Heliconia Research Articles

MEDICAL MYSTERY: ECTRODACTYLY A CASE REPORT

Address for Correspondence: Dr. Subhra Mandal, M.B.B.S., M.D. (Anatomy), Associate Professor, Dept. of Anatomy, Medical College, Kolkata, West Bengal, India. Contact No.: +919477458100 E-Mail: drsuvramandal@gmail.com Access this Article online Quick Response code Web site: *1Associate Professor, Dept. of Anatomy, Medical College, Kolkata, West Bengal, India. 2Post Graduate Resident in DNB ( PMR ), S.N.P. Hospital , Kolkata , West Bengal, India. 3Professor & MSVP, Bankura Sammillani Medical College, West Bengal, India. Congenital absence of all or part of one or more fingers or toes is defined as Ectrodactyly. It is the word derived from Greek ektroma meaning “abortion” and dactyly meaning digits. Also called split hand or foot malformation (SHFM), Ectrodactyly is a rare limb malformation with median cleft of hand and foot and aplasia or hypoplasia of the phalanges, metacarpals and metatarsals. Irregular pattern of inheritance exists for ectrodactyly; may be autosomal dominant, autosomal recessive or X linked. In this condition, the median cleft of the hand gives the hand, the appearance of lobster clawsso also called ‘Lobster Claw Hand”. Ectrodactyly may be very commonly associated with other anomalies of face, eyes and skeletal system. We report a rare case of unilateral ectrodactyly of right hand .The present case is not associated with any other anomaly as described in various textbooks and reference articles. The details of etiology, diagnosis, genetic causes, association with other anomalies and treatment will be discussed.

Focal dermal hypoplasia: a rare case report.

Focal dermal hypoplasia (Goltz syndrome) is a rare genetic multisystem disorder primarily involving the skin, skeletal system, eyes, and face. We report the case of an eight-month-old female child who presented with multiple hypopigmented atrophic macules along the lines of blaschko, skeletal anomalies, umbilical hernia, developmental delay, hypoplastic nails, syndactyly, and lobster claw deformity characteristic of Goltz syndrome.

Split-hand/split-foot malformation (SHFM)

Split-hand/split-foot malformation (SHFM), also known as ectrodactyly or lobster claw hand is a congenital limb malformation, characterized by a deep median cleft of the hand and/or foot due to the absence of the central rays of the autopod. It may occur singly or in association with syndromes, former being mostly autosomal dominant but autosomal recessive variety is rare. We are reporting a case of ectrodactyly with autosomal recessive mode of inheritance DOI: http://dx.doi.org/10.3126/ajms.v6i1.10018 Asian Journal of Medical Sciences Vol.6(1) 2015 122-123

Magnetic resonance urography of renal papillary necrosis

Sir, Renal papillary necrosis is a well-described entity on intravenous pyelogram (IVP) and CT urogram in both the adult and pediatric populations [1–3]. However pediatric renal papillary necrosis is less frequently encountered, particularly on magnetic resonance urogram [4–6]. Indeed, to the best of our knowledge renal papillary necrosis has yet to be described on MR urogram. We present a case of renal papillary necrosis demonstrated by MR urogram in a child with previously undiagnosed sickle cell trait. A 17-year-old previously healthy African-American girl presented to our institution with a 3-day history of gross hematuria. Her medical history was unremarkable except for recent streptococcal pharyngitis. After 2 months of extensive and inconclusive evaluation, including laboratory analysis, renal US, and a renal biopsy, the hematuria persisted, with hemoglobin decreasing to 4 g/deciliter. The girl was subsequently evaluated by cystoscopy, which demonstrated hemorrhage from the left ureteral orifice. Retrograde pyelogram and cystoureteroscope showed irregularity of the left upper pole papillary urothelium (Fig. 1). At this point, the pediatric urologist was suspicious but not confident of the diagnosis of renal papillary necrosis. Symptoms persisted, so MR urography was performed. MR urogram demonstrated classic changes of bilateral papillary necrosis (Fig. 2). Given these findings, a hemoglobin electrophoresis led to the diagnosis of sickle cell trait. The girl was placed on aminocaproic acid with cessation of hematuria. Imaging of renal papillary necrosis, which is most commonly caused by sickle cell disease and nonsteroidal anti-inflammatory medications in the pediatric population, is well documented within the radiology literature, with Praetorius first describing its radiologic appearance in 1937 [2]. Classically, intravenous pyelogram has been the examination of choice. From its appearance on intravenous pyelogram, multiple classic radiographic signs have been elucidated, e.g., ballon-tee, lobster claw, and signet ring. Recently several authors have demonstrated the imaging appearance and diagnosis of renal papillary necrosis in adults using CT urography [3]. However, given the use of iodinated contrast agent and the radiation dose of both CT and intravenous pyelogram, alternative imaging modalities are helpful for pediatric imaging in suspected renal papillary necrosis. In 1995 Rothpearl et al. [6] reported the techniques and application of MR urography in the depiction of the kidney and bladder pathology. One patient included in this study population had pathologically proven renal papillary necrosis. However, the authors did not elucidate whether the changes of renal papillary necrosis were demonstrable on MR urogram [6]. In a subsequent invited commentary, Hattery and King [7] called into question the utility of MR urogram in the detection of papillary necrosis given the resolution requirements for depicting the subtle changes of renal papillary necrosis at the caliceal and forniceal levels [7]. The authors did J. Schroeder Medical University of South Carolina, College of Medicine, Charleston, SC, USA

Krukenberg operation: The "lobster claw" for traumatic amputation of the left hand

We report a case of 28-year-old male patient who was run over by train, which resulted in above-elbow amputation on the right side and hand amputation on the left side. His financial status precluded the use of upper limb prosthesis. In this report, we evaluated the various aspects of the Krukenberg operation. The parameters were functionality, stereognosis, cosmesis, dominance, patient and societal acceptance. In our country interest in Krukenburg operation should be rekindled. Most of the surgeons neglected it favor of expensive prosthesis on grounds of unfamiliarity with the surgical procedure.

A Rare and Severe Case of Split-Hand/Foot Malformation in a Child in India

Ectrodactyly, termed Split-Hand/Split-Foot Malformation (SHFM) is a rare genetic condition characterized by defects of the central elements of the autopod (hand/foot). Clinical presentation is with the absence of one or more median rays or digits creating cone shaped clefts of the hands and/or feet. The present case of severe bilateral split-hand and foot malformations was presented in an 8-year-old girl in India. This case of SHFM involves the complete absence of the central rays of the autopod in which each of the hands and each foot is divided into two parts by a coned shape cleft tapering proximally, resembling a “lobster claw”. SHFM is often associated with other limb anomalies including monodactyly, syndactyly and aplasia and/or hypoplasia of the phalanges, metacarpals, and metatarsals. Most cases are sporadic; however familial forms do exist with pre-dominantly autosomal dominant inheritance. This case is an example of the non-syndromic form of SHFM expressed with isolated involvement of the limbs, while the syndromic form is associated with anomalies as intellectual disability, ectodermal and craniofacial findings and hearing loss. Non-syndromic isolated ectrodactyly does not usually require surgical intervention. We recommended against surgical reconstruction, due to lack of evidence of functional disability.

Split-hand/Split-foot malformation

Split-hand/split-foot malformation (SHFM), also known as ectrodactyly or lobster claw hand deformity, is a rare congenital limb malformation characterized by a median cleft of hands and/or feet due to the absence of the central rays. SHFM has a reported incidence of 1 in 90,000 live births. It may occur either as an isolated anomaly or as part of a syndrome. We present a rare case of a 9-year-old boy with SHFM who underwent surgical correction of the cleft hand, with improved cosmetic and functional outcome.

A case of ectrodactyly in a neonate

Ectrodactyly also known as Split hand/foot malformation is a rare limb malformation with autosomal dominant in heritance with variable penetrance, commonly known as "lobster claw hand". Usually it involves midline clefts of the hands and feet with syndactyly. We report a neonate with ectrodactyly and brief review of literature of condition.

Lobster claw syndrome: A rare entity

Lobster claw syndrome: A rare entity

A Geographic Mosaic of Plant–Pollinator Interactions in the Eastern Caribbean Islands

AbstractThe interplay between generalized and specialized plant–animal interactions is a core concept in understanding the evolution of mutualisms. Within the Eastern Caribbean, Heliconia bihai is a dominant forest species in the southern island of St. Vincent where H. caribaea is virtually absent. Heliconia caribaea is most common on the northern island of St. Kitts where H. bihai is restricted to the tops of the highest peaks. Both species are abundant on the central island of Dominica. We compared flowering patterns, nectar characteristics, and visitation frequency of hummingbirds in the two heliconias on the three islands to determine the extent of geographic variations in this plant–pollinator mutualism. The peak flowering season of the two heliconias was observed to be in April–May on all three islands with little within‐ and between‐island variations. Nectar production significantly varied between species and between islands. Visitation patterns by the principal hummingbird pollinators also varied between the islands: (1) on Dominica, only females of a single species of hummingbird pollinated the flowers of H. bihai (sexual specialization), whereas both sexes of the same hummingbird pollinated the flowers of H. caribaea (species specialization); (2) on St. Vincent, both sexes of the same hummingbird pollinated the flowers of H. bihai (species specialization); and (3) on St. Kitts, only females pollinated the flowers of H. bihai (sexual specialization), whereas several species of hummingbird visited the flowers of H. caribaea (species generalization). We propose that the Heliconia–hummingbird interactions in the Eastern Caribbean represent a geographically variable coevolutionary mosaic of plant–pollinator interactions.

Prenatal Diagnosis of EEC Syndrome with “Lobster Claw” Anomaly by 3D Ultrasound

The EEC syndrome is a genetic anomaly characterized by the triad: ectodermal dysplasia (development of anomalies of the structures derived from the embryonic ectodermal layer), ectrodactyly (extremities, hands and feet malformations) and cleft lip and/or palate; these malformations can be seen together or in isolation. The prenatal diagnosis can be made by two-dimensional ultrasonography (2DUS) that identifies the facial and/or limb anomalies, most characteristic being the “lobster-claw” hands. The three-dimensional ultrasonography (3DUS) provides a better analysis of the malformations than the 2DUS. A 25-year-old primigravida, had her first transvaginal ultrasonography that showed an unique fetus with crow-rump length of 47 mm with poorly defined hands and feet,. She was suspected of having sporadic form of EEC syndrome. The 2DUS performed at 19 weeks confirmed the EEC syndrome, showing a fetus with lobster-claw hands (absence of the 2nd and 3rd fingers), left foot with the absence of the 3rd toe and the right foot with syndactyly, and presence of cleft lip/palate. The 3DUS defined the anomalies much better than 2DUS including the lobster-claw hands.

Territorial Fidelity to Nectar Sources by Purple-throated Caribs, Eulampis Jugularis

We present the first record of territorial site-fidelity across multiple years by Purple-throated Caribs (Eulampis jugularis) on three different islands in the eastern Caribbean: St. Kitts, Dominica, and St. Vincent. Marked male Purple-throated Caribs were monitored throughout the flowering season of their main nectar resources, Heliconia caribaea (St. Kitts and Dominica) and H. bihai (St. Vincent), both native perennial herbs. Individual males were observed defending the same Heliconia patches for 3 years (St. Vincent), 4 years (St. Kitts), and 5 years (Dominica), and remained near these patches even when they were not in flower. The territorial behavior and resource dependence of Purple-throated Caribs on native heliconias likely have a key role in the coevolution of this specialized plant-pollinator interaction.

A Newborn Who Has Hand and Foot Deformities

A newborn presents with bilateral hand and foot deformities (Figures 1 and 2). Figure 1. Image of bilateral feet. Figure 2. Image of bilateral hands. ### Prenatal and Birth Histories ### Presentation At birth, the infant was noted to have deformities resembling a “lobster claw” to both her feet, and each hand had only a single digit. ### Vital Signs ### Physical Examination

Genetic diversity and molecular characterization of several Heliconia species in Colombia

Researchers have classified the Heliconia genus as a group of highly variable and diverse plants. Species and cultivars are visually differentiated primarily on the basis of the color and size of inflorescence bracts. At taxonomic level, flower type (parabolic, sigmoid, or erect) and size are taken into account. The vast morphological diversity of heliconias at intra-specific, intra-population, and varietal levels in central-west Colombia prompted the present study. We characterized the genetic variability of 67 genotypes of cultivated heliconias belonging to Heliconia caribaea Lamarck, H. bihai (L.) L., H. orthotricha L. Andersson, H. stricta Huber, H. wagneriana Petersen, and H. psittacorum L. f., as well as that of several interspecific hybrids such as H. psittacorum L. f. x H. spathocircinata Aristeguieta and H. caribaea Lamarck x H. bihai (L.) L. We also created an approximation to their phylogenetic analysis. Molecular analysis using amplified fragment length polymorphism (AFLP) markers revealed a total of 170 bands. Two large, well-defined groups resulted: the first grouped cultivars of the very closely related H. caribaea and H. bihai species with those of H. orthotricha and H. psittacorum, and the second grouped H. stricta and H. wagneriana cultivars. The lowest percentage of polymorphism was found in H. psittacorum (17.65%) and the highest was in H. stricta (55.88%). Using AFLP, phylogenetic analysis of the species studied revealed the monophyletic origin of the Heliconiaceae family, and identified the Heliconia subgenus as monophyletic while providing evidence of the polyphyletic origin of several representatives of the Stenochlamys subgenus.

Syndrome de Goltz chez un nouveau-né avec fente labiopalatine

Focal dermal hypoplasia (Goltz syndrome) is a rare congenital dysplasia of the mesoectodermal derived tissues. It is an X-linked inheritance syndrome caused by mutations in the PORCNgene mapped on Xp11.23. The condition is characterized by cutaneous lesions distributed in linear areas associated with diverse congenital deformities. Given the rarely described neonatal features, we report a case of Goltz syndrome in a black female newborn. This is the first case known in Burkina Faso. The cutaneous, hair, and nail lesions usually observed were present, characterized by their preponderance on the left side of the body with exclusive ipsilateral skeletal abnormalities (cleft lip and palate, agenesis of the metatarsals and toes of the foot, syndactyly, lobster claw, and absence of a rib). The limits in the management and the negative social and cultural perception of the deformities in the context of a developing country did not favor the child's survival.

Ectrodactyly and Prenatal Diagnosis

Ectrodactyly is an autosomal dominant ectodermal dysplasia presenting as bilateral congenital malformed hands and feet [1]. It affects about 1 in 90,000 births with males and females equally as likely to be affected. It is characterized by transverse terminal aphalangia or partial to total absence of the distal segments of fingers. It may involve one or more digits or the full hand and even part of the upper arm. More severe manifestations are hemimelia or amelia. All these abnormalities are considered to represent various degrees of severity of the same anomaly and may be due to an intrauterine vascular occlusion or insufficiency [2]. These different forms are connected with a different genetic mutation. Type I, the most frequent form has been found to be a mutation on chromosome 7 in a region that contains two homeobox genes, DLX5 and DLX6. Usually this is characterized as the split hand/foot deformity due to the absence of the third digit, with clefting into the proximal portion of the hand or foot and syndactyly of remaining digits on each side of the cleft. The hand resembles a lobster claw [3]. The association of ectrodactyly with cleft lip and palate was originally described by Cockayne [4]. It was known as Ectrodactyly-Ectodermal Dysplasia-Cleft lip/palate syndrome (EEC syndrome) [5].

Indução de embriões somáticos a partir de embriões zigóticos de Heliconia bihai (L.) L. cv. Lobster Claw Two

As flores tropicais têm contribuído para o aumento do mercado florístico no Brasil; dentre elas destacam-se as do gênero Heliconia. A propagação de helicônias que atinge maior número de mudas ocorre por via vegetativa, por meio da divisão de rizomas, aumentando a probabilidade de disseminação de doenças, podendo, num futuro próximo, comprometer a produção de flores. O cultivo in vitro surge como importante alternativa para proporcionar a produção de mudas de helicônia com elevado padrão de qualidade. Entretanto, a micropropagação de helicônias via ápices caulinares depara-se com problemas de contaminação endofítica, dificultando a multiplicação do explante. Diante disso, fez-se necessário avaliar alternativas que promovam a produção de mudas in vitro, por meio da utilização de outros explantes. Para tanto, realizou-se um experimento, utilizando embriões zigóticos, provenientes de frutos imaturos e maduros de H. bihai (L.) L. cv. Lobster Claw Two, em combinações de AIA (0; 5,70 e 11,41 µm L-1) e 2,4-D (0; 22,62; 45,24; 67,86 e 90,48 µm L-1), para induzir a formação de embriões somáticos durante 90 dias. Por meio de análise histológica, observou-se a formação de embriões somáticos apenas nos embriões zigóticos, provenientes de frutos maduros, cultivados na ausência de reguladores de crescimento.

Lobster claw syndrome

An 84-year-old woman with a history of cryoglobulinemic vasculitis due to multiple myeloma developed digital gangrene on her right second finger and left big toe (arrows). Her medical history was notable for mental retardation, hearing loss, facial dysmorphism and malformations of …

Storage of cut Heliconia bihai (L.) cv. Lobster Claw flowers at low temperatures

The postharvest conservation of cut Heliconia flowers is an important factor to the success of commercialization, especially with regard to exportation. In the present study, the maximal storage time of cut inflorescences of Heliconia bihai cv. Lobster Claw at two different temperatures (12 and 19 °C) was evaluated and compared to laboratory conditions (25 °C, control treatment). Changes in visual quality, fresh weight and bract color (L*, a* and b*) were determined. The visual quality of the inflorescences and fresh weight decreased with time in all treatments. Symptoms of chilling injury were observed on the inflorescences stored at 12 °C for six and eight days. Bract color was not affected by temperature, storage time or the senescence process. The results indicate that a temperature of 12 °C is not recommended for a storage time longer than four days, whereas 19 °C can be used for a storage time of up to eight days for cut inflorescences of H. bihai cv. Lobster Claw.

Recurrent flank pain from 'lobster claw'.

A 56-year-old woman with sickle-cell trait (hemoglobin electrophoresis with 53.5% hemoglobin A, 3.7% hemoglobin A2, 41.7% hemoglobin S and 1.1% hemoglobin F) was seen for recurrent episodes of ‘bilateral’ flank pain associated with gross hematuria and passage of cellular debris. These episodes had occurred on an infrequent basis for many years and seemed to be triggered by fasting. Her past medical history was pertinent for osteoarthritis and nephrolithiasis. She was on no medications and did not use any non-steroidal anti-inflammatory drugs or other over-the-counter medications. Physical examination was unremarkable. Renal function was normal (serum creatinine of 0.7 mg/dL). Hematocrit was 34.8. There was no proteinuria. A computed tomographic urogram (CTU) of abdomen demonstrated bilateral papillary necrosis. Papillary necrosis reflects ischemic damage to renal medulla. Sickling facilitated by hyperosmotic, hypoxic and acidotic environment of the medulla leads to infarct. Diabetes mellitus, analgesic abuse, pyelonephritis, renal vein thrombosis, tuberculosis and obstructive uropathy are other major causes of papillary necrosis. Multidetector-row CTU helps in the detailed assessment of the renal parenchyma and collecting system and facilitates identification of papillary necrosis at a very early stage [1]. Necrosis of the papilla causes swelling followed by shrinkage of the papilla with widening of the fornices. The necrotic papilla may either remain in situ or detach and form a cavity. Necrosis may be limited to the central tip of the papilla (medullary form) or involve the entire papilla (total papillary necrosis) [2]. Radiographically, tracks of contrast arising from the fornices of blunted calyces are seen. Sloughed papillae are seen as central filling defect with a surrounding ring of contrast. Figures 1 and ​and22 show the typical ‘egg in a cup’ appearance with central pooling of contrast and ‘lobster claw’ pattern of contrast extravasation seen in papillary necrosis [3]. Fig. 1. (A) CTU with intravenous contrast demonstrating bilateral papillary necrosis (3D reconstruction) shows the ‘egg in a cup’ appearance with central contrast pooling (long arrow, left), ring shadow of sloughed papilla with central lucency ... Fig. 2. CTU with intravenous contrast demonstrating bilateral papillary necrosis (coronal section) shows the ‘egg in a cup’ appearance with central contrast pooling (long arrow, left), ring shadow of sloughed papilla with central lucency (arrow ...