The Management of Pyromelic Polymelia in a Low-resource Setting: A Case Report.

Introduction: Polymelia is the presence of supernumerary limbs attached to a segment of the body. It occurs in about 6/10,000 live births with 1.1/10,000 cases involving the lower limbs. It has a heterogeneous pathogenesis including incomplete separation of monozygotic twins. Case Report: A 5-month-old baby with polymelia associated with ectopic right kidney, anorectal agenesis with a rectovaginal fistula, and right corneal opacity, delivered through cesarean section at 37 weeks, 3-day gestation to a 38-year-old mother. There were no known predisposing factors. Conclusion: In the management of polymelia, a thorough clinical and radiological assessment to identify additional anomalies is critical, and early surgical intervention is safe and optimizes survival.

Polymelia (thoracomelia), an extremely rare appearance of congenital anomalic limb in a Nepalese child and its embryological basis.

Polymelia is an extremely rare congenital anomaly where an individual is born with an abnormally developed extra or supernumerary limb which is generally shrunken and functionless. A case of thoracomelia (a type of polymelia) was observed macroscopically and confirmed radiologically in 1.5 years old boy born in Nepal with an abnormal supernumerary upper limb attached to his back in the thoracic region. The limb was successfully amputated, and the boy had a favorable outcome after surgical treatment, without any adverse effects or impairment. Understanding the embryogenesis of thoracomelia is essential for unraveling the complex mechanisms underlying this condition and potentially aiding in early diagnosis and intervention. This case report and review aims to shed light on the intricate processes governing forelimb formation and their perturbations leading to thoracomelia.

A case of polymelia associated with syndactyly in Didelphis aurita (Wied-Neuwied, 1826) / Relato de caso de polimelia associada a sindactilia em Didelphis aurita (Wied-Neuwied, 1826)

Abstract In January 2018, a young male of Didelphis aurita (Wied-Neuwied, 1826), the Brazilian common opossum, was admitted for veterinary evaluation at the Recovery Clinic of Wild Animals of Estácio de Sá University, CRAS, in Rio de Janeiro, Brazil, due to the presence of a partially duplicated right hind limb. After x-ray evaluation, polymelia associated with syndactyly was diagnosed. The images revealed the following alterations on the right limb: single femur with increased diameter, two tibiae, two fibulae and absence of hallux. The observed malformations were restricted to the hind right limb, which remains functional. This is the first report of such anomalies in an individual of Didelphis aurita.

Resumo Em janeiro de 2018, um macho jovem de Didelphis aurita (Wied-Neuwied, 1826), o gambá-de-orelha-preta, foi recebido para atendimento veterinário na Clínica de Recuperação de Animais Silvestres (CRAS) da Universidade Estácio de Sá, no Rio de Janeiro, por ser portador de membro posterior direito parcialmente duplicado. Após exame radiográfico, constatou-se a ocorrência de polimelia associada a sindactilia. As imagens radiográficas revelaram as seguintes alterações no membro posterior direito: um fêmur com diâmetro aumentado, duas tíbias, duas fíbulas e ausência de hálux. As malformações observadas restringiram-se apenas ao membro posterior direito, o qual mantém sua funcionalidade. Essas alterações nunca antes foram descritas na espécie Didelphis aurita.

Prenatal ultrasound-based diagnosis of fetal OEIS complex associated with lower limb polymelia and cardiac, hepatic dysplasia: A case report.

OEIS complex is a type of congenital malformation syndrome. Here, we report a case of fetal OEIS complex combined with lower limb polymelia, cardiac defects, and hepatic dysplasia. It was easily misdiagnosed when oligoamnios and the liver bulged. This case will provide reference information for early diagnosis of similar cases.

Polymelia in a chimeric Simmental calf: nociceptive withdrawal reflex, anaesthetic and analgesic management, anatomic and genetic analysis.

BACKGROUND: Polymelia is a congenital defect characterized by one or more supernumerary legs. The genetics and aetiology of this condition in cattle have not yet been thoroughly investigated even though several case reports do exist. The model of the nociceptive withdrawal reflex (NWR) has been characterized in several species to study spinal nociceptive processing. It is a polysynaptic spinal reflex that can be elicited by noxious electrical stimulation and recorded by electromyography. Thorough nociceptive examination and preventive analgesic management has not yet been an aspect in the perioperative management of polymelia cases. CASE PRESENTATION: A 4-month-old female Simmental calf was presented with notomelia. The animal was in good health and showed no neurologic deficiencies. Preoperatively, computed tomography was performed to gain more detailed anatomical information. To evaluate the sensitivity of the accessory limb, NWR testing was performed and revealed a connection of the afferent reflex pathway of the accessory limb to the efferent of the normal limb. The accessory limb was surgically removed under general anaesthesia. Intensive care included multimodal pain therapy adapted to the pain intensity scored during regular pain assessment. A gross anatomical dissection as well as a genetic analysis of the accessory limb were performed postoperatively. The calf was identified as a chimera. CONCLUSION: This calf was successfully relieved of its accessory limb. Chimerism has not been described in the congenital defect polymelia. As the accessory limb was pain sensitive and a common nociceptive reflex pathway was identified, thorough perioperative pain management was performed with the intention to prevent chronic neuropathic pain development.

Polymelia with unhealed navel in an Iranian indigenous young fowl.

Developing supernumerary limbs is a rare congenital condition that only a few cases have been documented. Depending on the cause and developmental conditions, they may be single, multiple or complicated, and occur as a syndrome or associated with other anomalies. Polymelia is defined as the presence of extra limb(s) which have been reported in human, mouse, chicken, calf and lamb. It seems that the precise mechanism regulating this type of congenital malformations is not yet clearly understood. While hereditary trait of some limb anomalies was proven in human and the responsible genetic impairments were found, this has not been confirmed in the other animals especially the birds. Regarding the different susceptibilities of various vertebrate species to the environmental and genetic factors in embryonic period, the probable cause of an embryonic defect in one species cannot be generalized to the all other species class. The present study reports a case of polymelia in an Iranian indigenous young fowl and discusses its possible causes.

Notomelia and related neural tube defects in a baby born in Niger: case report and literature review.

INTRODUCTION: Notomelia associated with neural tube defects are rare diseases. CASE REPORT: A baby was born in Niger with multiple congenital embryonic malformations on the posterior midline. The most rostral malformation was an accessory limb (polymelia) at the level of the lumbar vertebrae composed of two long bones, a foot and three toes. Accessory male genitalia were present at the base of this malformed accessory limb which had no apparent motor or sensory innervation. The second malformation was a sacral vestigial appendage with an adjacent dermal sinus opening onto the posterior midline and extending internally to the dura through a defect of the vertebral arches. From the published literature and this particular case, we conclude that notomelia is a rare clinical sequela of a neural tube defect (NTD) and is correctly classified as a dysraphic appendage. CONCLUSION: The recent occurrence of three similar cases in the same ethnic group from Niger, three from consanguineous parents, suggests that genetic factors are likely to contribute significantly to the genesis of this syndrome, consistent with a recent report that mutation of the bovine NHLRC2 gene resulting in a V311A substitution at a highly conserved locus in the NHLRC2 protein is, when homozygous, causally associated with several forms of polymelia including notomelia, with heteropagus conjoined twinning and with other NTD-related embryonic malformations. Detailed genome-wide studies of children with dysraphic appendages are indicated.

A case of polymelia associated with syndactyly in Didelphis aurita (Wied-Neuwied, 1826)

Abstract In January 2018, a young male of Didelphis aurita (Wied-Neuwied, 1826), the Brazilian common opossum, was admitted for veterinary evaluation at the Recovery Clinic of Wild Animals of Estácio de Sá University, CRAS, in Rio de Janeiro, Brazil, due to the presence of a partially duplicated right hind limb. After x-ray evaluation, polymelia associated with syndactyly was diagnosed. The images revealed the following alterations on the right limb: single femur with increased diameter, two tibiae, two fibulae and absence of hallux. The observed malformations were restricted to the hind right limb, which remains functional. This is the first report of such anomalies in an individual of Didelphis aurita.

Resumo Em janeiro de 2018, um macho jovem de Didelphis aurita (Wied-Neuwied, 1826), o gambá-de-orelha-preta, foi recebido para atendimento veterinário na Clínica de Recuperação de Animais Silvestres (CRAS) da Universidade Estácio de Sá, no Rio de Janeiro, por ser portador de membro posterior direito parcialmente duplicado. Após exame radiográfico, constatou-se a ocorrência de polimelia associada a sindactilia. As imagens radiográficas revelaram as seguintes alterações no membro posterior direito: um fêmur com diâmetro aumentado, duas tíbias, duas fíbulas e ausência de hálux. As malformações observadas restringiram-se apenas ao membro posterior direito, o qual mantém sua funcionalidade. Essas alterações nunca antes foram descritas na espécie Didelphis aurita.

Polymelia and Syndactyly in a Swainson's Hawk (Buteo swainsoni).

A hatch-year Swainson's Hawk (Buteo swainsoni) recovered from Modoc County, California, US, on 12 August 2012 had malformations of the rear limbs consisting of bilateral polymelia and syndactyly. We describe the malformations and evaluate potential causes. Postmortem examination revealed varus rotation of both femurs and abnormal appendages originating from the distal medial surface of the tibiotarsi with two nonfunctional digits on the right leg and one digit on the left leg. There was syndactyly between digits III and IV of both feet. Avian pox viral dermatitis was present on the skin of the ventral abdomen. A definitive cause of the skeletal malformations was not identified.

Occurrence of polymelia in a female child.

We report a rare case of polymelia in a 6-month-old female child who presented with developed lower limbs and an additional underdeveloped left lower limb.