The Rare Association of Cleft Lip and/or Palate and Wilms Tumor

2019 ◽  
Vol 56 (8) ◽  
pp. 1096-1106
Author(s):  
Elizabeth Theng ◽  
Meghan Tracy ◽  
Susan Starling Hughes ◽  
Alison Kaye
Keyword(s):  
Partial Nephrectomy ◽  
Retrospective Review ◽  
Congenital Anomalies ◽  
Cleft Lip ◽  
Developmental Delays ◽  
Wilms Tumor ◽  
Rare Association ◽  
Full Remission

There is currently no recognized connection between the occurrence of cleft lip and/or palate (CL/P) and Wilms tumor (WT). A retrospective review of cleft team records (2001-2015) revealed 3 cases of children, all male, with concomitant diagnoses of CL/P and WT treated at our institution. These patients presented as infants for care of their CL/P, all with additional congenital anomalies, developmental delays, and growth delays. Between the ages of 1 and 4 years, each was diagnosed with WT, which was treated with chemotherapy and partial nephrectomy, +/− radiation, leading to full remission in all cases.

Hereditary Cleft Lip/Palate and Wilms Tumor: A Rare Association

2002 ◽  
Vol 39 (3) ◽  
pp. 376-379 ◽  
Author(s):  
Chung-Chih Yu ◽  
Fen-Hwa Wong ◽  
Lun-Jou Lo ◽  
Yu-Ray Chen
Keyword(s):  
Cleft Lip ◽  
Wilms Tumor ◽  
Rare Association ◽  
Cleft Lip Palate

Hereditary Cleft Lip/Palate and Wilms Tumor: A Rare Association

2002 ◽  
Vol 39 (3) ◽  
pp. 376-379 ◽  
Author(s):  
Chung-Chih Yu ◽  
Fen-Hwa Wong ◽  
Lun-Jou Lo ◽  
Yu-Ray Chen
Keyword(s):  
Cleft Lip ◽  
Wilms Tumor ◽  
Abdominal Mass ◽  
Surgical Excision ◽  
Abdominal Tumor ◽  
Facial Cleft ◽  
Rare Association ◽  
Two Generations ◽  
Cleft Lip Palate ◽  
History Of

Objective: The association of cleft lip/palate (CLP) with other anomalies is not uncommon, but its association with Wilms tumor (WT) is very rare, especially in a familial pattern. In this report, we present a family in which six members in two generations were affected with CLP, WT, or both. Patients and Results: A male patient presented with right complete CLP. He had a family history of facial cleft and abdominal tumor. Lip repair was performed at 3 months of age. An abdominal mass was noticed at 12 months of age, which proved to be WT. Surgical excision of the tumor and chemotherapy were conducted. He subsequently underwent palate repair. His father had an unrepaired microform cleft lip. Three of his aunts were known to have similar problems: one had both facial cleft and WT, one had WT only, and the other had facial cleft only. One of his cousins also was affected with WT. Conclusions: This is a unique family affected with a rare association of CLP and WT. Pedigree study revealed an autosomal dominant hereditary pattern.

Is partial nephrectomy appropriate treatment for unilateral Wilms' tumor?

1998 ◽  
Vol 33 (2) ◽  
pp. 165-170 ◽  
Author(s):  
Catharina G.M Moorman-Voestermans ◽  
Daniel C Aronson ◽  
Chris R Staalman ◽  
Jan F.M Delemarre ◽  
Jan de Kraker
Keyword(s):  
Partial Nephrectomy ◽  
Wilms Tumor ◽  
Appropriate Treatment

IMMEDIATE NEONATAL OUTCOME OF LATE PRETERMS AND EARLY TERMS COMPARED TO TERMS

2021 ◽  
pp. 8-11
Author(s):  
Kausik Sur ◽  
Brajagopal Ray
Keyword(s):  
Retrospective Review ◽  
Congenital Anomalies ◽  
Neonatal Outcome ◽  
Neonatal Period ◽  
Neonatal Morbidity ◽  
University Hospital ◽  
Early Term ◽  
Nicu Admission ◽  
The City ◽  
Pro Forma

Introduction: Increasing number of Late Preterm (LPT) babies are being born in recent years, and they suffer from increased neonatal morbidities and moralities. Objective: +0 +6 To assess the immediate neonatal morbidities and mortalities of Late Pretrem - LPT - (34 -36 weeks) compared to +0 +6 +0 +6 those babies born at Early Term -ET- (37 - 38 weeks) and Term - T- (39 - 40 weeks) gestation. Methodology: A retrospective review of delivery, admission, discharge and death registers and necessary case notes of all live in-born babies excluding those with congenital anomalies were done in RKM Seva Pratisthan Hospital, a teriray level University Hospital in the City of Kolkata, between 1st January 2018 till 31st December 2019. Data collected in a predesigned pro forma were analysed with SPSS vs 23 software, after obtaining necessary permission. All the major neonatal morbidities and morality were compared between the three groups. Results: +0 +6 Total 6511 babies born between gestation 34 and 40 weeks were analysed of which 1021 were in LPT group (31.9%requiring NICU admission), 3408 in ET group (8.3% requiring NICU admission) and 2068 in T group (9.2% requiring NICU admission). There was no signicant difference in mortality (LPT vs ET vsT : 0.5% vs 0.3% vs 0.1%). All the major morbidities ( Respiratory, CNS, Infectious, Metabolic and Any morbidity) were signicantly higher in LPT group. No signicant difference were observed between ET and Term group in terms of any neonatal morbidity or mortality. Conclusion: Late Pretrem newborns suffer from increased neonatal morbidity compared to their Early Term or Term counterpart in immediate neonatal period. No difference of morbidity or mortality were noted between Early Term and Term groups.

Neonatal cleft lip repair: a retrospective review of anaesthetic complications

1997 ◽  
Vol 7 (1) ◽  
pp. 33-36 ◽  
Author(s):  
P. STEPHENS ◽  
P. SAUNDERS ◽  
R. BINGHAM
Keyword(s):  
Retrospective Review ◽  
Cleft Lip ◽  
Cleft Lip Repair

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

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.

Standard of care for adult Wilms tumor? From adult urologist to pediatric oncologist. A retrospective review

2020 ◽  
Author(s):  
Hélène Sudour-Bonnange ◽  
Aurore Coulomb-Lherminé ◽  
Jean Christophe Fantoni ◽  
Alexandre Escande ◽  
Hervé J. Brisse ◽  
...  
Keyword(s):  
Retrospective Review ◽  
Wilms Tumor ◽  
Standard Of Care ◽  
Pediatric Oncologist

Congenital Defects of Squirrel Monkeys (Saimiri sciureus)

1981 ◽  
Vol 18 (1) ◽  
pp. 29-36 ◽  
Author(s):  
H. F. Stills ◽  
B. C. Bullock
Keyword(s):  
Congenital Anomalies ◽  
Cleft Lip ◽  
Left Kidney ◽  
Squirrel Monkeys ◽  
Saimiri Sciureus ◽  
Congenital Defects ◽  
Skeletal Defects ◽  
High Incidence ◽  
Primary Palate ◽  
Ultimobranchial Gland

Nine congenital defects were seen in six animals during necropsies of 27 aborted or stillborn and 25 neonatal squirrel monkeys ( Saimiri sciureus) during a two-year period. The defects were left diaphragmatic hypoplasia with herniation; right renal aplasia in conjunction with a right cleft lip and primary palate; supernumerary left kidney; skeletal defects, hydrocephalus, and thyroid anomalies consistent with the campomelic syndrome; atresia ani; and ultimobranchial gland remnants. The high incidence of congenital anomalies (4.9% of births and 11.5% of nonsurviving births) is consistent with a previous report of congenital defects in squirrel monkeys.

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