Predictors of complication after adrenalectomy

ABSTRACT Purpose: To investigate risk factors for complications in patients undergoing adrenalectomy. Materials and Methods: A retrospective search of our institutional database was performed of patients who underwent adrenalectomy, between 2014 and 2018. Clinical parameters and adrenal disorder characteristics were assessed and correlated to intra and post-operative course. Complications were analyzed within 30-days after surgery. A logistic regression was performed in order to identify independent predictors of morbidity in patients after adrenalectomy. Results: The files of 154 patients were reviewed. Median age and Body Mass Index (BMI) were 52-years and 27.8kg/m2, respectively. Mean tumor size was 4.9±4cm. Median surgery duration and estimated blood loss were 140min and 50mL, respectively. There were six conversions to open surgery. Minor and major post-operative complications occurred in 17.5% and 8.4% of the patients. Intra-operative complications occurred in 26.6% of the patients. Four patients died. Mean hospitalization duration was 4-days (Interquartile Range: 3-8). Patients age (p=0.004), comorbidities (p=0.003) and pathological diagnosis (p=0.003) were independent predictors of post-operative complications. Tumor size (p<0.001) and BMI (p=0.009) were independent predictors of intra-operative complications. Pathological diagnosis (p<0.001) and Charlson score (p=0.013) were independent predictors of death. Conclusion: Diligent care is needed with older patients, with multiple comorbidities and harboring unfavorable adrenal disorders (adrenocortical carcinoma and pheocromocytoma), who have greater risk of post-operative complications. Patients with elevated BMI and larger tumors have higher risk of intra, but not of post-operative complications.

Hemorragia suprarrenal que se manifiesta como hematoma escrotal en el recién nacido: A propósito de un caso / Adrenal hemorrhage presenting as a scrotal hematoma in the newborn: A case report

La hemorragia suprarrenal neonatal es poco frecuente. Se presenta en el 0,2% de los recién nacidos. El 10% de los casos son bilaterales. Puede estar asociada a un traumatismo al nacimiento, peso alto al nacer o un curso neonatal complicado por hipoxia, asfixia, hipotensión o coagulopatía. El hematoma escrotal es una manifestación extremadamente rara de la hemorragia suprarrenal neonatal. La mayoría de los pacientes presentan tumefacción escrotal con coloración azulada. En los recién nacidos, la tumefacción escrotal, con o sin coloración azulada, puede deberse a diferentes causas. Comunicamos un caso inusual de hemorragia suprarrenal neonatal secundaria a asfixia perinatal, que se manifestó con un hematoma escrotal. El diagnóstico de hemorragia suprarrenal neonatal y hematoma escrotal fue ecográfico; el tratamiento conservador evitó la exploración quirúrgica innecesaria.

Neonatal adrenal hemorrhage is uncommon. It is present in 0,2% of newborns. Ten percent of the cases occur bilaterally. It can be associated with birth trauma, large birth weight, or neonatal course complicated by hypoxia and asphyxia, hypotension, or coagulopathy. Scrotal hematoma is an extremely rare manifestation of NAH. Most patients present scrotal swelling with bluish discolouration. Scrotal swelling with/without bluish discoloration in newborns may result from different causes. We report an unusual case of neonatal adrenal hemorrage secondary to perinatal asphyxia, associated with SH. Neonatal adrenal hemorrhage and scrotal hematoma were diagnosedby ultrasonography and treated by conservative treatment, avoiding unnecessary surgical exploration.

Quiste suprarrenal primario / Primary adrenal cyst

Adrenal cyst is the commonest type of benign lesions of adrenal gland, althought is a very rare entity. Tipically, they are presented with abdominal pain or palpable mass, but nowdays they are detected most frecuently an imaging studies like incidentaloma. Adrenal Cyst have a broad differential diagnoses, rendering definitive diagnosis and subsequent management difficult. Are categorized into four subtypes: endothelial, pseudocyst, epithelial, and parasitic. This management paradigm may be summarized as: ruling out functional status of the cyst, evaluating chances of incidental malignancy by imaging, avoiding potential complications of surveillance (hemorrhage, infection), particularly in large cysts. A case of a patient with a left non-functioning adrenal cyst is reported, with abdominal pain. The diagnostic and therapeutic options are discussed and the literature is reviewed.

Los quistes suprarrenales son las lesiones quísticas más frecuentes de la glándula suprarrenal, a pesar de que constituyen una rara entidad. Típicamente se presentan por un cuadro de dolor abdominal o masa palpable, pero en la actualidad se detectan cada vez con más frecuencia en estudios radiológicos como incidentalomas. Los quistes suprarrenales cuentan con amplios diagnósticos diferenciales, lo que hace un difícil diagnóstico definitivo y una dificultad en el manejo posterior. Estos quistes se clasifican en cuatro subtipos: endoteliales, pseudoquiste, epiteliales y parasitarios. El manejo de un quiste suprarrenal se puede resumir en tres pilares fundamentales: descartar el estado funcional del quiste, evaluación de eventual malignidad por imágenes, y evitar las posibles complicaciones (hemorragia, infección), sobre todo en los quistes de gran tamaño. Se presenta el caso de una paciente con quiste suprarrenal izquierdo no funcionante asociado a dolor abdominal. Se realiza revisión de la literatura y se plantean los distintos diagnósticos y alternativas terapéuticas.

Scrotal hematoma as a sign of adrenal hemorrhage in newborns / Hematoma escrotal como um sinal de hemorragia da adrenal no recém-nascido

CONTEXT: Bluish discoloration and swelling of the scrotum in newborns can arise from a number of diseases, including torsion of the testes, orchitis, scrotal or testicular edema, hydrocele, inguinal hernia, meconium peritonitis, hematocele, testicular tumor and traumatic hematoma. Forty-two cases of scrotal abnormalities as signs of neonatal adrenal hemorrhage were found in the literature. CASE REPORT: We present a case of scrotal hematoma due to adrenal hemorrhage in a newborn. Conservative treatment with clinical follow-up was adopted, with complete resolution within 10 days. The possible differential diagnoses are reviewed and discussed.

CONTEXTO: Edema e coloração azulada do escroto do recém-nascido podem sugerir uma série de doenças, incluindo torção dos testículos, orquite, edema escrotal e testicular, hidrocele, hérnia inguinal, peritonite meconial, hematocele, tumor testicular e hematoma traumático. Quarenta e dois casos de alterações escrotais como sinal de hemorragia da glândula adrenal foram encontrados na literatura. RELATO DE CASO: Apresentamos um caso de hematoma escrotal devido a hemorragia da glândula adrenal em um recém-nascido. O tratamento adotado foi conservador, com acompanhamento clínico, com resolução completa em 10 dias. Os possíveis diagnósticos diferenciais são revisados e discutidos.

Hiperplasia suprarrenal macronodular bilateral independiente de ACTH: caso clínico / ACTH independent bilateral macronodular adrenal hyperplasia: report of one case

ACTH Independent cushing syndrome, involving both adrenal glands, represents a minority of endogenous hyperadrenocorticisms and is caused by infrequent diseases. ACTH independent macronodular adrenal hyperplasia (AIMAH) is one of them and is caused by aberrant cortical adrenal receptors expression that are able to stimulate cortisol secretion. We report a 62 years old male, presenting with hypertension, weight gain, urinary lithiasis and osteopenia. The diagnosis of Cushing's syndrome was made with a morning cortisol level after 1 mg nocturnal dexamethasone of 8.2 ug/dL. Urinary free 24 hour cortisol levels were normal, circadian rhythm of cortisol was maintained and ACTH was <5 pg/mL. Abdominal magnetic resonance imaging showed bilateral nodular adrenal enlargement. The functional study, looking for abnormal receptors, showed a potent cortisol secretory response after stimulation with vasopressin.

Cellular and molecular abnormalities of a macronodular adrenal hyperplasia causing beta-blocker-sensitive Cushing's syndrome

Cushing's syndrome due to ACTH-independent macronodular adrenal hyperplasia (AIMAH) can be associated with abnormal responses of aberrantly expressed adrenocortical receptors. This study aimed to characterize in vitro the pathophysiology of hypercortisolism in a b-blocker-sensitive Cushing's syndrome due to AIMAH. Cortisol secretion profile under aberrant receptors stimulation revealed hyperresponsiveness to salbutamol (beta2-adrenoceptor agonist), cisapride (5-HT4 receptor agonist), and vasopressin in AIMAH cultured cells, but not in normal adrenocortical cells. By RT-PCR, AIMAH tissues revealed beta2-adrenoceptor overexpression rather than ectopical expression. MC2R expression was similar in both AIMAH and normal adrenocortical tissues. Curiously, cortisol levels of AIMAH cells under basal condition were 15-fold higher than those of control cells and were not responsive to ACTH. Analysis of culture medium from AIMAH cells could detect the presence of ACTH, which was immunohistochemically confirmed. Finally, the present study of AIMAH cells has identified: a) cortisol hyperresponsiveness to catecholamines, 5-HT4 and vasopressin in vitro, in agreement with clinical screening tests; b) abnormal expression of beta2-adrenoceptors in some areas of the hyperplastic adrenal tissue; c) autocrine loop of ACTH production. Altogether, the demonstration of aberrant responses to hormonal receptors and autocrine hormone production in the same tissue supports the assumption of multiple molecular alterations in adrenal macronodular hyperplasia.

A síndrome de Cushing secundária à hiperplasia adrenal macronodular independente de ACTH (AIMAH) pode estar associada com respostas anômalas a estímulos sobre receptores hormonais expressos de maneira aberrante no córtex adrenal. O objetivo deste trabalho foi caracterizar a fisiopatologia do hipercortisolismo in vitro na síndrome de Cushing responsiva a beta-bloqueadores decorrente de AIMAH. Em cultura de células, a secreção de cortisol apresentou resposta aumentada ao salbutamol (agonista beta2-adrenérgico), à cisaprida (agonista de receptor 5-HT4) e à vasopressina, na AIMAH mas não no córtex adrenal normal. O estudo de receptores aberrantes por RT-PCR demonstrou que o gene do receptor beta2-adrenérgico estava superexpresso (e não expresso ectopicamente) nos fragmentos da AIMAH quando comparado ao tecido normal. A expressão de MC2R foi semelhante em ambos. Curiosamente, o nível basal de secreção de cortisol pelas células da AIMAH foi 15 vezes superior às células normais, não havendo resposta das células AIMAH ao estímulo com ACTH. A análise do meio de cultura das células AIMAH revelou a presença de ACTH, que foi confirmada por estudo imuno-histoquímico. Em suma, este estudo demonstrou: a) aumento dos níveis de cortisol in vitro em resposta a catecolaminas, 5-HT4 e vasopressina, correspondendo aos resultados dos testes clínicos para pesquisa de receptores aberrantes; b) expressão anormal de receptores beta2-adrenérgicos em algumas áreas de hiperplasia; c) produção autócrina de ACTH. Estes resultados envolvendo ativação de receptores aberrantes e estímulo hormonal autócrino no mesmo tecido favorecem a hipótese da existência de alterações moleculares múltiplas na hiperplasia adrenal macronodular.

Disseminated herpes simplex infection with cystic fibrosis: a case report.

A 6 months old female infant presented with history of fever, cough and severe respiratory distress. There was past history of recurrent attacks of pneumonia. She succumbed to the illness after a hospital stay of 7 days. Postmortem revealed morphological evidence of cystic fibrosis along with herpes simplex infection of liver and adrenals. The co-existence of disseminated herpes simplex infection and cystic fibrosis is very rare.

Congenital Nephrotic Syndrome with adrenal calcification and cardiac malformation.

Congenital Nephrotic Syndrome (CNS) with adrenal calcification and CNS with congenital heart disease (CHD) have rarely been reported. However, CNS with both these rare associations has never been previously reported. Here we report a case of CNS with both rare associations, perhaps the first report from India to the best of our knowledge.

Complex glycerol kinase deficiency: an X-linked disorder associated with adrenal hypoplasia congenita.

Complex glycerol kinase deficiency (GKD) results from the contiguous deletion on Xp21 of all or part of the gene for glycerol kinase together with that for adrenal hypoplasia congenita (AHC) and /or Duchenne muscular dystrophy (DMD). The authors present the case of a newborn whose initial issues were refractory hypoglycaemia along with hyponatremia and hyperkalemia. He also had low serum cortisol levels and raised urinary excretion of glycerol and required steroid supplementation. His creatinine phosphokinase (CPK) levels were normal. Molecular studies revealed a contiguous Xp21 deletion. Therapy in such cases must be prompt and includes correction of hypoglycaemia and dyselectrolytemia, a low fat diet and steroid replacement.

Pseudoquiste suprarrenal hemorrágico: Descripción de un caso y revision de la literatura / Hemorrhagic Adrenal Pseudocyst: Case Report and a Review of the Literature

Los quistes de suprarrenal son raros y aún más los pseudoquistes hemorrágicos, los que generalmente son entidades no sospechadas y que pueden presentarse con datos sugestivos de abdomen agudo. Algunos de estos pueden llegar a desarrollar ruptura con la consiguiente hemorragia retroperitoneal e incluso la muerte. Presentamos el caso de una paciente con un pseudoquiste suprarrenal hemorrágico, con un cuadro clínico sugestivo de apenaicitis.

Adrenal cysts are rare entities, and hemorrhagic pseudocysts are even less frequent. Generally, they are unsuspected during first patient evaluation and can suggest acute abdomen. We present the case of a hemorrhagic pseudocyst with sudden on set of abdominal pain and features compatible with acute appendicitis.

Traumatic rupture of adrenal pseudocyst leading to massive hemorrhage in retroperitoneum

We present the case of a patient who had a large pseudocyst in the right adrenal gland, which was ruptured following blunt abdominal trauma, leading to a voluminous hemorrhage in retroperitoneum. A 29-year old female patient was admitted in the emergency room following a fall from stairs with trauma in right flank. She underwent a computerized tomography that evidenced a large retroperitoneal collection, with no apparent renal damage. She was submitted to surgery, where a large ruptured cyst was observed, originating from the upper portion of the right adrenal gland. Cystic diseases of adrenal gland are rare. Highly voluminous cysts can be damaged in cases of blunt trauma to the lumbar region leading to large hematomas in retroperitoneum.

Hemophagocytic Syndrome Associated with Bilateral Adrenal Gland Tuberculosis

We report a case of a patient who presented with hemophagocytic syndrome (HPS) and adrenal crisis associated with bilateral adrenal gland tuberculosis, and resulted in a poor outcome. A 50-year-old man was transferred to our hospital from a local clinic due to fever, weight loss, and bilateral adrenal masses. Laboratory findings showed leukopenia, mild anemia, and elevated lactate dehydrogenase. Computed tomography (CT) of the abdomen revealed bilateral adrenal masses and hepatosplenomegaly. CT-guided adrenal gland biopsy showed numerous epithelioid cells and infiltration with caseous necrosis consistent with tuberculosis. Bone marrow aspiration and biopsy showed significant hemophagocytosis without evidence of malignancy, hence HPS associated with bilateral adrenal tuberculosis was diagnosed. During anti-tuberculosis treatment the patient showed recurrent hypoglycemia and hypotension. Rapid ACTH stimulation test revealed adrenal insufficiency, and we added corticosteroid treatment. But pancytopenia, especially thrombocytopenia, persisted and repeated bone marrow aspiration showed continued hemophagocytosis. On treatment day 41 multiple organ failure occurred in the patient during anti-tuberculous treatment and steroid replacement.

Adrenal cyst: a case report

A case of benign endothelial adrenal cyst in an asymptomatic 33-year-old woman is presented along with MRI photographs

Hipoadrenalismo en el síndrome antifosfolípidos/cofactores / Hypoadrenalism and Antiphospholipids/Cofactors

El síndrome antifosfolipidos/cofactores (SACF) se define como la presencia de anticuerpos antifosfolípidos (anticardiolipinas y/o anticoagulante lúpico), trombocitopenia, y asociación con manifestaciones tromboembólicas en territorio arterial y/o venoso. La hemorragia adrenal es una complicación poco común pero con riesgo potencial para la vida. Durante los últimos años ha ido en aumento el reporte de la asociación de hipoadrenalismo y enfermedad de Addison en presencia de anticuerpos antifosfolípidos. Su difícil diagnóstico está probablemente relacionado a las manifestaciones clínicas inespecíficas de la insuficiencia suprarrenal dentro de una enfermedad principal concurrente. Se realizó una revisión de las características más importantes tanto clínicas como de laboratorio, así como las bases patogénicas que incluyen la confluencia del binomio SAFC-hemorragia adrenal. Reconocer que un paciente está en riesgo de hemorragia suprarrenal podría incrementar la posibilidad de un diagnóstico temprano, tratamiento y supervivencia de estos desórdenes

The incidentally discovered asymptomatic adrenal tuberculous mass mimicking malignancy.

Tuberculous adrenal mass without any tuberculous changes in other sites is a rare entity. In this study we describe such a case. A male patient of 47 years with 6 months history of anorexia and weakness was presented. Ultrasonography and computed tomography revealed a mass in the right adrenal gland. It was mistaken as a malignant neoplasm and the diagnosis was confirmed by surgical exploration.

Insuficiencia supra-renal na infancia associada a severas alteracoes do sistema nervoso central: relato de um caso de adrenoleucodistrofia / Supra-renal failure in infancy associated with severe alterations of central nervous system: a adrenoleukodystrophy case report

E apresentado um caso de adrenoleucodistrofia (ALD), doenca metabolica hereditaria ligada ao sexo e caracterizada por alteracoes no metabolismo de acidos graxos de cadeia muito longa (Very Long-Chain Fatty Acids - VLCFA), manifestando-se clinicamente por insuficiencia adrenal e anormalidades neurologicas progressivas. Sao discutidas a forma de apresentacao da doenca e o seu diagnostico, sendo enfatizada a importancia de ter-se presente a possibilidade de ALD em criancas portadoras de doenca de Addison, porque trata-se de molestia sujeita a intervencao terapeutica, com prevencao e ate regressao dos disturbios neurologicos