Anaesthetic challenges in perioperative management of thoracic paraganglioma in an 8-year-old child

2021 ◽  
Vol 14 (8) ◽  
pp. e243521
Author(s):  
Ram Gopal maurya ◽  
Shefali Gautam ◽  
Gyan Prakash Singh ◽  
Chaitra srinivas

Pheochromocytomas (PCC) are catecholamine-secreting tumours that arise from chromaffin cells of the sympathoadrenal system. These rare catecholamine-secreting tumours arising from adrenal glands are termed as PCC and those from extra-­adrenal sites are termed paraganglioma (PGL). Thoracic PGL is very rarely found. we report the anaesthetic challenges and management of an 8-year-old child with functional thoracic PGL and its successful outcome.

Author(s):  
H.B. Pollard ◽  
C.E. Creutz ◽  
C.J. Pazoles ◽  
J.H. Scott

Exocytosis is a general concept describing secretion of enzymes, hormones and transmitters that are otherwise sequestered in intracellular granules. Chemical evidence for this concept was first gathered from studies on chromaffin cells in perfused adrenal glands, in which it was found that granule contents, including both large protein and small molecules such as adrenaline and ATP, were released together while the granule membrane was retained in the cell. A number of exhaustive reviews of this early work have been published and are summarized in Reference 1. The critical experiments demonstrating the importance of extracellular calcium for exocytosis per se were also first performed in this system (2,3), further indicating the substantial service given by chromaffin cells to those interested in secretory phenomena over the years.


1982 ◽  
Vol 242 (3) ◽  
pp. E137-E145 ◽  
Author(s):  
M. H. Carvalho ◽  
J. C. Prat ◽  
A. G. Garcia ◽  
S. M. Kirpekar

Ionomycin, a polyether antibiotic, stimulated the secretion of catecholamines and dopamine beta-hydroxylase from perfused adrenal glands and [3H]norepinephrine ([3H]NE) from spleens of the cat. Release was calcium dependent, and strontium or barium did not substitute for calcium. Ionomycin failed to release [3H]NE from reserpinized spleens. High magnesium did not interfere in the ionomycin response, but lanthanum and manganese blocked it. Ionomycin response that was pH dependent was not affected by potassium depolarization. The secretory response to ionomycin was enhanced when both glycolysis and oxidative metabolism were inhibited. It is concluded that ionomycin introduces calcium into the chromaffin cells and adrenergic nerve terminals to cause the secretory response and that a rise in intracellular calcium may be an adequate stimulus for secretion.


2017 ◽  
Author(s):  
Abbas Al-Kurd ◽  
Haggi Mazeh

The adrenal glands represent an essential component of the endocrine system, and their failure can have catastrophic consequences to several aspects of bodily homeostasis. Each adrenal gland can be divided into two different endocrine components, the cortex and the medulla, each with distinct functions. This in-depth review of normal adrenal embryology, anatomy, and physiology also emphasizes the clinical relevance of various irregularities in adrenal functioning. Every surgeon attempting to manage adrenal diseases is expected to be familiar with the detailed pathophysiology of these conditions because such an understanding is essential for sound preoperative evaluation and perioperative management of this potentially complicated patient group.  This review contains 4 figures, 1 table, and 70 references. Key words: adrenal, adrenal glands, adrenal pathophysiology, adrenal physiology, anatomy of adrenal glands, cortex, embryology, endocrine system, medulla


2017 ◽  
Author(s):  
Abbas Al-Kurd ◽  
Haggi Mazeh

The adrenal glands represent an essential component of the endocrine system, and their failure can have catastrophic consequences to several aspects of bodily homeostasis. Each adrenal gland can be divided into two different endocrine components, the cortex and the medulla, each with distinct functions. This in-depth review of normal adrenal embryology, anatomy, and physiology also emphasizes the clinical relevance of various irregularities in adrenal functioning. Every surgeon attempting to manage adrenal diseases is expected to be familiar with the detailed pathophysiology of these conditions because such an understanding is essential for sound preoperative evaluation and perioperative management of this potentially complicated patient group.  This review contains 4 figures, 1 table, and 70 references. Key words: adrenal, adrenal glands, adrenal pathophysiology, adrenal physiology, anatomy of adrenal glands, cortex, embryology, endocrine system, medulla


2017 ◽  
Author(s):  
Abbas Al-Kurd ◽  
Haggi Mazeh

The adrenal glands represent an essential component of the endocrine system, and their failure can have catastrophic consequences to several aspects of bodily homeostasis. Each adrenal gland can be divided into two different endocrine components, the cortex and the medulla, each with distinct functions. This in-depth review of normal adrenal embryology, anatomy, and physiology also emphasizes the clinical relevance of various irregularities in adrenal functioning. Every surgeon attempting to manage adrenal diseases is expected to be familiar with the detailed pathophysiology of these conditions because such an understanding is essential for sound preoperative evaluation and perioperative management of this potentially complicated patient group.  This review contains 4 figures, 1 table, and 70 references. Key words: adrenal, adrenal glands, adrenal pathophysiology, adrenal physiology, anatomy of adrenal glands, cortex, embryology, endocrine system, medulla


2021 ◽  
Vol 36 (1) ◽  
Author(s):  
Soha Zahid ◽  
Ahtesham Khizar

Abstract Background An encephalocele is a congenital neural tube defect characterized by herniation of cranial contents through a defect in the cranium and is caused by failure of the closure of the cranial part of the developing neural tube. An encephalocele is termed as “giant encephalocele” when the size of encephalocele is larger than the size of the head. They depend on size of the sac, percentage of neural tissue content, hydrocephalus, infection, and other associated pathologies for a favorable neurological outcome. Case presentation We report a case of a four-month-old boy with a giant occipital encephalocele measuring 21 × 15 × 19 cm in size, which was a surgical and anesthetic challenge for us. Intubation was achieved in lateral position. Part of occipital and cerebellar parenchyma was present in the sac and bony defect was approximately 2.5 cm in occipital bone in midline. We performed surgical excision and repair with a good overall outcome. Conclusion Perioperative management of a giant occipital encephalocele is a challenge for both anesthesiologists and neurosurgeons. Managing such a case demands a search for other congenital abnormalities, expertise in handling airway, and proper intraoperative care. Careful planning and perioperative management are essential for a successful outcome.


1988 ◽  
Vol 116 (1) ◽  
pp. 149-NP ◽  
Author(s):  
M. Jousselin-Hosaja

ABSTRACT The effects of long-term transplantation on the ultrastructure of adrenaline- and noradrenaline-storing cells from the adrenal medulla were determined using morphometric methods. Mouse adrenal medulla were freed from the adrenal cortex and grafted into the occipital cortex of the brain. Two types of chromaffin cells were identified by electron microscopy in grafts fixed with glutaraldehyde and osmium tetroxide. Noradrenaline-type cells were predominant and formed 70–80% of the surviving population of grafted chromaffin cells. A minority of the chromaffin cells contained medium-sized granules (140–210 nm in diameter) (medium granule cell; MGC) with finely granular moderately electron dense cores. Morphometric analysis of noradrenaline phenotype cells and MGC cells in transplants showed no significant differences compared with the noradrenaline-storing cells of normal adrenal glands. In contrast, noradrenaline-type cells and MGC cells in the grafts had areas of secretory vesicles which were significantly (P<0·01) larger and areas of rough endoplasmic reticulum which were significantly (P<0 ·01) smaller than those of the adrenaline-storing cells of normal adrenal glands. It was concluded that long-term transplantation caused no degenerative changes in the ultrastructure of mouse adrenal chromaffin cells. J. Endocr. (1988) 116, 149–153


Development ◽  
2002 ◽  
Vol 129 (20) ◽  
pp. 4729-4738 ◽  
Author(s):  
Katrin Huber ◽  
Barbara Brühl ◽  
François Guillemot ◽  
Eric N. Olson ◽  
Uwe Ernsberger ◽  
...  

The sympathoadrenal (SA) cell lineage is a derivative of the neural crest (NC), which gives rise to sympathetic neurons and neuroendocrine chromaffin cells. Signals that are important for specification of these two types of cells are largely unknown. MASH1 plays an important role for neuronal as well as catecholaminergic differentiation. Mash1 knockout mice display severe deficits in sympathetic ganglia, yet their adrenal medulla has been reported to be largely normal suggesting that MASH1 is essential for neuronal but not for neuroendocrine differentiation. We show now that MASH1 function is necessary for the development of the vast majority of chromaffin cells. Most adrenal medullary cells in Mash1–/– mice identified by Phox2b immunoreactivity, lack the catecholaminergic marker tyrosine hydroxylase. Mash1 mutant and wild-type mice have almost identical numbers of Phox2b-positive cells in their adrenal glands at embryonic day (E) 13.5; however, only one-third of the Phox2b-positive adrenal cell population seen in Mash1+/+ mice is maintained in Mash1–/– mice at birth. Similar to Phox2b, cells expressing Phox2a and Hand2 (dHand) clearly outnumber TH-positive cells. Most cells in the adrenal medulla of Mash1–/– mice do not contain chromaffin granules, display a very immature, neuroblast-like phenotype, and, unlike wild-type adrenal chromaffin cells, show prolonged expression of neurofilament and Ret comparable with that observed in wild-type sympathetic ganglia. However, few chromaffin cells in Mash1–/– mice become PNMT positive and downregulate neurofilament and Ret expression. Together, these findings suggest that the development of chomaffin cells does depend on MASH1 function not only for catecholaminergic differentiation but also for general chromaffin cell differentiation.


1992 ◽  
Vol 40 (7) ◽  
pp. 1043-1045 ◽  
Author(s):  
A S Tischler ◽  
L A Ruzicka ◽  
J C Riseberg

The bromodeoxyuridine (BrdU) incorporation technique for immunocytochemical labeling of S-phase nuclei was optimized for the study of chromaffin cell proliferation. Sequential fixation in ethanol followed by paraformaldehyde, and the use of DNAse to render incorporated BrdU accessible to antibody, permitted permanent double staining for BrdU and tyrosine hydroxylase. The efficacy of the technique was demonstrated in microcultures of dissociated neonatal rat adrenal glands, in which chromaffin cells exhibited proliferative responses to nerve growth factor and fibroblast growth factor similar to those previously demonstrated by autoradiography. Growth factor responsiveness was observed in both serum-containing and serum-free medium.


Sign in / Sign up

Export Citation Format

Share Document