Robot-Assisted Sistrunk’s Operation, Total Thyroidectomy, and Neck Dissection via a Transaxillary and Retroauricular (TARA) Approach in Papillary Carcinoma Arising in Thyroglossal Duct Cyst and Thyroid Gland

Background

Carcinomas arising in the thyroglossal duct cysts are rare, accounting only for about 0.7?C1.5?% of all thyroglossal duct cysts.1 ^?C 3 Synchronous occurrence of thyroglossal duct carcinoma and thyroid carcinoma is reported to be even rarer.4 Traditionally, surgical treatments of such coexisting thyroglossal duct cyst carcinoma (TGDCa) and papillary thyroid carcinoma (PTC) were typically performed through a single transverse or double incisions on the overlying skin. A longer, extended cervical incision might be required if neck dissection is necessary. Though this method provides the operator with the optimal surgical view, the detrimental cosmetic effect on the patient of possessing a scar cannot be avoided, despite the effort of the surgeon to camouflage the scar by placing the incision in natural skin creases. Recently, the authors have previously reported the feasibility of robot-assisted neck dissections via a transaxillary and retroauricular (??TARA??) approach or modified face-lift approach in early head and neck cancers.5 ^, 6 On the basis of the forementioned surgical technique, we demonstrate our novel technique for robot-assisted Sistrunk??s operation via retroauricular approach as well as robot-assisted neck dissection with total thyroidectomy via transaxillary approach.

Methods

This is a case presentation of a 22-year-old woman with synchronous TGDCa and PTC with minimal lymph node metastasis who underwent resection of TGDCa and total thyroidectomy with left neck level III and IV lymph node dissection as well as central compartment lymph node dissection (CCND) via TARA approach with a robotic surgery system after approval from the institutional review board at Severance Hospital, Yonsei University College of Medicine. The incision was just like the TARA approach in head and neck cancer, which has been reported by our institute.6 The operation was proceeded as follows. First, excision of the TGDCa through the retroauricular incision was done followed by total thyroidectomy with CCND via transaxillary approach. Finally, neck dissection of left level III, IV was conducted via transaxillary approach. The da Vinci surgical system (Intuitive Surgical, Sunnyvale, CA) was introduced via retroauricular or transaxillary port. A 30° dual-channel endoscope was used, and the two instrument arms were equipped with 5?mm Maryland forceps and a 5?mm spatula monopolar cautery for TGDCa excision via retroauricular approach. When conducting total thyroidectomy and neck dissection via transaxillary approach, three instrument arms were utilized, each equipped with 5?mm Maryland forceps, ProGrasp forceps and a 5?mm spatula monopolar cautery or Harmonic curved shears. The rest of the surgery was completed with the robotic system (see Video).

Results

The operative procedure was successfully completed utilizing the robotic surgical system with no conversion to open surgery. The operation time for TGDCa excision was 97?min, including the time for skin flap elevation (15?min), setting up the robotic system (5?min), and console time using the robotic system (77?min). Also, the total operation time for the consecutive total thyroidectomy with CCND and level III, IV dissection was 142?min including the time for skin flap elevation (27?min), setting up the robotic system (3?min), and console time using the robotic system (112?min). There were no intraoperative complications. The retroauricular approach for the removal of the TGDCa allowed for an excellent magnified surgical view revealing important structures of the local anatomy. It also created sufficient space for the cutting of the relevant portion of the hyoid bone. Handling of the robotic instruments through the incision was technically feasible and safe without any mutual collisions throughout the operation. The patient??s postoperative parathyroid hormone (PTH) level was within normal range and functions of her both vocal cords were intact. The histopathologic results of the specimens revealed thyroglossal duct cyst with internal papillary carcinoma measuring 1.1?cm with infiltrative tumor margins and papillary microcarcinoma measuring 0.9?cm within the left thyroid lobe with extrathyroidal soft tissue extension. There was no evidence of tumor in the right lobe and the pyramidal lobe of the thyroid gland. As for the lymph nodes resected, 7 out of 9 paratracheal nodes and 2 out of 7 left level III, IV nodes revealed metastatic carcinomas. The patient was discharged on the 8th?day after the operation with no complications. The patient was extremely satisfied with the cosmetic results. The patient has received high-dose radioiodine ablation (RAI) therapy and is currently doing well with no evidence of recurrence.

Discussion

Although there is still a great deal of controversy regarding the treatment of TGDCa, there is little debate that for the cases of synchronous TGDCa and PTC, total thyroidectomy in addition to the Sistrunk procedure must be performed.5 ^?C 6,12 As for the patient in our case where left level IV lymph node metastasis was detected under preoperative ultrasonography (USG), if the usual method of surgical procedure was to be selected, double incisions or a single extended transverse incision must be adopted for the Sistrunk??s operation and total thyroidectomy with lateral neck dissection. The conventional method to remove neck masses was to do so by placing an incision on the overlying skin. This ??open?? approach to viewing the lesion has an advantage of providing the operator with the best surgical view, but the recognizable surgical scar that results from the surgery can be displeasing for patients. Therefore the surgeon can try to make a small incision and camouflage the scar by placing the incision in natural skin creases, yet the cosmetic results can still be displeasing for the patient due to its visibility and permanence. This can be an even greater problem if the patient is young and an active member of his/her society and if the lesion is benign or low-grade malignancy which can be simply dissected and excised. Therefore it is the surgeon??s best interest to perform an operation successfully with a ??least obvious?? or ??hidden?? scar whenever possible. Accordingly, we have adopted a novel approach, the transaxillary and retroauricular approach, in view of our increasing surgical experience with various indications such as submandibular gland (SMG) resections and neck dissections in head and neck cancer or thyroid papillary carcinoma.5 ^?C 7 Some investigators have demonstrated that robot-assisted neck dissections performed on patients with thyroid cancer and lateral neck node metastasis are feasible and safe.13 We conducted total thyroidectomy with bilateral CCND and level III and IV dissection using the same approach. Although the technical feasibility and safety of neck dissection or SMG resection via retroauricular approach has already been reported previously at our institute, Sistrunk??s operation via retroauricular approach will be challenging. In spite of that, we were able to demonstrate successfully Sistrunk??s operation including the hyoid bone resection through the retroauricular approach. There are however, certain areas of potential difficulties which must be considered with caution during the operation procedure. First, when removing the TGDCa through the retroauricular port, identification of the ipsilateral hyoid bone is primarily important and it is also crucial that dissection along the capsule must be done carefully so as not to rupture the tumor. It is essential that sufficient working space must be created for the comfortable movement of the robotic arms through the retroauricular port and in order to do so, sufficient skin flap elevation in both superior and inferior directions must be performed. It is necessary to elevate the superior skin flap up to the level of the inferior border of the mandible but during this process, the platysma muscle must be identified and meticulous dissection along the subplatysmal plane must be carried out so as not to damage the marginal mandibular branch of the facial nerve. Another area of potential pitfalls concerns the total thyroidectomy with neck dissection through the transaxillary port. Sufficient amount of working space must be secured in order to perform comfortably the contralateral thyroidectomy and neck dissection and in order to do so, skin flap elevation must be done at least 2?cm further based on the ipsilateral omohyoid muscle and the contralateral thyroid gland must be adequately exposed. Using the robotic surgical system in removing the thyroglossal duct cyst, the free movement of wristed instrumentation through the retroauricular incision allowed for efficient dissection and easy handling of the tissue. In this particular case we could not identify the tract beyond the hyoid and up to the foramen cecum, but we anticipate that there would be no technical problems of dissection and excision had it been so. To our knowledge, Sistrunk??s operation and total thyroidectomy with lateral neck dissection via TARA approach utilizing the robotic surgical system has never been attempted before. It has some advantages over the conventional surgery in terms of cosmesis. However, careful consideration in selecting appropriate cases is required and prospective trials should be conducted to recognize long-term outcomes and to overcome potential limitations.     

Adrenocortical Carcinoma: A Clinician’s Perspective

Within the category of orphan diseases and rare malignancies, adrenocortical carcinoma (ACC) represents an aggressive entity with high mortality and morbidity. While localized tumors which are diagnosed early can be cured with surgical intervention, there are prognostic factors which predict for micrometastases and consequent recurrent and advanced disease. In such cases, mitotane and cytotoxic chemotherapy have been utilized with a modest degree of benefit. The poor prognosis of recurrent and advanced ACC has underscored the interest in nuanced characterization of ACC cases to guide the personalized use of immunotherapeutic and novel targeted therapies.     

Low-Grade Salivary Duct Carcinoma or Low-Grade Intraductal Carcinoma? Review of the Literature

Low-grade salivary duct carcinoma (LG-SDC) is a rare neoplasm characterized by predominant intraductal growth, luminal ductal phenotype, bland microscopic features, and favorable clinical behavior with an appearance reminiscent of florid to atypical ductal hyperplasia to low grade intraductal breast carcinoma. LG-SDC is composed of multiple cysts, cribriform architecture with “Roman Bridges”, “pseudocribriform” proliferations with floppy fenestrations or irregular slits, micropapillae with epithelial tufts, fibrovascular cores, and solid areas. Most of the tumor cells are small to medium sized with pale eosinophilic cytoplasm, and round to oval nuclei, which may contain finely dispersed or dark condensed chromatin. Foci of intermediate to high grade atypia, and invasive carcinoma or micro-invasion have been reported in up to 23 % of cases. The neoplastic cells have a ductal phenotype with coexpression of keratins and S100 protein and are surrounded by a layer of myoepithelial cells in non-invasive cases. The main differential diagnosis of LG-SDC includes cystadenoma, cystadenocarcinoma, sclerosing polycystic adenosis, salivary duct carcinoma in situ/high-grade intraductal carcinoma, and papillary-cystic variant of acinic cell carcinoma. There is no published data supporting the continuous classification of LG-SDC as a variant of cystadenocarcinoma. Given that most LG-SDC are non-invasive neoplasms; the terms “cribriform cystadenocarcinoma” and LG-SDC should be replaced by “low-grade intraductal carcinoma” (LG-IDC) of salivary gland or “low-grade intraductal carcinoma with areas of invasive carcinoma” in those cases with evidence of invasive carcinoma.     

Lemierre’s Syndrome in a Patient with Antiphospholipid Syndrome

Internal jugular vein thrombosis is usually associated with intravenous drug abuse, long-term venous catheterization, local infection, or spontaneous occlusion. The antiphospholipid syndrome is now recognized as one of the commonest causes of acquired arterial or venous thrombosis. Lemierre’s syndrome is an uncommon but potentially lethal complication of internal jugular vein thrombosis after an oropharyngeal infection. A 34-year-old man presented with fever and progressive, painful right neck swelling 6 days after an episode of pharyngitis. Clinical studies including radiological and laboratory tests confirmed the diagnosis of Lemierre’s syndrome associated with antiphospholipid syndrome. Although Lemierre’s syndrome is a well-known cause of internal jugular vein thrombosis, association with antiphospholipid syndrome is very rare.     

Hypokalemic rhabdomyolysis in a child with Bartter’s syndrome

Hypokalemia represents a rare cause of rhabdomyolysis. Some reports have described a few adult patients affected by Bartters syndrome and Gitelmans syndrome with rhabdomyolysis due to severe hypokalemia. We report the first pediatric patient with Bartters syndrome in whom rhabdomyolysis developed when her plasma potassium level was less than 2 mEq/l. Prompt intravenous fluid and potassium prevented tubular damage and acute renal failure. We recommend determining serum creatine phosphokinase in all patients affected by Bartters syndrome and profound hypokalemia.     

Hypokalemic rhabdomyolysis in a child with Gitelman’s syndrome

We report here the first published case of a pediatric patient with Gitelman’s syndrome (GS) in whom hypokalemia-associated rhabdomyolysis developed. A 13-year-old girl was admitted with weakness of the extremities, walking difficulty and calf pain. Laboratory data showed a serum potassium level of 2.1 mmol/l and a serum creatinine phosphokinase level of 1,248 IU/l plus myoglobinemia. The presence of normomagnesemia was the basis for a genetic analysis of the thiazide-sensitive sodium chloride cotransporter gene, which revealed compound heterozygous mutations in this gene. Prompt fluid expansion and potassium supplementation led to regression of the muscle symptoms. Hypokalemia can be a rare cause of rhabdomyolysis in patients with GS, even in childhood. We emphasize that genetic analysis is advisable to determine whether the suspicion of GS is warranted.     

Anaesthetic management of a child with Bartter’s syndrome

We report the anaesthetic management of an eight-year-old asthmatic boy with Bartter’s syndrome who had bilateral orchidopexy with caudal epidural analgesia. Bartter’s syndrome is a rare congenital disorder characterized by hypokalaemic hypochloraemic metabolic alkalosis, hyperaldosteronism, hyperreninaemia and hyperplasia of the juxtaglomerular apparatus of the kidneys. Characteristically, although these patients are normotensive they may be hypovolaemic. They may have unstable baroreceptor responses and show marked resistance to vasopressors. Hence, fluid, acid-base and electrolyte imbalances along with haemodynamic instability pose particular problems in their anaesthetic management. Previous case reports have described the management of these patients with general anaesthesia, our patient had his orchidopexy with caudal epidural analgesia using plain bupivacaine 0.5%. The patient was haemodynamically stable throughout surgery and was comfortable with caudal analgesia as the sole anaesthetic. Hypovalaemia, acid-base status and electrolyte imbalance were treated before instituting caudal epidural analgesia. We present this case report which describes the anaesthetic considerations in the light of the pathophysiology of Bartter’s syndrome.     

Anaesthetic management of a patient with Leigh’s syndrome

Purpose

Leigh’s syndrome, a progressive neurodegenerative disorder of infancy and childhood, is clinically charactenzed mainly by developmental delay, nervous system dysfunction and respiratory abnormalities such as aspiration, wheezing, breathing difficulties, gasping, hypoventilation and apnoea. Acute exacerbation and respiratory failure may follow surgery, general anaesthesia or intercurrent illnesses. Hyperlactataemia is variably present. Histopathological findings include necrosis, vascular proliferation, astrocytosis and demyelination of several brain areas. We present a 30-month-old patient with Leigh’s syndrome anaesthetized for extracorporeal shockwave lithotripsy, and describe the anaesthetic considerations.

Clinical features

Leigh’s syndrome was diagnosed at five months of age based on failure to thrive, lethargy, hypotonicity, choreo-athetosis and lactic acidaemia, with basal ganglia hypodense areas demonstrated by brain computerized tomographic scan. Muscle pyruvate dehydrogenase complex and NADH-coenzyme Q oxidoreductase activity were 25% and 13% of control. No preoperative respiratory symptoms or signs were present. Preoperative fasting lasted two hours and gastric aspiration was negative. Anaesthesia was induced with ketamine and midazolam im, and N₂O in oxygen, and maintained with propofol and N₂O. No volatile anaesthetics were used. Intravenous fluids given were 1/2 normal saline and glucose 5% administered. Besides laryngospasm dunng anaesthetic induction, relieved by sublingual succinylcholine injection, the perianaesthetic course was uneventful. The lungs were mechanically ventilated and lithotripsy was performed. No adverse sequelae have occurred, and the patient was discharged one day later.

Conclusion

Perioperative management of patients with Leigh’s syndrome requires cautious attention to the metabolic, neurological and respiratory aspects of the disease, and appropriate selection of anaesthetic drugs.     

Video-Assisted Thyroidectomy for Papillary Thyroid Carcinoma: Oncologic Outcome in Patients with Follow-Up ≥ 10 Years

Background

Video-assisted thyroidectomy (VAT) arisen as a valid treatment for selected patients with papillary thyroid carcinoma (PTC), but no data concerning long-term oncologic outcome are available. The primary aim of the study was to evaluate the oncologic outcome of patients who underwent VAT for PTC with a follow-up ≥ 10 years.

Methods

The medical charts of all the patients who successfully underwent VAT for PTC were reviewed. The patients with a minimum follow-up period of 120-months were included. Patients with unifocal PTC ≤ 1 cm, in the absence of lymph node metastases, without gross extracapsular invasion and age < 45 years were considered “low-risk” patients and followed with ultrasound and serum thyroglobulin (sTg) on levothyroxine (LT4); the remaining patients underwent nuclear medicine evaluation.

Results

Two hundred and fifty-seven patients, operated on between May 2000 and October 2006, were included. Postoperative complications included four transient recurrent palsies, 76 transient and 1 permanent hypocalcemia. One hundred and four low-risk patients were followed with ultrasound and sTg on LT4. At a mean follow-up of 136.6 months, mean sTg on LT4 was 0.1 ± 0.1 ng/ml. None of them showed recurrence. The remaining 153 patients underwent nuclear medicine evaluation. Among these 153, 62 did not undergo radioiodine ablation (RAI). At a mean follow-up of 150.8 months, mean sTg on LT4 was 0.1 ± 0.1 ng/ml. None of them showed recurrence. The remaining 91 patients underwent RAI. Mean pre-RAI sTg off-LT4 was 8.3 ± 5.8 ng/ml, mean radioiodine uptake was 2.8 ± 4.4%. Among these 91, three pN1a patients developed a lateral neck node recurrence. No other recurrence was registered. At the latest follow-up mean sTg on LT4 in this subgroup of patients was 0.1 ± 0.2 ng/ml.

Conclusions

The long-term (≥ 10 years) oncologic outcome further demonstrates that VAT is a valid option for selected PTC patients.

     

Gitelman’s syndrome associated with chondrocalcinosis: a case report

Abstract

Gitelman’s syndrome (GS) is a rare disease with autosomal recessive trait, characterized by hypokalemia, hypomagnesemia, metabolic alkalosis, hypocalciuria and hyperkinemic hyperaldosteronism. While muscle weakness, tetany, stomachache, nausea and fever are very common, it could sometimes be completely asymptomatic as is the case in our patient. It is generally benign, but some severe complications like growth retardation and, though rare, paralysis and cardiac arrest could also be seen. A 57-year-old male patient sent to our hospital for further examination because of hypokalemia was diagnosed with GS as a result of clinical and laboratory assessments. Potassium and magnesium replacement was started. We are presenting our case seeing that GS is not a syndrome to be overlooked as it bears a risk of severe complications, although it might be asymptomatic until advanced ages.     

Aneurysmal subarachnoid hemorrhage in a patient with Wegener’s granulomatosis

A 34-year-old Japanese man admitted to hospital with pneumonia had previously undergone surgery for paranasal sinusitis and also for the clipping of an aneurysm at the origin of the anterior choroidal artery after subarachnoid hemorrhage. Laboratory tests performed at the present admission showed renal insufficiency and serological findings of raised proteinase 3 antineutrophil cytoplasmic antibody level. A renal biopsy was performed that showed diffuse necrotizing glomerulonephritis with fibrocellular crescents. The diagnosis of Wegeners granulomatosis(WG) was confirmed on the basis of the clinical picture, laboratory findings, and biopsies of renal tissues. The disease responded to prednisolone and cyclophosphamide. The association of WG with a ruptured intracranial aneurysm is rare and has not previously been confirmed.     

Retrograde fibreoptic intubation in a child with Nager’s syndrome

Purpose

The authors describe a retrograde fibreoptic technique for tracheal intubation in a micrognathic child with a tracheo-cutaneous fistula.

Clinical features

A four-year-old child with Nager’s syndrome presented for surgical closure of a tracheocutaneous fistula. A tracheostomy tube had been placed in the neonatal period for management of upper airway obstruction due to severe micrognathia. At 2 1/2 yr of age, after a successful mandibular advancement procedure, the tracheostomy was removed and the child allowed to breathe through the natural airway. Preoperative physical examination revealed an uncooperative child, unable to open her mouth due to limited temporo-mandibular motion. The child was first anaesthetized with ketamine, 70 mg im, then halothane by mask. The authors were unable to open the child’s mouth sufficiently to allow rigid laryngoscopy. Attempts at oral and nasal fibreoptic intubation were unsuccessful. Ultimately, the authors were able to intubate nasally by passing an ultrathin Olympus LF-P laryngoscope under direct vision through the tracheocutaneous fistula in a cephalad direction, through the larynx and nasopharynx, then out the nares. An endotracheal tube was then advanced over the fibreoptic scope and positioned distal to the tracheocutaneous fistula. The surgical procedure was successfully accomplished and the trachea was extubated postoperatively without difficulty.

Conclusion

Retrograde fibreoptic intubation may be an option for airway management of a select group of children who cannot be intubated by traditional techniques.     

Mitochondrial DNA deletion in a girl with Fanconi’s syndrome

We report a sporadic large-scale mitochondrial deletion in a paediatric patient with Fanconi’s syndrome. Renal biopsy disclosed chronic interstitial nephritis. Ultrastructural examination of the renal tissue showed many giant atypical mitochondria. Histochemical stains revealed markedly reduced cytochrome c oxidase (COX). Genetic analysis disclosed a novel mitochondrial deletion of 7.3 kb in both peripheral blood and renal tissue. Mitochondrial diseases have heterogeneous clinical phenotypes; mutation analysis has proved to be an effective tool in confirming the diagnosis.