Dear Editor,
We read with interest the report in the present Journal of Edington M. et al [1] titled “Prescribing lessons from an ocular chemical injury: Vitaros inadvertently dispensed instead of VitA-POS”.
Erectile disfunction drugs play a role increasing levels of cyclic guanosine monophosphate (cGMP) with subsequent effects on nitric-oxide release. This condition can lead to acute angle-closure glaucoma (AACG) in case of anatomical predisposition. AACG is an ophthalmic emergency, it can lead to irreversible blindness if not identified and treated immediately and precipitating factors include certain drugs as nitrates, bronchodilators, cough mixtures, cold and flu medication, antidepressants, antihistamines and anticonvulsants [2]. Furthermore, a precedent case of AACG following sildenafil citrated therapy is also described [3].
We would like underline that this situation could lead to more serious effects, that only the mild chemical ocular injury, in presence of ophthalmic structural diseases.
References:
1. Edington M, Connolly J, Lockington D. Prescribing lessons from an ocular chemical injury: Vitaros inadvertently dispensed instead of VitA-POS. BMJ Case Rep. 2018 Dec 3;11(1). doi: 10.1136/bcr-2018-227468.
2. Murray D. Emergency management: angle-closure glaucoma. Community Eye Health. 2018;31(103):64.
3. Ramasamy B, Rowe F, Nayak H, Peckar C, Noonan C. Acute angle-closure glaucoma following sildenafil citrate-aided sexua...
Dear Editor,
We read with interest the report in the present Journal of Edington M. et al [1] titled “Prescribing lessons from an ocular chemical injury: Vitaros inadvertently dispensed instead of VitA-POS”.
Erectile disfunction drugs play a role increasing levels of cyclic guanosine monophosphate (cGMP) with subsequent effects on nitric-oxide release. This condition can lead to acute angle-closure glaucoma (AACG) in case of anatomical predisposition. AACG is an ophthalmic emergency, it can lead to irreversible blindness if not identified and treated immediately and precipitating factors include certain drugs as nitrates, bronchodilators, cough mixtures, cold and flu medication, antidepressants, antihistamines and anticonvulsants [2]. Furthermore, a precedent case of AACG following sildenafil citrated therapy is also described [3].
We would like underline that this situation could lead to more serious effects, that only the mild chemical ocular injury, in presence of ophthalmic structural diseases.
References:
1. Edington M, Connolly J, Lockington D. Prescribing lessons from an ocular chemical injury: Vitaros inadvertently dispensed instead of VitA-POS. BMJ Case Rep. 2018 Dec 3;11(1). doi: 10.1136/bcr-2018-227468.
2. Murray D. Emergency management: angle-closure glaucoma. Community Eye Health. 2018;31(103):64.
3. Ramasamy B, Rowe F, Nayak H, Peckar C, Noonan C. Acute angle-closure glaucoma following sildenafil citrate-aided sexual intercourse. Acta Ophthalmol Scand. 2007; 85: 229-230.
Influenza-induced rhabdomyolysis by Martin Brunnstrom et al 1 has called my attention because they refer to the patient's story "A man aged 29 years with a past medical history of cerebral palsy, seizures and chronic constipation".
The authors refer to "Other causes for rhabdomyolysis were investigated and excluded" and "A muscle biopsy ruling out an underlying metabolic myopathy or polymyositis to identify a risk factor for rhabdomyolysis was not obtained."
My comments are related to cerebral palsy and seizures.
Can these manifestations be warning signs of an underlying metabolic or mitochondrial disease?
Then the rhabdomyolysis could be a manifestation, for example of a mitochondrial disease exacerbated by an infection.
Cerebral palsy (CP) is defined by its nonprogressive features. Characterization of CP is traditionally based on the predominant quality of motor impairment (spastic, dyskinetic, ataxic-hypotonic or mixed, assessed on standard neurologic examination 2
A number of neurodegenerative, including metabolic and genetic disorders may present with similar symptoms and signs. 3
The presence of dyskinesia or spastic quadriplegic in clinical examination, in addition to abnormal findings in neuroimaging such as syrinx malformation, cerebellar hypoplasia, white matter paucity, and abnormal signal in basal ganglia may warrant further investigation for a disorder other than CP. As inborn errors...
Influenza-induced rhabdomyolysis by Martin Brunnstrom et al 1 has called my attention because they refer to the patient's story "A man aged 29 years with a past medical history of cerebral palsy, seizures and chronic constipation".
The authors refer to "Other causes for rhabdomyolysis were investigated and excluded" and "A muscle biopsy ruling out an underlying metabolic myopathy or polymyositis to identify a risk factor for rhabdomyolysis was not obtained."
My comments are related to cerebral palsy and seizures.
Can these manifestations be warning signs of an underlying metabolic or mitochondrial disease?
Then the rhabdomyolysis could be a manifestation, for example of a mitochondrial disease exacerbated by an infection.
Cerebral palsy (CP) is defined by its nonprogressive features. Characterization of CP is traditionally based on the predominant quality of motor impairment (spastic, dyskinetic, ataxic-hypotonic or mixed, assessed on standard neurologic examination 2
A number of neurodegenerative, including metabolic and genetic disorders may present with similar symptoms and signs. 3
The presence of dyskinesia or spastic quadriplegic in clinical examination, in addition to abnormal findings in neuroimaging such as syrinx malformation, cerebellar hypoplasia, white matter paucity, and abnormal signal in basal ganglia may warrant further investigation for a disorder other than CP. As inborn errors metabolism and mitocondrial diseases 4.
Some mitocondrial diseases underlying cerebral palsy 5
• Coenzyme Q10 deficiency: Spastic paresis; progressive ataxia and dystonia
• MELAS: Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes
• Pyruvate dehydrogenase deficiency: Spastic quadriplegia; dystonia
• Depletion Syndrome POLG1
Some disorders may be misdiagnosed as cerebral palsy 3:
• With apparent or real muscle weakness
• With significant dystonia/involuntary movements
• With predominant diplegia/tetraplegia
• With significant ataxia
References
1. Runnstrom M, Ebied AM, Khoury AP, Reddy R. Influenza-induced rhabdomyolysis. BMJ Case Rep. 2018;11:
2. Olney RS, Doernberg NS, Yeargin-Allsop M. Exclusion of Progressive Brain Disorders of Childhood for a Cerebral Palsy Monitoring System: A Public Health Perspective. J Registry Manag. 2014;41:182-9.
3. Appleton RE, Gupta R. Cerebral palsy: not always what it seems. Arch Dis Child 2001;85:356–360
4. Al-Jabri B, Goez H. Atypical Cerebral Palsy: Edmonton Experience. IJSR 2017;6: 1537-8
5. Leach EL, et al. Treatable inborn errors of metabolism presenting as cerebral palsy mimics: systematic literature review. Orphanet J Rare Dis. 2014;9:197
Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) are high-grade malignant tumours typically found in children and adolescents. These tumours belong to the family of small round cell tumours and are of neuro ectodermal origin Primary Ewing sarcoma (EWS) of the kidney is a rare tumor in adults. It was first described in 1975 by Seemayer and colleagues, and has since been sporadically documented in the literature Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) were originally described as two distinct pathologic entities, although both share common stem-cell precursor and unique chromosomal abnormality. Because of their similar histologic and cytogenetic characteristics, these tumors are now considered part of a spectrum of neoplastic diseases now known as Ewing’s sarcoma family tumors (ESFT), which also includes other malignancies The ESFT are most common in bone. Extraskeletal ESFT are less common and can affect the skin, soft tissue, or viscera So Renal primary sarcomas are a rare group of renal tumours. Ewing sarcoma/PNET of the kidney is distinctly rare, with more than 100 cases reported globally. Among these, leiomyo sarcoma is the most common (40–60%) followed by lipo sarcoma (10–15%) Sources of renal EWS include neural cells that invaginate into the kidney during its development some authors theorize that embryonic neural crest cells migrate into the kidney and undergo tumorigenesis It is primarily a genetic disease with case...
Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) are high-grade malignant tumours typically found in children and adolescents. These tumours belong to the family of small round cell tumours and are of neuro ectodermal origin Primary Ewing sarcoma (EWS) of the kidney is a rare tumor in adults. It was first described in 1975 by Seemayer and colleagues, and has since been sporadically documented in the literature Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) were originally described as two distinct pathologic entities, although both share common stem-cell precursor and unique chromosomal abnormality. Because of their similar histologic and cytogenetic characteristics, these tumors are now considered part of a spectrum of neoplastic diseases now known as Ewing’s sarcoma family tumors (ESFT), which also includes other malignancies The ESFT are most common in bone. Extraskeletal ESFT are less common and can affect the skin, soft tissue, or viscera So Renal primary sarcomas are a rare group of renal tumours. Ewing sarcoma/PNET of the kidney is distinctly rare, with more than 100 cases reported globally. Among these, leiomyo sarcoma is the most common (40–60%) followed by lipo sarcoma (10–15%) Sources of renal EWS include neural cells that invaginate into the kidney during its development some authors theorize that embryonic neural crest cells migrate into the kidney and undergo tumorigenesis It is primarily a genetic disease with cases affected sporadically, mainly a translocation type of mutation causing a fusion between the EWS gene on chromosome 22 and FLI1 on chromosome
Mean age at diagnosis of 30.4 years with the majority being male. Presentation of such tumors reported in the literature is non-specific and frequently involves localised pain which usually involves flank, subcostal or abdominal area with or without hematuria Differential diagnosis include Renal cell carcinoma and Wilm’s tumor ; neuroblastoma; malignant lymphoma ;metastatic renal involvement from sarcoma elsewhere in the body
No specific signs of PNET have been described in ultra sonography, computed tomography (CT), or magnetic resonance imaging (MRI). The imaging characteristics of most renal sarcomas are indistinguishable from those of RCC . These tumors appears as ill-defined, large heterogeneous masses with necrotic and hemorrhagic areas. Using ultrasonography, the tumour appears isoechogenic or hyperechogenic to the renal parenchyma. The CT findings include areas of internal haemorrhage or necrosis, peripheral hyper vascularity, and diffuse calcification. On MRI, the tumour appeared iso intense or hypo intense on T1-weighted images but heterogeneous intermediate to high T2 signal intensity . Venous extension into the renal vein, inferior vena cava, and right heart has been described .
Biopsy of the kidney mass show that Ewing sarcoma/primitive neuroectodermal tumor The pathologic findings shows usually multiple areas of necrosis and haemorrhage. In some cases, the tumour may exhibits calcifications and can be surrounded by a fibrous capsule. Microscopic features associated with ESFT/PNET are considered however non-specific as they can be seen in many other tumors, hence, the use of immunochemistry with CD99 over expression and cytogenetic analysis for definitive diagnosis Diagnosis is to be confirmed by fluorescence in situ hybridization (FISH) technique, which showed translocations involving the EWS locus (EWSR1 gene rearrangement).
We wish to share our clinical experience and support the importance of long term monitoring of thyroid function in patients with central congenital hypothyroidism caused by maternal thyrotoxicosis. This is a case series of three infants with prolonged hypothyroxinaemia unrelated to the initial management of neonatal Graves’ disease (NGD). In contrast to the minimal antenatal care reported in the case report. mothers of all infants in our case series had antenatal diagnosis of Graves’ disease with appropriate management and close follow up for signs of fetal hyperthyroidism. All infants were diagnosed with NGD within two weeks of birth with two infants being commenced on antithyroid medication for 2-4 weeks as they were symptomatic with NGD.
With resolution of NGD, thyroid function tests were monitored with subsequent hypothyroxinaemia noted between 4-8 weeks of age. Having confirmed persistent hypothyroxinaemia, all infants were commenced on thyroxine (4-10mcg/kg/day) with regular follow up of their thyroid function tests.
The development of hypothyroxinaemia after initial treatment of NGD is uncommon however has been described previously (1). In most cases, NGD remits by 3-12 weeks once maternal antibodies are cleared (2, 3). Early transient hypothyroxinaemia in infants of poorly controlled maternal Graves’ is well reported due to high circulating antibodies particularly in the third trimester (1, 4). Postulated mechanisms include suppression of the pituita...
We wish to share our clinical experience and support the importance of long term monitoring of thyroid function in patients with central congenital hypothyroidism caused by maternal thyrotoxicosis. This is a case series of three infants with prolonged hypothyroxinaemia unrelated to the initial management of neonatal Graves’ disease (NGD). In contrast to the minimal antenatal care reported in the case report. mothers of all infants in our case series had antenatal diagnosis of Graves’ disease with appropriate management and close follow up for signs of fetal hyperthyroidism. All infants were diagnosed with NGD within two weeks of birth with two infants being commenced on antithyroid medication for 2-4 weeks as they were symptomatic with NGD.
With resolution of NGD, thyroid function tests were monitored with subsequent hypothyroxinaemia noted between 4-8 weeks of age. Having confirmed persistent hypothyroxinaemia, all infants were commenced on thyroxine (4-10mcg/kg/day) with regular follow up of their thyroid function tests.
The development of hypothyroxinaemia after initial treatment of NGD is uncommon however has been described previously (1). In most cases, NGD remits by 3-12 weeks once maternal antibodies are cleared (2, 3). Early transient hypothyroxinaemia in infants of poorly controlled maternal Graves’ is well reported due to high circulating antibodies particularly in the third trimester (1, 4). Postulated mechanisms include suppression of the pituitary-thyroid axis due to fetal hyperthyroxinaemia caused by transplacental transfer of thyroxine in untreated hyperthyroid mothers or of thyroid-stimulating immunoglobulin from the mother (4, 5). It is not surprising the longstanding effects of poorly controlled maternal Graves’ disease given the early effects on thyroid function, with TSH receptors becoming responsive to TSH and to TSH-receptor antibodies around 20 weeks (6) and the hypothalamic-pituitary-thyroid axis continues to mature from the second half of gestation until one to two months postnatal.
The role of thyrotropin receptor antibodies has also been reported in the ‘switching’ between hypothyroidism to hyperthyroidism or vice versa. McLachlan et al described that varying concentration and affinities of thyroid stimulating versus blocking antibodies and immunological effect associated with the primary disease itself could contribute to ‘switching’ (7). Recovery of TSH levels tend to be delayed and has been reported to take up to 3-19 months in childhood Graves and up to 3¼ years in treated NGD (4).
Hypothyroxinaemia post initial management of NGD is an uncommon entity in addition to the rare background occurrence of NGD, but requires early recognition so that appropriate treatment may be instigated. There are known risk factors that predict the development of NGD but from this case series, there were no obvious factors that suggested the subsequent development of hypothyroxinaemia. It raises the importance of frequent clinical monitoring of growth and development supported by ongoing screening of TFTs in NGD.
References
1. Papendieck P, Chiesa A, Prieto L, Gruneiro-Papendieck L. Thyroid disorders of neonates born to mothers with Graves' disease. J Pediatr Endocrinol Metab. 2009;22(6):547-53.
2. Levy-Shraga Y, Tamir-Hostovsky L, Boyko V, Lerner-Geva L, Pinhas-Hamiel O. Follow-up of newborns of mothers with Graves' disease. Thyroid : official journal of the American Thyroid Association. 2014;24(6):1032-9.
3. Kamishlian A, Matthews N, Gupta A, Filipov P, Maclaren N, Anhalt H, et al. Different outcomes of neonatal thyroid function after Graves' disease in pregnancy: patient reports and literature review. J Pediatr Endocrinol Metab. 2005;18(12):1357-63.
4. Matsuura N, Harada S, Ohyama Y, Shibayama K, Fukushi M, Ishikawa N, et al. The mechanisms of transient hypothyroxinemia in infants born to mothers with Graves' disease. Pediatric research. 1997;42(2):214-8.
5. Higuchi R, Kumagai T, Kobayashi M, Minami T, Koyama H, Ishii Y. Short-term hyperthyroidism followed by transient pituitary hypothyroidism in a very low birth weight infant born to a mother with uncontrolled Graves' disease. Pediatrics. 2001;107(4):E57.
6. Polak M, Le Gac I, Vuillard E, Guibourdenche J, Leger J, Toubert ME, et al. Fetal and neonatal thyroid function in relation to maternal Graves' disease. Best practice & research Clinical endocrinology & metabolism. 2004;18(2):289-302.
7. McLachlan SM, Rapoport B. Thyrotropin-blocking autoantibodies and thyroid-stimulating autoantibodies: potential mechanisms involved in the pendulum swinging from hypothyroidism to hyperthyroidism or vice versa. Thyroid : official journal of the American Thyroid Association. 2013;23(1):14-24.
The terms Pancoast tumors, superior sulcus tumors, and superior pulmonary sulcus tumors are applied to neoplasms located at the apical pleuro pulmonary groove. In 1924, Henry K. Pancoast, described a patient afflicted with a lung carcinoma occupying the apical thoracic cavity that was associated with a constellation of symptoms that included shoulder pain radiating down the arm, atrophy of the hand muscles, and Horner’s syndrome.[ 1] Since then, it has become widely accepted that the term Pancoast syndrome can be applied to any clinical condition in which a neoplasm in the apex of a lung is accompanied by shoulder or arm pain. Anatomically, the definition includes any tumor invading through the parietal pleura at the level of the first rib and above. The pulmonary sulcus refers to the costo vertebral gutter extending from the first rib to the diaphragm. The superior pulmonary sulcus is therefore analogous to the superior most portion of this recess. The first rib is at the base of the thoracic inlet. The thoracic inlet contains the subclavian vein anteriorly, the subclavian artery, phrenic nerve and trunks of the brachial plexus medially, and the nerve roots of the brachial plexus and the stellate ganglion posteriorly. The bony thorax in the superior sulcus includes the upper ribs and the associated vertebral bodies. It is invasion of this complex anatomical area that accounts for the classic symptoms of the Pancoast tumor. Superior sulcus carcinomas have the same biologic...
The terms Pancoast tumors, superior sulcus tumors, and superior pulmonary sulcus tumors are applied to neoplasms located at the apical pleuro pulmonary groove. In 1924, Henry K. Pancoast, described a patient afflicted with a lung carcinoma occupying the apical thoracic cavity that was associated with a constellation of symptoms that included shoulder pain radiating down the arm, atrophy of the hand muscles, and Horner’s syndrome.[ 1] Since then, it has become widely accepted that the term Pancoast syndrome can be applied to any clinical condition in which a neoplasm in the apex of a lung is accompanied by shoulder or arm pain. Anatomically, the definition includes any tumor invading through the parietal pleura at the level of the first rib and above. The pulmonary sulcus refers to the costo vertebral gutter extending from the first rib to the diaphragm. The superior pulmonary sulcus is therefore analogous to the superior most portion of this recess. The first rib is at the base of the thoracic inlet. The thoracic inlet contains the subclavian vein anteriorly, the subclavian artery, phrenic nerve and trunks of the brachial plexus medially, and the nerve roots of the brachial plexus and the stellate ganglion posteriorly. The bony thorax in the superior sulcus includes the upper ribs and the associated vertebral bodies. It is invasion of this complex anatomical area that accounts for the classic symptoms of the Pancoast tumor. Superior sulcus carcinomas have the same biologic behavior as lung carcinomas located in the lung parenchyma. Consequently, their diagnosis, staging, and treatment follow the same principles as for any other lung cancer. The unique characteristics of Pancoast tumors are related to the anatomy of the region where these tumors occur (thoracic inlet) and not to their biologic behavior.
Epidemiology
Pancoast tumors are a relatively rare subset of non-small cell lung cancers (NSCLC), accounting for fewer than 5% of all lung cancers. At least 50% of cases are histologically seen as adenocarcinomas, while the rest are squamous cell and large-cell carcinomas. Small cell carcinoma occurs rare. Patients often present with complaints of pain distributed to the upper anterior chest wall. These tumors may manifest with signs and symptoms related to the compression or infiltration of the middle and lower trunks of the brachial plexus such as shoulder and arm pain (in the distribution of the C8, T1, and T2 dermatome). The peripheral location of these tumors minimizes standard lung cancer symptoms such as cough, hemoptysis, and dyspnea and is the main reason why patients with Pancoast tumors present at a later stage of diagnosis . Diagnosis is established through biopsy of the mass. Given their location, these lesions are amenable to CT or ultrasound-guided fine-needle aspiration. Because of the peripheral location of the tumor, fiberoptic bronchoscopy is only able to establish the diagnosis in less than 30% of cases (unless there is nodal involvement). A tissue diagnosis via video-assisted thoracoscopy (VATS) may be indicated when other investigations are negative and to eliminate the possibility of pleural metastatic disease. Axillary minithoractomy is an alternative to VATS to obtain a tissue diagnosis of the mass in small apical tumors.
As with other lung carcinomas located in the lung parenchyma, it is imperative to stage the mediastinum with Pancoast tumors. Metastases to the mediastinal nodes is a major negative prognostic factor — 5-year survival rates in the presence of N2 disease are below 10%.4 According to the 2013 ACCP guidelines, before surgery for Pancoast tumors, an endobronchial ultrasound with transbronchial needle aspiration or a cervical mediastinoscopy is warranted to exclude N2/N3 disease, even in the absence of involved nodes in CT or PET scans. Pancoast tumors are by definition T3 or T4 tumors. Most of the lesions are classified as T3 tumors because they invade only the chest wall and/or the sympathetic chain. The rest invade brachial plexus, vertebral bodies, and vascular structures, and are classified as T4 tumors. According to the new staging system for lung cancer developed by the International Association for the Study of Lung Cancer (IASLC), their final stage, in the absence of distant metastases, depends on the N status of the tumor:
IIB if T3NO,
IIIA if T3N1-2, or T4NO-1,
IIIB if T3N3 or T4N2-3.
Therefore, even the earliest of these lesions are staged at least IIB. Invasion of the ipsilateral supraclavicular nodes in the setting of lung cancer is classified as N3 disease. In superior sulcus carcinomas the importance of supraclavicular node involvement is quite different because it is considered to represent a locoregional lymph node extension.
References
1] Pancoast H.K. Superior pulmonary sulcus tumor. JAMA. 1932;99:1391.
Dear Editor,
We read with interest the report in the present Journal of Edington M. et al [1] titled “Prescribing lessons from an ocular chemical injury: Vitaros inadvertently dispensed instead of VitA-POS”.
Erectile disfunction drugs play a role increasing levels of cyclic guanosine monophosphate (cGMP) with subsequent effects on nitric-oxide release. This condition can lead to acute angle-closure glaucoma (AACG) in case of anatomical predisposition. AACG is an ophthalmic emergency, it can lead to irreversible blindness if not identified and treated immediately and precipitating factors include certain drugs as nitrates, bronchodilators, cough mixtures, cold and flu medication, antidepressants, antihistamines and anticonvulsants [2]. Furthermore, a precedent case of AACG following sildenafil citrated therapy is also described [3].
We would like underline that this situation could lead to more serious effects, that only the mild chemical ocular injury, in presence of ophthalmic structural diseases.
References:
1. Edington M, Connolly J, Lockington D. Prescribing lessons from an ocular chemical injury: Vitaros inadvertently dispensed instead of VitA-POS. BMJ Case Rep. 2018 Dec 3;11(1). doi: 10.1136/bcr-2018-227468.
2. Murray D. Emergency management: angle-closure glaucoma. Community Eye Health. 2018;31(103):64.
3. Ramasamy B, Rowe F, Nayak H, Peckar C, Noonan C. Acute angle-closure glaucoma following sildenafil citrate-aided sexua...
Dear Editor,
We read with interest the report in the present Journal of Edington M. et al [1] titled “Prescribing lessons from an ocular chemical injury: Vitaros inadvertently dispensed instead of VitA-POS”.
Erectile disfunction drugs play a role increasing levels of cyclic guanosine monophosphate (cGMP) with subsequent effects on nitric-oxide release. This condition can lead to acute angle-closure glaucoma (AACG) in case of anatomical predisposition. AACG is an ophthalmic emergency, it can lead to irreversible blindness if not identified and treated immediately and precipitating factors include certain drugs as nitrates, bronchodilators, cough mixtures, cold and flu medication, antidepressants, antihistamines and anticonvulsants [2]. Furthermore, a precedent case of AACG following sildenafil citrated therapy is also described [3].
We would like underline that this situation could lead to more serious effects, that only the mild chemical ocular injury, in presence of ophthalmic structural diseases.
References:
1. Edington M, Connolly J, Lockington D. Prescribing lessons from an ocular chemical injury: Vitaros inadvertently dispensed instead of VitA-POS. BMJ Case Rep. 2018 Dec 3;11(1). doi: 10.1136/bcr-2018-227468.
2. Murray D. Emergency management: angle-closure glaucoma. Community Eye Health. 2018;31(103):64.
3. Ramasamy B, Rowe F, Nayak H, Peckar C, Noonan C. Acute angle-closure glaucoma following sildenafil citrate-aided sexual intercourse. Acta Ophthalmol Scand. 2007; 85: 229-230.
The terms Pancoast tumors, superior sulcus tumors, and superior pulmonary sulcus tumors are applied to neoplasms located at the apical pleuro pulmonary groove. In 1924, Henry K. Pancoast, described a patient afflicted with a lung carcinoma occupying the apical thoracic cavity that was associated with a constellation of symptoms that included shoulder pain radiating down the arm, atrophy of the hand muscles, and Horner’s syndrome.[ 1] Since then, it has become widely accepted that the term Pancoast syndrome can be applied to any clinical condition in which a neoplasm in the apex of a lung is accompanied by shoulder or arm pain. Anatomically, the definition includes any tumor invading through the parietal pleura at the level of the first rib and above. The pulmonary sulcus refers to the costo vertebral gutter extending from the first rib to the diaphragm. The superior pulmonary sulcus is therefore analogous to the superior most portion of this recess. The first rib is at the base of the thoracic inlet. The thoracic inlet contains the subclavian vein anteriorly, the subclavian artery, phrenic nerve and trunks of the brachial plexus medially, and the nerve roots of the brachial plexus and the stellate ganglion posteriorly. The bony thorax in the superior sulcus includes the upper ribs and the associated vertebral bodies. It is invasion of this complex anatomical area that accounts for the classic symptoms of the Pancoast tumor. Superior sulcus carcinomas have the same biologic...
The terms Pancoast tumors, superior sulcus tumors, and superior pulmonary sulcus tumors are applied to neoplasms located at the apical pleuro pulmonary groove. In 1924, Henry K. Pancoast, described a patient afflicted with a lung carcinoma occupying the apical thoracic cavity that was associated with a constellation of symptoms that included shoulder pain radiating down the arm, atrophy of the hand muscles, and Horner’s syndrome.[ 1] Since then, it has become widely accepted that the term Pancoast syndrome can be applied to any clinical condition in which a neoplasm in the apex of a lung is accompanied by shoulder or arm pain. Anatomically, the definition includes any tumor invading through the parietal pleura at the level of the first rib and above. The pulmonary sulcus refers to the costo vertebral gutter extending from the first rib to the diaphragm. The superior pulmonary sulcus is therefore analogous to the superior most portion of this recess. The first rib is at the base of the thoracic inlet. The thoracic inlet contains the subclavian vein anteriorly, the subclavian artery, phrenic nerve and trunks of the brachial plexus medially, and the nerve roots of the brachial plexus and the stellate ganglion posteriorly. The bony thorax in the superior sulcus includes the upper ribs and the associated vertebral bodies. It is invasion of this complex anatomical area that accounts for the classic symptoms of the Pancoast tumor. Superior sulcus carcinomas have the same biologic behavior as lung carcinomas located in the lung parenchyma. Consequently, their diagnosis, staging, and treatment follow the same principles as for any other lung cancer. The unique characteristics of Pancoast tumors are related to the anatomy of the region where these tumors occur (thoracic inlet) and not to their biologic behavior.
Epidemiology
Pancoast tumors are a relatively rare subset of non-small cell lung cancers (NSCLC), accounting for fewer than 5% of all lung cancers. At least 50% of cases are histologically seen as adenocarcinomas, while the rest are squamous cell and large-cell carcinomas. Small cell carcinoma occurs rare. Patients often present with complaints of pain distributed to the upper anterior chest wall. These tumors may manifest with signs and symptoms related to the compression or infiltration of the middle and lower trunks of the brachial plexus such as shoulder and arm pain (in the distribution of the C8, T1, and T2 dermatome). The peripheral location of these tumors minimizes standard lung cancer symptoms such as cough, hemoptysis, and dyspnea and is the main reason why patients with Pancoast tumors present at a later stage of diagnosis . Diagnosis is established through biopsy of the mass. Given their location, these lesions are amenable to CT or ultrasound-guided fine-needle aspiration. Because of the peripheral location of the tumor, fiberoptic bronchoscopy is only able to establish the diagnosis in less than 30% of cases (unless there is nodal involvement). A tissue diagnosis via video-assisted thoracoscopy (VATS) may be indicated when other investigations are negative and to eliminate the possibility of pleural metastatic disease. Axillary minithoractomy is an alternative to VATS to obtain a tissue diagnosis of the mass in small apical tumors.
As with other lung carcinomas located in the lung parenchyma, it is imperative to stage the mediastinum with Pancoast tumors. Metastases to the mediastinal nodes is a major negative prognostic factor — 5-year survival rates in the presence of N2 disease are below 10%.4 According to the 2013 ACCP guidelines, before surgery for Pancoast tumors, an endobronchial ultrasound with transbronchial needle aspiration or a cervical mediastinoscopy is warranted to exclude N2/N3 disease, even in the absence of involved nodes in CT or PET scans. Pancoast tumors are by definition T3 or T4 tumors. Most of the lesions are classified as T3 tumors because they invade only the chest wall and/or the sympathetic chain. The rest invade brachial plexus, vertebral bodies, and vascular structures, and are classified as T4 tumors. According to the new staging system for lung cancer developed by the International Association for the Study of Lung Cancer (IASLC), their final stage, in the absence of distant metastases, depends on the N status of the tumor:
IIB if T3NO,
IIIA if T3N1-2, or T4NO-1,
IIIB if T3N3 or T4N2-3.
Therefore, even the earliest of these lesions are staged at least IIB. Invasion of the ipsilateral supraclavicular nodes in the setting of lung cancer is classified as N3 disease. In superior sulcus carcinomas the importance of supraclavicular node involvement is quite different because it is considered to represent a locoregional lymph node extension.
References
1] Pancoast H.K. Superior pulmonary sulcus tumor. JAMA. 1932;99:1391.
This prescribing-dispensing error is unusual in that no-one spotted the obvious mistake. Superficially it would seem that recommending that handwritten prescriptions are in capital letters would improve safety, but this could introduce a different type of error, that is more common. Calligraphers know from experience that attempting to use an unfamiliar upper-case style is harder and distracting. Concentrating on forming the letters takes attention away from the content and before you know it you’ve just written a perfectly formed but incorrect letter. There is no research to transfer knowledge from this craft to prescribing, but the danger is that by asking prescribers to focus on an unfamiliar style of writing diverts attention from getting the correct drug name. One of the commonest and most dangerous errors is simply prescribing the wrong drug. This is easy to do when two very different drugs have similar names, as in the case report. So common is this potentially serious error, that previously the RCGP Quality Unit in collaboration with ten other organisations, including universities, indemnity providers, and colleges, issued a pamphlet “In Safer Hands” and sent it to every GP in the country pointing out this danger. Despite the huge collaboration, of the eighteen drugs given as examples of high-risk similar names, three were misspelt. All capitals might improve legibility of a drug name, the receiving end of the communication, but at the cost of damaging the transmis...
This prescribing-dispensing error is unusual in that no-one spotted the obvious mistake. Superficially it would seem that recommending that handwritten prescriptions are in capital letters would improve safety, but this could introduce a different type of error, that is more common. Calligraphers know from experience that attempting to use an unfamiliar upper-case style is harder and distracting. Concentrating on forming the letters takes attention away from the content and before you know it you’ve just written a perfectly formed but incorrect letter. There is no research to transfer knowledge from this craft to prescribing, but the danger is that by asking prescribers to focus on an unfamiliar style of writing diverts attention from getting the correct drug name. One of the commonest and most dangerous errors is simply prescribing the wrong drug. This is easy to do when two very different drugs have similar names, as in the case report. So common is this potentially serious error, that previously the RCGP Quality Unit in collaboration with ten other organisations, including universities, indemnity providers, and colleges, issued a pamphlet “In Safer Hands” and sent it to every GP in the country pointing out this danger. Despite the huge collaboration, of the eighteen drugs given as examples of high-risk similar names, three were misspelt. All capitals might improve legibility of a drug name, the receiving end of the communication, but at the cost of damaging the transmission accuracy.
Thanks for reporting this interesting case of VAD.
The clinical versatility of this pathology calls for careful history
taking and examination to avoid the pitfall of inappropriate imaging
requests for patients who present with craniocervical pain with or without
headache.
The presence of new neurological deficits such as those described in
the case report would certainly warrant urgent imaging.
In the absence of...
Thanks for reporting this interesting case of VAD.
The clinical versatility of this pathology calls for careful history
taking and examination to avoid the pitfall of inappropriate imaging
requests for patients who present with craniocervical pain with or without
headache.
The presence of new neurological deficits such as those described in
the case report would certainly warrant urgent imaging.
In the absence of these,strong clinical concern by itself may not be
enough but should be supported by significant risk factors to justify
imaging,this would protect patients from undue radiation exposure and its
inherent risks,as some institutions tend to conduct a CT-brain prior to
MRA.
Thank you
I thank Drs. Onder and Jahanroshan for their interest in this report.
They raise excellent points. A video would have added a great deal,
however I was unable to locate proxies to consent for such a recording
during the time of what was a short lived tremor. An EEG done prior to
the appearance of the tremor showed global slowing with no epileptiform
activity. I can't exclude self limited hypoxia prior to being found bu...
I thank Drs. Onder and Jahanroshan for their interest in this report.
They raise excellent points. A video would have added a great deal,
however I was unable to locate proxies to consent for such a recording
during the time of what was a short lived tremor. An EEG done prior to
the appearance of the tremor showed global slowing with no epileptiform
activity. I can't exclude self limited hypoxia prior to being found but
his oxygenation was monitored and adequate at least from when he was found
breathing until after resolution of the tremor. Priopriospinal myoclonus
is certainly a consideration, though the explanation is not parsimonious.
Phenotypically, the tremor was nearly identical to the cited report of the
rest tremor in brain death which did include a video. Specifically, it
involved rhythmic finger flexion/extension with a pill rolling quality
which would have been unremarkable in a person with idiopathic Parkinson
disease.
Regards,
Laura S. Boylan, MD
Dear Editor,
We read with interest the report in the present Journal of Edington M. et al [1] titled “Prescribing lessons from an ocular chemical injury: Vitaros inadvertently dispensed instead of VitA-POS”.
Erectile disfunction drugs play a role increasing levels of cyclic guanosine monophosphate (cGMP) with subsequent effects on nitric-oxide release. This condition can lead to acute angle-closure glaucoma (AACG) in case of anatomical predisposition. AACG is an ophthalmic emergency, it can lead to irreversible blindness if not identified and treated immediately and precipitating factors include certain drugs as nitrates, bronchodilators, cough mixtures, cold and flu medication, antidepressants, antihistamines and anticonvulsants [2]. Furthermore, a precedent case of AACG following sildenafil citrated therapy is also described [3].
We would like underline that this situation could lead to more serious effects, that only the mild chemical ocular injury, in presence of ophthalmic structural diseases.
References:
Show More1. Edington M, Connolly J, Lockington D. Prescribing lessons from an ocular chemical injury: Vitaros inadvertently dispensed instead of VitA-POS. BMJ Case Rep. 2018 Dec 3;11(1). doi: 10.1136/bcr-2018-227468.
2. Murray D. Emergency management: angle-closure glaucoma. Community Eye Health. 2018;31(103):64.
3. Ramasamy B, Rowe F, Nayak H, Peckar C, Noonan C. Acute angle-closure glaucoma following sildenafil citrate-aided sexua...
Influenza-induced rhabdomyolysis by Martin Brunnstrom et al 1 has called my attention because they refer to the patient's story "A man aged 29 years with a past medical history of cerebral palsy, seizures and chronic constipation".
Show MoreThe authors refer to "Other causes for rhabdomyolysis were investigated and excluded" and "A muscle biopsy ruling out an underlying metabolic myopathy or polymyositis to identify a risk factor for rhabdomyolysis was not obtained."
My comments are related to cerebral palsy and seizures.
Can these manifestations be warning signs of an underlying metabolic or mitochondrial disease?
Then the rhabdomyolysis could be a manifestation, for example of a mitochondrial disease exacerbated by an infection.
Cerebral palsy (CP) is defined by its nonprogressive features. Characterization of CP is traditionally based on the predominant quality of motor impairment (spastic, dyskinetic, ataxic-hypotonic or mixed, assessed on standard neurologic examination 2
A number of neurodegenerative, including metabolic and genetic disorders may present with similar symptoms and signs. 3
The presence of dyskinesia or spastic quadriplegic in clinical examination, in addition to abnormal findings in neuroimaging such as syrinx malformation, cerebellar hypoplasia, white matter paucity, and abnormal signal in basal ganglia may warrant further investigation for a disorder other than CP. As inborn errors...
Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) are high-grade malignant tumours typically found in children and adolescents. These tumours belong to the family of small round cell tumours and are of neuro ectodermal origin Primary Ewing sarcoma (EWS) of the kidney is a rare tumor in adults. It was first described in 1975 by Seemayer and colleagues, and has since been sporadically documented in the literature Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) were originally described as two distinct pathologic entities, although both share common stem-cell precursor and unique chromosomal abnormality. Because of their similar histologic and cytogenetic characteristics, these tumors are now considered part of a spectrum of neoplastic diseases now known as Ewing’s sarcoma family tumors (ESFT), which also includes other malignancies The ESFT are most common in bone. Extraskeletal ESFT are less common and can affect the skin, soft tissue, or viscera So Renal primary sarcomas are a rare group of renal tumours. Ewing sarcoma/PNET of the kidney is distinctly rare, with more than 100 cases reported globally. Among these, leiomyo sarcoma is the most common (40–60%) followed by lipo sarcoma (10–15%) Sources of renal EWS include neural cells that invaginate into the kidney during its development some authors theorize that embryonic neural crest cells migrate into the kidney and undergo tumorigenesis It is primarily a genetic disease with case...
Show MoreWe wish to share our clinical experience and support the importance of long term monitoring of thyroid function in patients with central congenital hypothyroidism caused by maternal thyrotoxicosis. This is a case series of three infants with prolonged hypothyroxinaemia unrelated to the initial management of neonatal Graves’ disease (NGD). In contrast to the minimal antenatal care reported in the case report. mothers of all infants in our case series had antenatal diagnosis of Graves’ disease with appropriate management and close follow up for signs of fetal hyperthyroidism. All infants were diagnosed with NGD within two weeks of birth with two infants being commenced on antithyroid medication for 2-4 weeks as they were symptomatic with NGD.
With resolution of NGD, thyroid function tests were monitored with subsequent hypothyroxinaemia noted between 4-8 weeks of age. Having confirmed persistent hypothyroxinaemia, all infants were commenced on thyroxine (4-10mcg/kg/day) with regular follow up of their thyroid function tests.
The development of hypothyroxinaemia after initial treatment of NGD is uncommon however has been described previously (1). In most cases, NGD remits by 3-12 weeks once maternal antibodies are cleared (2, 3). Early transient hypothyroxinaemia in infants of poorly controlled maternal Graves’ is well reported due to high circulating antibodies particularly in the third trimester (1, 4). Postulated mechanisms include suppression of the pituita...
Show MoreThe terms Pancoast tumors, superior sulcus tumors, and superior pulmonary sulcus tumors are applied to neoplasms located at the apical pleuro pulmonary groove. In 1924, Henry K. Pancoast, described a patient afflicted with a lung carcinoma occupying the apical thoracic cavity that was associated with a constellation of symptoms that included shoulder pain radiating down the arm, atrophy of the hand muscles, and Horner’s syndrome.[ 1] Since then, it has become widely accepted that the term Pancoast syndrome can be applied to any clinical condition in which a neoplasm in the apex of a lung is accompanied by shoulder or arm pain. Anatomically, the definition includes any tumor invading through the parietal pleura at the level of the first rib and above. The pulmonary sulcus refers to the costo vertebral gutter extending from the first rib to the diaphragm. The superior pulmonary sulcus is therefore analogous to the superior most portion of this recess. The first rib is at the base of the thoracic inlet. The thoracic inlet contains the subclavian vein anteriorly, the subclavian artery, phrenic nerve and trunks of the brachial plexus medially, and the nerve roots of the brachial plexus and the stellate ganglion posteriorly. The bony thorax in the superior sulcus includes the upper ribs and the associated vertebral bodies. It is invasion of this complex anatomical area that accounts for the classic symptoms of the Pancoast tumor. Superior sulcus carcinomas have the same biologic...
Show MoreDear Editor,
We read with interest the report in the present Journal of Edington M. et al [1] titled “Prescribing lessons from an ocular chemical injury: Vitaros inadvertently dispensed instead of VitA-POS”.
Erectile disfunction drugs play a role increasing levels of cyclic guanosine monophosphate (cGMP) with subsequent effects on nitric-oxide release. This condition can lead to acute angle-closure glaucoma (AACG) in case of anatomical predisposition. AACG is an ophthalmic emergency, it can lead to irreversible blindness if not identified and treated immediately and precipitating factors include certain drugs as nitrates, bronchodilators, cough mixtures, cold and flu medication, antidepressants, antihistamines and anticonvulsants [2]. Furthermore, a precedent case of AACG following sildenafil citrated therapy is also described [3].
We would like underline that this situation could lead to more serious effects, that only the mild chemical ocular injury, in presence of ophthalmic structural diseases.
References:
Show More1. Edington M, Connolly J, Lockington D. Prescribing lessons from an ocular chemical injury: Vitaros inadvertently dispensed instead of VitA-POS. BMJ Case Rep. 2018 Dec 3;11(1). doi: 10.1136/bcr-2018-227468.
2. Murray D. Emergency management: angle-closure glaucoma. Community Eye Health. 2018;31(103):64.
3. Ramasamy B, Rowe F, Nayak H, Peckar C, Noonan C. Acute angle-closure glaucoma following sildenafil citrate-aided sexua...
The terms Pancoast tumors, superior sulcus tumors, and superior pulmonary sulcus tumors are applied to neoplasms located at the apical pleuro pulmonary groove. In 1924, Henry K. Pancoast, described a patient afflicted with a lung carcinoma occupying the apical thoracic cavity that was associated with a constellation of symptoms that included shoulder pain radiating down the arm, atrophy of the hand muscles, and Horner’s syndrome.[ 1] Since then, it has become widely accepted that the term Pancoast syndrome can be applied to any clinical condition in which a neoplasm in the apex of a lung is accompanied by shoulder or arm pain. Anatomically, the definition includes any tumor invading through the parietal pleura at the level of the first rib and above. The pulmonary sulcus refers to the costo vertebral gutter extending from the first rib to the diaphragm. The superior pulmonary sulcus is therefore analogous to the superior most portion of this recess. The first rib is at the base of the thoracic inlet. The thoracic inlet contains the subclavian vein anteriorly, the subclavian artery, phrenic nerve and trunks of the brachial plexus medially, and the nerve roots of the brachial plexus and the stellate ganglion posteriorly. The bony thorax in the superior sulcus includes the upper ribs and the associated vertebral bodies. It is invasion of this complex anatomical area that accounts for the classic symptoms of the Pancoast tumor. Superior sulcus carcinomas have the same biologic...
Show MoreThis prescribing-dispensing error is unusual in that no-one spotted the obvious mistake. Superficially it would seem that recommending that handwritten prescriptions are in capital letters would improve safety, but this could introduce a different type of error, that is more common. Calligraphers know from experience that attempting to use an unfamiliar upper-case style is harder and distracting. Concentrating on forming the letters takes attention away from the content and before you know it you’ve just written a perfectly formed but incorrect letter. There is no research to transfer knowledge from this craft to prescribing, but the danger is that by asking prescribers to focus on an unfamiliar style of writing diverts attention from getting the correct drug name. One of the commonest and most dangerous errors is simply prescribing the wrong drug. This is easy to do when two very different drugs have similar names, as in the case report. So common is this potentially serious error, that previously the RCGP Quality Unit in collaboration with ten other organisations, including universities, indemnity providers, and colleges, issued a pamphlet “In Safer Hands” and sent it to every GP in the country pointing out this danger. Despite the huge collaboration, of the eighteen drugs given as examples of high-risk similar names, three were misspelt. All capitals might improve legibility of a drug name, the receiving end of the communication, but at the cost of damaging the transmis...
Show MoreThanks for reporting this interesting case of VAD. The clinical versatility of this pathology calls for careful history taking and examination to avoid the pitfall of inappropriate imaging requests for patients who present with craniocervical pain with or without headache.
The presence of new neurological deficits such as those described in the case report would certainly warrant urgent imaging. In the absence of...
I thank Drs. Onder and Jahanroshan for their interest in this report. They raise excellent points. A video would have added a great deal, however I was unable to locate proxies to consent for such a recording during the time of what was a short lived tremor. An EEG done prior to the appearance of the tremor showed global slowing with no epileptiform activity. I can't exclude self limited hypoxia prior to being found bu...
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