Adrenal Fusion

Fusion of the adrenal glands is a rare congenital anomaly. The six cases described here were encountered in 3537 pediatric-perinatal autopsy cases. A fused adrenal was always associated with multiple congenital anomalies, including major central nervous system malformations in four cases, renal agenesis in three cases, anomalies of internal genitalia in three cases, and complex cardiac anomalies in two cases. The fused adrenal had either a horseshoe or butterfly shape. Neither adrenal hypoplasia nor hyperplasia appeared to be present, and the histologic appearance of the fused adrenal was normal in all cases.

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Bifid Epiglottis

Annals of Otology Rhinology & Laryngology ◽

10.1177/000348949610500211 ◽

1996 ◽

Vol 105 (2) ◽

pp. 155-157 ◽

Cited By ~ 12

Author(s):

John D. Goldenberg ◽

Fred J. Bressler ◽

Lauren D. Holinger ◽

Lewis R. Hutchinson

Keyword(s):

Congenital Anomaly ◽

Central Nervous System ◽

Nervous System ◽

Endocrine Disorders ◽

Central Nervous System Neoplasms ◽

Rare Congenital Anomaly ◽

Specific Syndrome ◽

Midline Defects ◽

Nervous System Neoplasms ◽

Congenital Hypopituitarism

The true bifid epiglottis is a rare congenital anomaly typically discovered during the evaluation of stridor in an infant or newborn. While it is not classified as a specific syndrome, there are frequent associations of other congenital anomalies with the bifid epiglottis. These include midline defects (such as microphallus, hypospadius, imperforate anus, and midline laryngeal cleft), endocrine disorders (including congenital hypopituitarism), and central nervous system neoplasms, including hypothalamic hamartoblastoma. The embryogenesis and options for surgical management of this anomaly are reviewed, and one case is presented in detail.

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A rare case of a complex of multiple congenital anomalies diagnosed using computed tomography in a male puppy

Veterinární Medicína ◽

10.17221/91/2017-vetmed ◽

2018 ◽

Vol 63 (No. 1) ◽

pp. 50-53

Author(s):

SW Bae ◽

DJ Chung ◽

WH Chung ◽

NW Park ◽

CY Lim ◽

...

Keyword(s):

Computed Tomography ◽

Congenital Anomaly ◽

Congenital Malformation ◽

Surgical Procedures ◽

Congenital Anomalies ◽

Rare Case ◽

Clinical Signs ◽

Urogenital Tract ◽

Multiple Congenital Anomalies ◽

Rare Congenital Anomaly

A 50-day-old male Maltese puppy was presented with difficulty in defaecation. Based on the clinical signs, and physical and radiographic examinations, including computed tomography, his condition was diagnosed as hypospadias, along with atresia ani and urethrorectal fistula. Hypospadias is a congenital malformation of the urethra that is relatively uncommon in dogs, while atresia ani is a rare congenital anomaly of the rectum and anus. An additional anatomic abnormality that can be associated with atresia ani is a fistula between the urogenital tract and rectum. After appropriate surgical procedures, the puppy recovered and defaecation via the anus was restored.

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The Spectrum of Congenital Central Nervous System Anomalies Among Stillborn: An Autopsy Based Study

Annals of Neurosciences ◽

10.1177/0972753121990169 ◽

2021 ◽

pp. 097275312199016

Author(s):

S. P. Vinutha ◽

D. Narayanappa ◽

G. V. Manjunath ◽

M. S. Sujatha ◽

M. C. Sapna Patel ◽

...

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Congenital Anomalies ◽

Developmental Process ◽

Holistic Approach ◽

Fetal Autopsy ◽

Exact Test ◽

Medical College ◽

Ultrasound Findings ◽

Cns Anomalies

Background: Congenital central nervous system (CNS) anomalies are the structural or functional abnormalities of the brain and spinal cord that occur during the intrauterine developmental process. Purpose: The present study aims to detect the prevalence of congenital CNS anomalies among stillborn fetuses, the association between congenital anomalies and maternal factors, and also the association between autopsy and ultrasound findings. Methods: This study was conducted on 50 stillborn fetuses, obtained from the Department of Obstetrics and Gynecology at JSS Medical College and Hospital, Mysuru. The fetuses were fixed in 10% formalin and autopsies were performed as per the standard fetal autopsy protocol. The congenital CNS anomalies were studied in detail. Results: CNS anomalies were the most common congenital anomalies observed. Out of the total 50 stillborn fetuses studied, CNS anomalies were found in 17 fetuses and their occurrence was more common among male stillborn than females. Meningomyelocele was the most common anomaly, followed by anencephaly. The other anomalies documented were meningocele, encephalocele, meningoencephalocele, agenesis of the corpus callosum, craniorachischisis, bifid cerebellum with hypoplastic vermis, holoprosencephaly, and sirenomelia. Fisher’s exact test showed a significant association between maternal hypothyroidism and congenital CNS anomalies ( P < .05). The autopsy confirmed the ultrasound findings in 40 (80%) fetuses. There were significant additional findings observed in seven (14%) fetal autopsies and ultrasound diagnosis completely changed in three (6%) cases, after the final autopsy procedure. Conclusion: The fetal autopsy is the single most directly evident investigation, which gives information that changes or significantly improves the clinical diagnosis. A multidisciplinary holistic approach toward pregnancy will help to detect any kind of abnormality in the fetus and thus to take a timely decision toward the management.

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Agenesis of Isthmus of the Thyroid Gland in a Patient with Graves-Basedow Disease and a Solitary Nodule

Case Reports in Surgery ◽

10.1155/2013/608481 ◽

2013 ◽

Vol 2013 ◽

pp. 1-2

Author(s):

Omer Faruk Ozkan ◽

Mehmet Asık ◽

Huseyin Toman ◽

Faruk Ozkul ◽

Oztekin Cıkman ◽

...

Keyword(s):

Congenital Anomaly ◽

Thyroid Gland ◽

Surgical Procedures ◽

Congenital Anomalies ◽

Clinical Symptoms ◽

Endocrine Gland ◽

Solitary Nodule ◽

Rare Congenital Anomaly ◽

Wide Range

The thyroid is a vascular endocrine gland with two lateral lobes connected by a narrow, median isthmus. Although a wide range of congenital anomalies of the thyroid gland has been reported in the literature, agenesis of the thyroid isthmus is a very rare congenital anomaly. Thyroid isthmus agenesis does not manifest clinical symptoms, and it can be confused with other thyroid pathologies. We describe a patient with no isthmus of the thyroid, associated with Graves-Basedow disease. Thyroid isthmus agenesis should be kept in mind in order for surgical procedures involving thyroid pathologies to be carried out safely.

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Major congenital anomalies at birth in Benghazi, Libyan Arab Jamahiriya, 1995

Eastern Mediterranean Health Journal ◽

10.26719/2000.6.1.65 ◽

2000 ◽

Vol 6 (1) ◽

pp. 66-75

Author(s):

R. Singh ◽

O. Al Sudani

Keyword(s):

Risk Factors ◽

Central Nervous System ◽

Congenital Anomalies ◽

Perinatal Death ◽

Multiple Congenital Anomalies ◽

Live Births ◽

Low Birth Weight Babies ◽

The Central Nervous System ◽

Associated Risk Factors ◽

Over 40 Years

A study was conducted to investigate the magnitude, nature and associated risk factors of major congenital anomalies [MCAs]at birth in Benghazi, Libyan Arab Jamahiriya in 1995. The incidence of MCAs was 7.4 per 1000 live births and 9.3 per 1000 total births. The rate was higher in summer, in babies born to mothers over 40 years and low-birth-weight babies or those born preterm or post-term. In 56.1% of cases there were multiple congenital anomalies and > two-thirds of the major congenital anomalies were chromosomal, musculoskeletal or of the central nervous system. The incidence of perinatal death in births with MCAs was 49.1% compared with 2.7% for all births

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Development of adrenomedullary function in suckling rats: Effects of high doses of thyroxine and cortisol

Acta Endocrinologica ◽

10.1530/acta.0.1230100 ◽

1990 ◽

Vol 123 (1) ◽

pp. 100-107

Author(s):

L. Goya ◽

C. Aláez ◽

A. M. Pascual-Leone

Keyword(s):

Central Nervous System ◽

Nervous System ◽

Adrenal Glands ◽

Normal Development ◽

Insulin Administration ◽

Newborn Rats ◽

Short Intervals ◽

Slowing Down ◽

The Central Nervous System ◽

High Doses

Abstract. The development of epinephrine, norephinephrine, and total catecholamine secretion in plasma and andrenal glands was studied in newborn rats at short intervals: at day 2, 4, 6, 8, 10, 12 and 23. The increase in the plasma level of epinephrine represents a maturation of the secretion of the adrenal medulla. The increase in plasma of epinephrine and norepinephrine and the content of catecholamines in the adrenal glands of both normal animals and those treated with either high doses of T4 or cortisol at birth suggest a slowing down of the normal development of epinephrine secretion. This was confirmed by inducing hypoglycemia in these three groups of animals by a 20-h fast or by insulin administration (0.1436 μmol/kg). We conclude that both high doses of T4 and cortisol administered at birth seem to retard the development of the autonomic nervous system similar to the effect on the central nervous system.

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Rare association of Klippel-Feil syndrome with situs inversus totalis and review of the genetic background

BMJ Case Reports ◽

10.1136/bcr-2021-241906 ◽

2021 ◽

Vol 14 (5) ◽

pp. e241906

Author(s):

Husain Abdulameer Abdali ◽

Joseph Rivendra Duddu ◽

Mohamed Jawad Mubarak ◽

Almughirah Salahaldin Mohamed

Keyword(s):

Congenital Anomaly ◽

Causative Agent ◽

Situs Inversus ◽

Congenital Anomalies ◽

Genetic Background ◽

Cervical Vertebrae ◽

Situs Inversus Totalis ◽

Rare Congenital Anomaly ◽

Rare Association ◽

High Possibility

Klippel-Feil syndrome (KFS) is a rare congenital anomaly in forming the cervical vertebrae resulting in the fusion of two or more of the vertebrae. KFS is associated with many congenital anomalies, some of which are common and well known. Here, we report a child with an extremely rare association of KFS with situs inversus totalis (SIT). Both KFS and SIT are genetically heterogeneous and their co-occurrence suggests a high possibility of sharing the same underlying causative agent. Here, we review the genetic background that is known for these two conditions in the literature.

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Peripheral and brain tissue catecholamine content in intact and anti-NGF-treated fetal sheep

AJP Regulatory Integrative and Comparative Physiology ◽

10.1152/ajpregu.1987.252.1.r7 ◽

1987 ◽

Vol 252 (1) ◽

pp. R7-R12 ◽

Cited By ~ 2

Author(s):

J. A. Schuijers ◽

D. W. Walker ◽

C. A. Browne ◽

G. D. Thorburn

Keyword(s):

Spinal Cord ◽

Central Nervous System ◽

Nervous System ◽

Gestational Age ◽

Adrenal Glands ◽

Matched Control ◽

Cervical Spinal Cord ◽

Fetal Sheep ◽

Catecholamine Content ◽

The Central Nervous System

Fetal lambs were treated with a single dose of anti-mouse nerve growth factor (anti-NGF) at 80 days gestational age. The catecholamine content of tissues was determined at 135 days gestational age. There was a reduction of either norepinephrine, epinephrine, or both, in the thymus, thyroid, atrium (but not ventricle), lung, liver, kidney, and jejunum when compared with age-matched control fetuses. The spleen, ileum, colon, and the adrenal glands were not affected by anti-NGF. In treated fetuses there was a reduction in catecholamine content of the thalamus, hypothalamus, hippocampus, medulla, cerebellum, and cervical spinal cord. These results show that some tissues are sensitive to, and some are refractory to, the action of anti-NGF at 80 days gestation. Also the results suggest that NGF may play a role in the development of catecholamine-containing neurons within the central nervous system.

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