively, the vertebral artery was ligated distal to the tumour. The tumours were totally excised in five cases and partially in two. There was no preoperative mortality. The major complications included cerebrospinal fluid leak from the wound (3 cases) and increase in lower cranial nerve paresis (2 cases). At follow up, ranging from 6 months to 2 years, 5 patients showed no tumour recurrence. There was improvement in neurological status. One patient, with a partially excised aneurysmal bone cyst, showed no added deficits or increase in the tumour size. However, there was a massive regrowth in the patient with chondrosarcoma after 6 months. This technique provided a wide surgical exposure with direct visualization of the tumour-anterior cord interface, early proximal control of the VA and preservation of lower cranial nerves.

Growth hormone-producing pituitary tumours: clinical profile and results of surgery.

BACKGROUND: Growth hormone-producing pituitary tumours present with a wide variety of manifestations. The optimum diagnostic work up, management and follow up of such patients is complex and involves a multidisciplinary approach. There is paucity of data from India with regard to the clinical presentation and results of surgery for growth hormone-producing tumours. METHODS: We studied the first 50 patients presenting during 1989-94 with growth hormone-producing pituitary tumours to our centre. The work up included detailed endocrine and radiological assessment. The surgical outcome was analysed for 35 patients who were operated (trans-sphenoidal 29, transcranial 6) at our centre. RESULTS: All the patients had macroadenomas [mean (SD) diameter 3.12 (0.87) cm]. Seventy-five per cent of the patients had supra- and/or parasellar extension and 57% had visual field defects. Tumour size correlated with the preoperative basal (r = 0.57) and glucose-suppressed (r = 0.54) growth hormone levels. Thirty-three of the 35 patients operated at our centre (trans-sphenoidal 28, transcranial 5) were available for follow up (median duration 34 months). After trans-sphenoidal surgery alone, 12 of the 28 (43%) patients had normalization of growth hormone levels (post-glucose growth hormone < 5 ng/ml), and 9 of 11 (82%) showed improvement in visual fields. CONCLUSION: In India, growth hormone-producing pituitary tumours are usually large in size. The growth hormone levels correlate with the size of the tumour. These tumours can be effectively treated by trans-sphenoidal or transcranial surgery.

In vivo localized proton magnetic resonance spectroscopy of intracranial tuberculomas.

In vivo proton magnetic resonance spectroscopy (MRS) was performed in ten patients with intracranial tuberculomas with the aim of finding biochemical finger prints which may help in the noninvasive diagnosis of the disease. Diagnosis of tuberculoma was confirmed in all these cases retrospectively. Ex vivo spectroscopy of formalin fixed samples of four granulomas and a normal brain (cerebellar) tissue, was performed to confirm the in vivo resonances. The in vivo study showed resonance at 1.3 ppm and 0.9 ppm assigned to methylene group (CH2)n and terminal methyl groups (-CH3) of fatty acids respectively which was subsequently confirmed by the ex vivo study. Large resonances of fatty acids in in vivo study appeared to be due to high lipid content of caseous material. It is concluded that in vivo proton spectroscopy may be helpful in differentiating tuberculomas from other intracranial mass lesions which have diagnostic difficulties on MR imaging.

Blood requirement and utilization in spinal surgery (blood in spinal surgery).

The blood requisition practice for the most commonly performed spinal prolapsed disc surgery was assessed in a prospective manner for 18 months, in total 108 patients. It was observed that nearly 78% operations did not require any transfusion. Transfusion index (TI) was .4, showing a tendency of over ordering of blood for these procedures. The cross match: transfusion (C/T) ratio was very high 4.3. It is obvious that these procedures hardly require blood transfusion. Thus by introducing an ABO-Rh typing screening and immediate spin cross match scheme, can drastically reduce the patients agony, laboratory work load and patients treatment cost.